• Fundamentals
    • 1

      Clinical Trial Design and Statistics

      By Julie Ann Sosa, MA, MD, FACS
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      Clinical Trial Design and Statistics

      • JULIE ANN SOSA, MA, MD, FACSAssociate Professor of Surgery, Divisions of Endocrine Surgery and Surgical Oncology, Department of Surgery, Yale University School of Medicine, New Haven, CT

      A clinical trial is a planned experiment designed to prospectively measure the efficacy or effectiveness of an intervention by comparing outcomes in a group of subjects treated with the test intervention with those observed in one or more comparable group(s) of subjects receiving another intervention.  Historically, the gold standard for a clinical trial has been a prospective, randomized, double-blind study, but it is sometimes impractical or unethical to conduct such in clinical medicine and surgery. Conventional outcomes have traditionally been clinical end points; with the rise of new technologies, however, they are increasingly being supplemented and/or replaced by surrogate end points, such as serum biomarkers. Because patients are involved, safety considerations and ethical principles must be incorporated into all phases of clinical trial design, conduct, data analysis, and presentation. This review covers the history of clinical trials, clinical trial phases, ethical issues, implementing the study, basic biostatistics for data analysis, and other resources. Figures show drug development and clinical trial process, and type I and II error. Tables list Food and Drug Administration new drug application types, and types of missing data in clinical trials.

      This review contains 2 highly rendered figures, 2 tables, and 38 references

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    • 2

      Principles of Cancer Treatment

      By Samantha J. Baker, MD; J. Bart Rose, MD, MAS
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      Principles of Cancer Treatment

      • SAMANTHA J. BAKER, MDGeneral Surgery Resident, Department of Surgery, University of Alabama at Birmingham, Birmingham, AL
      • J. BART ROSE, MD, MASAssistant Professor, Department of Surgery, University of Alabama at Birmingham, Birmingham, AL

      Surgeons often play a pivotal role in the treatment plans for cancer patients, especially when the plan of action includes resection. Locoregional therapies have the advantage of treating the tumor and its local environment while minimizing systemic effects. Other examples of local treatment include radiotherapy and chemotherapy delivered with isolation techniques. In contrast, systemic chemotherapy, systemic radiation, hormone therapy, and immunotherapy are administered throughout the body. Systemic therapy is most useful in treating disease distant to the site of origin but can be limited by systemic side effects. This chapter explores each of these treatment arms in more detail and provides examples of when such options may be deployed.

      This review contains 1 figure, 2 tables, and 67 references.

      Key Words: Biopsy, Cancer, Chemotherapy, Directed therapy, Hormone therapy, Immunotherapy, Lymph nodes, Proton therapy, Radiation therapy, Staging

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    • 3

      The Immunocompromised Surgical Patient

      By Rhiannon Deierhoi Reed, MPH; Brittany Shelton, MPH; Jayme E. Locke, MD, MPH, FACS
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      The Immunocompromised Surgical Patient

      • RHIANNON DEIERHOI REED, MPHClinical Database Manager, Comprehensive Transplant Institute Outcomes Center, University of Alabama at Birmingham, Birmingham, AL
      • BRITTANY SHELTON, MPHResearch Assistant, University of Alabama at Birmingham, Birmingham, AL
      • JAYME E. LOCKE, MD, MPH, FACSAssistant Professor of Surgery, Division of Transplantation, University of Alabama at Birmingham, Birmingham, AL

      General surgeons are encountering an increasing number of cases involving immunosuppressed patients due to a number of factors, including the improvement in treatment for HIV, increased survival following solid-organ transplantation, and more aggressive chemotherapy. These groups of patients present unique challenges for the surgeon and often require more comprehensive preoperative assessment and perioperative monitoring. This review addresses the surgical management of these immunocompromised populations, with specific recommendations for each type of patient. Tables outline opportunistic infections and antibiotic prophylaxis; common immunosuppressive medications, posttransplantation drug levels, and side effects for renal transplant recipients; components of preoperative workup involving suspected infection in immunocompromised patients; and anesthetics and demonstrated impact on immune response and cancer recurrence. Graphs display the number of AIDS diagnoses and deaths and people living with AIDS and HIV in the United States over time, and compare percentages of death certificates reporting opportunistic infection versus chronic disease in the HIV-infected population. Management algorithms outline approaches to patients with defects in host defenses and candidates for transplantation to be deliberately immunosuppressed.

      This review contains 2 graphs, 2 management algorithms, 4 tables, 157 references, and 5 annotated key references.

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    • 4

      Principles of Cancer Diagnosis

      By Samantha J. Baker, MD; J. Bart Rose, MD, MAS
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      Principles of Cancer Diagnosis

      • SAMANTHA J. BAKER, MDGeneral Surgery Resident, Department of Surgery, University of Alabama at Birmingham, Birmingham, AL
      • J. BART ROSE, MD, MASAssistant Professor, Department of Surgery, University of Alabama at Birmingham, Birmingham, AL

      As knowledge of cancer pathology deepens, so does the complexity of cancer care. Recommendations from the National Cancer Policy Board Cancer Care System focus on using high-volume centers for patients undergoing high-mortality procedures and clinical trials to develop evidence-based guidelines for cancer prevention, diagnosis, treatment, palliative care, and quality care. Through the implementation of many of these principles, patient-centered care has become increasingly recognized as fundamental model for healthcare.

      This review contains 2 figures, 1 table, and 25 references.

      Key Words: Cancer, Cancer staging, Core needle biopsy, Fine needle aspiration, Liquid biopsy, Lymph nodes, Sentinel lymph node(s), Surgical biopsy

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  • Breast Cancer
    • 1

      Pregnancy-associated Breast Cancer

      By Soojin Ahn, MD; Elisa R. Port, MD, FACS
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      Pregnancy-associated Breast Cancer

      • SOOJIN AHN, MDAssistant Professor, Icahn School of Medicine at Mount Sinai, Department of Surgery
      • ELISA R. PORT, MD, FACSAssociate Professor, Icahn School of Medicine at Mount Sinai, Department of Surgery

      Breast cancer diagnosed during pregnancy or in the first postpartum year is referred to as pregnancy-associated breast cancer (PABC). With more women postponing childbearing until later in life, the incidence of PABC is expected to increase. Often there is a delay in diagnosis as small tumors are difficult to detect on a physical examination, and new findings may be obscured against the background of normal physiologic changes associated with pregnancy. Therefore, PABC tends to be diagnosed at a more advanced stage. Once an abnormality is suspected, diagnostic testing should be pursued. Breast ultrasonography is the imaging study of choice, although mammography can be safely performed with proper shielding. Magnetic resonance imaging with contrast is contraindicated due to possible teratogenic effects of gadolinium. Core-needle biopsy of any suspicious masses should be performed. After the establishment of the diagnosis, multidisciplinary treatment planning should be undertaken to optimize the outcome for both the mother and the baby. As certain systemic therapy agents and radiotherapy cannot be safely given during pregnancy, the sequence and timing of the different components of multimodality treatment must be carefully planned. PABC was previously thought to confer a poorer prognosis, but recent studies have shown no significant difference in survival between pregnant and nonpregnant patients when matched by age and stage. 

      Key words: antihormonal therapy, breast cancer, breast surgery, chemotherapy, oncofertility, pregnancy, prognosis, radiation, sentinel lymph node biopsy

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    • 2

      Ductal Carcinoma in Situ (DCIS)

      By Patricia A Cronin, MD, FRCS; Kimberly J Van Zee, MS, MD, FACS
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      Ductal Carcinoma in Situ (DCIS)

      • PATRICIA A CRONIN, MD, FRCSSurgical Fellow, Breast Service, Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, NY
      • KIMBERLY J VAN ZEE, MS, MD, FACSAttending Surgeon, Breast Service, Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, NY

      Ductal carcinoma in situ(DCIS) is noninvasive intraductal carcinoma of the breast and is defined as a malignant proliferation of ductal epithelial cells that are confined to the milk ducts. It is a nonobligate precursor of invasive breast cancer, but at present, there is no reliable method of predicting which lesions will invade in a given time frame. Historically, DCIS was an uncommon lesion; however, widespread use of screening mammography has resulted in a significant increase in the rate of detection, and DCIS now accounts for about 20% of all breast cancers in the United States. Current treatment options for DCIS include breast-conserving surgery (BCS) alone, BCS with radiotherapy (RT), BCS with endocrine therapy, BCS with both RT and endocrine therapy, mastectomy, and even bilateral mastectomy. There is currently concern about overtreatment of this lesion, but there are no reliable data regarding outcomes without treatment. Although survival is excellent with all standard treatments, local recurrence rates vary widely with various treatment options. Given the variety of options available, the goal of treatment is to tailor the management plan to the individual and optimize the balance of risks and benefits according to the values and priorities of the woman herself.

      This review contains 10 figures, 6 tables and 54 references.

      Key words: active surveillance, breast conservation, ductal carcinoma in situ, endocrine therapy, intraductal carcinoma, margins, mastectomy, radiation, recurrence, risk factors

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    • 3

      Lobular Breast Cancer

      By Anita Mamtani, MD; Tari A King, MD
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      Lobular Breast Cancer

      • ANITA MAMTANI, MDChief Resident, Department of Surgery, Beth Israel Deaconess Medical Center, Boston, MA; Clinical Fellow in Surgery, Harvard Medical School, Boston, MA
      • TARI A KING, MDAssociate Chair for Multidisciplinary Oncology, Department of Surgery, Brigham and Women’s Hospital, Boston, MA; Chief of Breast Surgery, Dana-Farber/Brigham and Women’s Cancer Center, Boston, MA; Anne E. Dyson Associate Professor of Surgery in the Field of Women’s Cancers, Harvard Medical School, Boston, MA

      Invasive lobular carcinoma (ILC) is the second most common type of breast cancer, with a unique pathogenesis and distinct clinical biology. These cancers display a characteristic loss of E-cadherin, and the vast majority are estrogen receptor positive (ER+), with a low-to-intermediate grade. These features generally portend a favorable prognosis, but there is a propensity for late recurrences and metastasis to atypical locations. These infiltrative tumors can be more clinically challenging than the more common ductal cancers. Their insidious nature can make them more difficult to diagnose, and they may show a differing response to standard therapies, keeping with the predominantly ER+ phenotype. Although ILC patients comprise a small minority in large breast cancer trials, most fundamentals of locoregional and systemic therapy presently remain shared between all cancer types. Recognizing the nuances of treating ILC remains important, and the recent discovery of novel mutations that are more frequently expressed in ILC holds promise for further investigations into lobular-specific targeted therapies.

      This review 3 figures, 8 tables and 50 references

      Key words: aromatase inhibitors, breast cancer, breast conservation, chemotherapy, E-cadherin, invasive lobular carcinoma, mastectomy, The Cancer Genome Analysis 

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    • 4

      Benign Breast Disease

      By Stephanie A Valente, DO, FACS; Stephen R Grobmyer, MD, FACS
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      Benign Breast Disease

      • STEPHANIE A VALENTE, DO, FACSAssistant Professor of Surgery Staff Breast Surgeon Director, Breast Fellowship Program Department of General Surgery, Division of Breast Services, Cleveland Clinic, Cleveland, OH
      • STEPHEN R GROBMYER, MD, FACSProfessor of Surgery Section Head, Surgical Oncology Co-Leader, Cleveland Clinic Comprehensive Breast Cancer Program Department of General Surgery, Division of Breast Services, Cleveland Clinic, Cleveland, OH

      Benign breast disease constitutes any abnormality that upon further workup is found to be noncancerous and does not establish premalignant potential. Benign breast diseases can present in various ways, such as breast pain, a palpable breast lump, nipple discharge, or an imaging abnormality. Appropriate workup is essential to ensure that the new change is benign. This may entail additional imaging, biopsy, or surgery if necessary. Surgeons need to be able to recognize the presentation, appropriate workup, and treatment recommendations for the various benign breast diseases. This review addresses the broad range of benign breast lesions and their management recommendations. 


      This review contains 7 figures, 5 tables and 54 references

      Key words: benign breast disease, breast cyst, breast mass, breast pain, idiopathic granulomatous mastitis, nipple discharge, papilloma, periductal mastitis, sclerosing adenosis

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    • 5

      Surgical Management of the Breast

      By Anees B Chagpar, MD, MSc, MPH, MA, MBA
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      Surgical Management of the Breast

      • ANEES B CHAGPAR, MD, MSC, MPH, MA, MBAAssociate Professor of Surgery, Yale University School of Medicine, New Haven, CT

      The last several decades have seen a dramatic metamorphosis in the surgical management of breast cancer. The diagnosis of this malignancy is now more often made using minimally invasive imaging-directed techniques. Cancers that were routinely managed with disfiguring radical mastectomies are now treated with breast-conserving surgery, and axillary dissection has yielded to sentinel node biopsy, which may or may not be followed with completion dissection even in the setting of node-positive disease. Increasingly, breast surgical oncology is nested within a multidisciplinary context, and advances in a number of disciplines have yielded revolutionary changes in the surgical management of the leading malignancy affecting women in the United States. This review discusses advances in the surgical management of breast cancer in this context, highlighting key clinical trials that have led to these changes.

      This review contains 4 figures, 6 tables and 72 references

      Key words: axillary dissection, breast conservation, chemotherapy, margins, mastectomy, multidisciplinary, oncoplastic, radiation therapy, reconstruction, sentinel node biopsy 

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    • 6

      High-risk Breast Lesions

      By Richard J Bleicher, MD, FACS
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      High-risk Breast Lesions

      • RICHARD J BLEICHER, MD, FACSProfessor, Department of Surgical Oncology, Leader, Breast Service Line, Director, Breast Fellowship Program, Fox Chase Cancer Center, Philadelphia, PA

      Clinicians who treat breast disease deal with a variety of pathologic findings. Although there is often a focus on paradigms and data regarding malignant breast disease, benign breast disease ranges from that which requires no imaging, evaluation, or treatment to that which requires further testing to rule out concomitant cancer and ameliorate subsequent risk. This review discusses the most frequent types of high-risk lesions facing the breast practitioner, including atypical ductal hyperplasia, lobular neoplasia, radial scar, and papillary lesions. It is critical that the clinician understand the implications of each finding to advise about the risks associated with each of these entities, fully assess the need for further therapy, and mitigate the patient’s future risk. 

      This review contains 2 tables, and 69 references.

      Key words: atypical ductal hyperplasia, atypical lobular hyperplasia, lobular neoplasia, lobular carcinoma in situ, papillary lesions, pleomorphic, pseudoangiomatous stromal hyperplasia, radial scar, usual ductal hyperplasia

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    • 7

      Management of Patients With Genetic Predisposition to Breast Cancer

      By David M Euhus, MD; Maureen O’Donnell, MD
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      Management of Patients With Genetic Predisposition to Breast Cancer

      • DAVID M EUHUS, MDProfessor of Surgery
      • MAUREEN O’DONNELL, MD Clinical Associate, Department of Surgery, Johns Hopkins Sibley Memorial Hospital, Washington, DC

      Commercialization of massive parallel sequencing and reversal of patent protection for raw DNA sequences have fueled competition in the genetic testing marketplace, leading to lower costs and abundant choices for consumers. Multigene panel testing will identify a pathogenic mutation in 4 to 12% of familial high-risk patients who have tested negative for BRCA1 and BRCA2 mutation. BRCA1 and BRCA2 are still the genes most frequently implicated in hereditary breast cancer predisposition.  PALB2, CHEK2, and ATM are the next most common. Variants of uncertain clinical significance are reported for 20 to 40% of tests. This is likely to improve as data accumulate. Average cancer risk can be estimated for most of the genes, but this should be adjusted up or down based on family history for management decisions. Most patients with a pathologic mutation in any of the breast cancer predisposition genes meet the criteria for enhanced surveillance with magnetic resonance imaging. Risk-reducing surgery is considered for the highest-risk patients. The results of a genetic test can influence management decisions for the newly diagnosed breast cancer patient. 

      This review contains 4 figures, 7 tables and 51 references

      Key Words: breast cancer, chemoprevention, enhanced surveillance, hereditary predisposition, multigene panels, risk-reducing mastectomy, risk-reducing salpingo-oophorectomy

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    • 8

      Surgical Management of the Axilla in Breast Cancer

      By Cory Donovan, MD; Armando E Giuliano, MD
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      Surgical Management of the Axilla in Breast Cancer

      • CORY DONOVAN, MDSurgeon, Breast Oncology, Cedars-Sinai Medical Center, Los Angeles, CAg
      • ARMANDO E GIULIANO, MDProfessor of Surgery, Executive Vice Chair, Surgery, Linda and Jim Lippman Chair in Surgical Oncology, Cedars-Sinai Medical Center, Clinical Professor of Surgery, University of California-Los Angeles, Los Angeles, CA

      The management of the axilla in breast cancer has shifted from axillary dissection in all patients to sentinel lymph node biopsy (SLNB) alone for most patients, including patients with sentinel lymph node metastases. Although important to clinical staging, physical examination alone does not accurately predict axillary metastasis. There are some circumstances where SLNB is contraindicated or should be used with caution. The impact of SLNB after neoadjuvant chemotherapy remains unproven, but its use is reasonable for some patients. Patients with tumor-free sentinel lymph nodes or nodes with micrometastatic disease require no further axillary surgery. Most patients with one to three lymph nodes positive for macrometastatic disease who undergo segmental mastectomy and radiation do not require an axillary lymph node dissection (ALND). There has not been a dramatic increase in axillary recurrence or a decrease in survival with the decreased use of ALND. In the future, with improvements in genomic analysis, ALND and even SLNB may be even less important in local control and prognosis. 

      This review contains 9 figures, 7 tables and 52 references. 

      Key words: ACOSOG Z0011, axilla, axillary dissection, axillary radiation, breast cancer, macrometastasis, micrometastasis, sentinel lymph node biopsy 

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    • 9

      Phyllodes Tumors

      By Tina J Hieken, MD; Rafael E Jimenez, MD
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      Phyllodes Tumors

      • TINA J HIEKEN, MDAssociate Professor, Department of Surgery, Mayo Clinic, Rochester, MN
      • RAFAEL E JIMENEZ, MDAssociate Professor, Department of Pathology, Mayo Clinic, Rochester, MN

      Phyllodes tumors are rare primary breast neoplasms graded as benign, borderline, or malignant based on pathology characterization of the stromal component. Core-needle biopsy is recommended as the first diagnostic step for breast lesions suspicious for phyllodes. Surgical excisional biopsy is recommended for a core-needle biopsy showing cellular fibroepithelial lesion or a mass suspicious for phyllodes tumor as pathology diagnosis is challenging, especially on limited tissue specimens. Surgical treatment parallels that for soft tissue sarcoma rather than breast adenocarcinoma. Wide local excision and mastectomy, with 1 cm tumor-free margins, provide equivalent oncologic outcomes. Tumor enucleation, subtotal resection, and positive final margins should be avoided. Axillary surgery is not recommended as lymph node metastases are rare. Adjuvant radiation is not recommended as routine, nor is adjuvant chemotherapy, although either or both may be considered for primary or recurrent high-risk malignant phyllodes tumors on a case-by-case basis. Local recurrence rates are influenced by margin status and tumor grade. Distant metastases occur in up to 25% of patients with borderline/malignant tumors and bestow a poor prognosis. Recent molecular genetic analyses of phyllodes tumors have identified potentially targetable mutations that may guide future therapy for high-risk, recurrent, or metastatic phyllodes tumors.

      This review contains 5 figures, 5 tables, and 53 references.

      Key words: biomarkers, breast sarcoma, diagnosis, outcomes, pathology, phyllodes tumor, surgery, treatment

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    • 10

      Locoregional Therapy for the Primary Tumor in Stage IV Breast Cancer

      By Sasa M Espino, MD; Swati Kulkarni, MD, FACS
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      Locoregional Therapy for the Primary Tumor in Stage IV Breast Cancer

      • SASA M ESPINO, MDBreast Surgery Fellow, Division of Breast Surgery, Northwestern Memorial Hospital, Chicago, IL
      • SWATI KULKARNI, MD, FACSAssociate Professor of Surgery, Division of Breast Surgery, Northwestern Memorial Hospital, Chicago, IL

      Locoregional therapy, comprising surgery and radiation, has traditionally been reserved for managing symptoms from the primary tumor in women with stage IV breast cancer. However, in recent years, there has been significant interest in expanding the indications of local therapy in women with metastatic breast cancer beyond palliation to prolonging survival. In this review, we first discuss biological hypotheses of breast cancer metastasis that support the use of locoregional therapy in stage IV breast cancer. Second, we summarize the data from the key retrospective studies of locoregional therapy in stage IV breast cancer. Although many of these studies demonstrated a survival advantage over women who received systemic therapy alone, a number of key questions remained unanswered, including the timing, extent, and type of locoregional therapy that would provide maximal benefit to patients with metastatic disease while preserving quality of life. Lastly, three recently completed prospective trials have reported inconsistent results, with two demonstrating no survival advantage over systemic therapy alone and the third showing an increased overall survival after longer follow-up. Three other prospective trials are ongoing and attempt to address the limitations of the retrospective review and determine which class of patients would benefit most from additional intervention.

      This review contains 8 figures, 4 tables, and 44 references.

      Key words: axillary surgery, biology of breast cancer metastasis, breast surgery, clinical trials, locoregional therapy, margin status, quality of life, radiation therapy, stage IV breast cancer

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    • 11

      Breast Reconstruction

      By Graham S Schwarz, MD, FACS; Eliana F R Duraes, MD
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      Breast Reconstruction

      • GRAHAM S SCHWARZ, MD, FACSAssistant Professor, Department of Plastic Surgery, Cleveland Clinic, Cleveland, OH
      • ELIANA F R DURAES, MDClinical Research Fellow, Department of Plastic Surgery, Cleveland Clinic, Cleveland, OH

      Breast reconstruction is an essential component of breast cancer multidisciplinary care. Restoring the breast form has repeatedly been shown to improve self-image, psychological well-being, and overall quality of life. Excellent aesthetic and functional results may be obtained with close collaboration between the breast surgical oncology and reconstructive plastic surgery teams. Plastic surgical consultation should be obtained for all women undergoing mastectomy and in selected women with anticipated contour deformity following breast conservation therapy. Most breast cancer patients will undergo reconstruction in stages coordinated with the individual’s multimodal oncologic therapy. In the majority of patients undergoing mastectomy, this reconstructive process may begin immediately at the time of breast tissue removal. Implant-based and autologous reconstructive approaches may both yield excellent results but need to be matched with patients’ goals, lifestyle, anatomy, and medical comorbidities.   

      This review contains 15 figures, 7 tables and 55 references

      Key words: autologous reconstruction, breast implant, breast reconstruction, flap, microsurgical breast reconstruction, nipple-sparing mastectomy, oncoplastic, perforator flap, single stage

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    • 12

      Male Breast Cancer

      By Kimberly S. Stone, MD; Irene L Wapnir, MD
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      Male Breast Cancer

      • KIMBERLY S. STONE, MDClinical Instructor, Department of Surgery, Stanford University School of Medicine, Stanford, CA.
      • IRENE L WAPNIR, MDProfessor of Surgery, Chief of Breast Surgery, Department of Surgery, Stanford University School of Medicine, Stanford, CA.

      This is a contemporary review of risk factors and genetic predisposition associated with breast cancer in men. The workup and evaluation of breast masses and surgical management of cancer in this population are detailed. In addition, systemic therapies for the treatment of breast cancer in men are reviewed and compared with the therapeutic approaches used for women.   

      This reviews contains 5 figures, 2 tables and 50 references. 

      Key words: breast-conserving surgery, chemotherapy, endocrine therapy, genetic predisposition testing, genomic profiling, lymphadenectomy, male breast cancer, mastectomy, radiotherapy, sentinel lymph node biopsy

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    • 13

      Paget Disease of the Breast

      By Amanda Wheeler, MD; Sarah McLaughlin, MD
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      Paget Disease of the Breast

      • AMANDA WHEELER, MDAssistant Professor of Surgery, Stanford University, Stanford, CA
      • SARAH MCLAUGHLIN, MDAssociate Professor of Surgery, Mayo Clinic Florida, Jacksonville, FL

      Paget disease of the breast is an in situ malignancy located within the epidermal layer of the nipple-areolar complex. It accounts for 0.5% of all breast cancers in women and 1 to 3% of new breast cancers diagnosed annually in the United States. Patients most likely present with erosion at the tip or base of the nipple. These symptoms are often confused with the symptoms of eczema. Persistent itching can occur. As the disease progresses, ulceration of the nipple expands outward over the areola. Full-thickness biopsy of the nipple or involved skin is needed to confirm diagnosis. Further workup includes mammography and, frequently, breast magnetic resonance imaging to rule out an associated breast parenchymal malignancy. Surgical excision requires removal of the nipple and involved areolar complex as well as any underlying malignancy. Mastectomy is also an option and may be required if additional multicentric disease is present.

      This review contains 10 figures, 6 tables, and 52 references.

      Key words: breast conservation, breast magnetic resonance imaging, eczema, intraepithelial adenocarcinoma, nipple abnormalities, nipple biopsy, occult malignancy, Paget disease 

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    • 14

      Sarcomas of the Breast

      By Corbin A Rayfield, MD; Sanjay P Bagaria, MD; Nabil Wasif, MD, MPH
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      Sarcomas of the Breast

      • CORBIN A RAYFIELD, MD
      • SANJAY P BAGARIA, MD
      • NABIL WASIF, MD, MPH

      Sarcomas of the breast can be sporadic (primary) or secondary (postradiation). In this review, we discuss clinical presentation, imaging characteristics, diagnostic evaluation, and histologic classification of these rare tumors. Evidence-based management of breast sarcoma with regard to surgery, neoadjuvant or adjuvant chemotherapy and radiation, nodal staging, and outcomes are also discussed. Finally, a clinical case with radiologic and pathologic correlates is presented.

      This review contains 3 figures, 5 tables, and 44 references.

      Key words: angiosarcoma, breast sarcoma, radiation-induced sarcoma

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    • 15

      Estrogen Receptor–positive, HER-2 Nonamplified Breast Cancer

      By Leisha C Elmore, MD, MPHS; Julie A. Margenthaler, MD
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      Estrogen Receptor–positive, HER-2 Nonamplified Breast Cancer

      • LEISHA C ELMORE, MD, MPHSSurgical Resident, Department of Surgery, Washington University in St. Louis School of Medicine, St. Louis,
      • JULIE A. MARGENTHALER, MDProfessor of Surgery, Department of Surgery, Washington University in St. Louis School of Medicine, St. Louis, MO

      Although histologic type provides critical insight into the behavior and natural history of breast cancer, gene expression profiling plays an important role in determining prognosis and guiding treatment selection. Tumor cells express biologically active molecules on the cell surface and within the cell that serve as receptors and rely on ligands to mediate a response to the cell. Two nuclear receptors, estrogen receptor (ER) and progesterone receptor (PR), and one cell surface receptor, human epidermal growth factor 2 receptor (HER-2), have been established as critical factors in the management of breast cancer. The first hormone receptor, ER, was identified and described in the 1960s. Advances in genomic analysis have allowed for molecular subtyping within the umbrella of hormone receptor status. Here we focus on tumors that express ER but do not express HER-2 and the relevant diagnostic workup and treatment (neoadjuvant and adjuvant) strategies.

      This review contains 5 figures, 5 tables, and 41 references.

      Key words: adjuvant therapy, breast cancer, endocrine therapy, estrogen receptor positive, HER-2 nonamplified, hormone receptors

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    • 16

      Axillary Metastasis With Occult Primary Breast Cancer

      By Anne Kuritzky, MD; Laila Khazai, MD; Roberto Diaz, MD, PhD; Christine Laronga, MD
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      Axillary Metastasis With Occult Primary Breast Cancer

      • ANNE KURITZKY, MDBreast Surgical Oncology Fellow, Department of Breast Oncology, H. Lee Moffitt Cancer Center, Tampa, FL
      • LAILA KHAZAI, MDAssistant Member, Department of Pathology, H. Lee Moffitt Cancer Center, Tampa, FL
      • ROBERTO DIAZ, MD, PHDAssociate Member, Department of Radiation Oncology, H. Lee Moffitt Cancer Center, Tampa, FL
      • CHRISTINE LARONGA, MDSenior Member, Department of Breast Oncology, H. Lee Moffitt Cancer Center, Tampa, FL

      The identification of an axillary metastasis in the absence of a primary breast cancer can pose a diagnostic and therapeutic dilemma. The clinician should first use more sensitive imaging modalities, such as breast magnetic resonance imaging, to attempt to find the primary index lesion. If the primary cancer remains occult and the molecular markers are consistent with a breast origin, then the recommended treatment includes multimodality therapy including surgery, chemotherapy/endocrine treatment, and radiation. Historically, the modified radical mastectomy was the standard of practice. Recently, in the era of improved adjuvant therapies, breast-conserving surgery with irradiation is also being considered. Multiple retrospective reviews have shown no difference in survival or recurrence with these two surgical pathways. However, due to the rare nature of this clinical presentation, no multiinstitutional or prospective clinical trial data are available. 

      Key words: axillary lymph node dissection, axillary metastasis, breast cancer, breast conservation, local recurrence, occult primary tumor, radiotherapy 

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    • 17

      Breast Cancer in the Elderly

      By Catherine Pesce, MD; Katharine Yao, MD
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      Breast Cancer in the Elderly

      • CATHERINE PESCE, MDDirector, Breast Surgical Program, Department of Surgery, NorthShore University Health System, Evanston, IL; Clinical Assistant Professor of Surgery, University of Chicago, Pritzker School of Medicine, Chicago, IL
      • KATHARINE YAO, MDChief, Division of Surgical Oncology, Department of Surgery, NorthShore University Health System, Evanston, IL; Clinical Professor of Surgery, University of Chicago, Pritzker School of Medicine, Chicago, IL

      Elderly patients with breast cancer are more likely to present with more favorable tumor characteristics and molecular subtypes; however, outcomes are worse, with lower survival rates compared with younger women. Less use of screening, undertreatment, the frequency of comorbidities, and the lack of information in clinical trials on the use of systemic therapy in this population all likely play a role. Unless patients have a prohibitive risk for surgery or a life expectancy less than 5 years, surgery should be considered for elderly patients who are surgically resectable. Radiation and chemotherapy are less likely to be used in elderly patients; however, with an increase in the use of neoadjuvant therapy for breast cancer patients, recommendations or guidelines for neoadjuvant therapy for the elderly are needed. Further tools that can assist physicians with risk assessment of elderly patients for both surgery and adjuvant therapies are needed. A multidisciplinary discussion that includes discussion of the need for adjuvant therapy is necessary and should be balanced against the patient’s comorbidities and functional status when deciding on the best course of treatment for these patients. It should be emphasized that elderly patients should be offered standard treatments that nonelderly patients receive, and these recommendations should only be modified if there is limited life expectancy or other socioeconomic factors that influence whether patients can undergo standard treatments.

      This review contains 2 figures, 11 tables, and 60 references

      Key words: breast surgery, breast cancer in the elderly, elderly breast surgery, elderly oncology, geriatric breast surgery


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    • 18

      Her-2-amplified Breast Cancer

      By Zahraa Al-Hilli, MD; Judy C Boughey, MD
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      Her-2-amplified Breast Cancer

      • ZAHRAA AL-HILLI, MDAssistant Professor of Surgery, Department of General Surgery, Cleveland Clinic, Cleveland, OH
      • JUDY C BOUGHEY, MDProfessor of Surgery, Department of Surgery, Mayo Clinic, Rochester, MN

      Amplification of the human epidermal growth factor receptor–2 (HER-2) gene is found in approximately 15 to 30% of breast cancers. Historically, HER-2 overexpression has been associated with aggressive disease and a poor prognosis. However, the use of targeted anti-HER2 therapy has revolutionized the treatment of HER-2-positive disease, and the use of the monoclonal antibody trastuzumab in combination with chemotherapy is now standard of care for tumors greater than 1 cm in size and in node-positive disease. More recently, the value of dual-agent anti-HER-2 therapy has been demonstrated in large clinical trials. This review provides an overview of HER-2-positive breast cancer, its molecular basis, methods of identification, and treatment options and strategies.

      This review contains 2 figures and 70 references

      Key words: anti-HER-2 therapy, breast cancer, HER-2-positive breast cancer, HER-2 resistance, lapatinib, neoadjuvant chemotherapy, pertuzumab, small HER-2-positive breast cancer, trastuzumab

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    • 19

      Breast Cancer in Young Women

      By Lisa A Newman, MD, MPH, FACS, FASCO
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      Breast Cancer in Young Women

      • LISA A NEWMAN, MD, MPH, FACS, FASCODirector, Breast Oncology Program and International Center for the Study of Breast Cancer Subtypes, and Chief, Breast Surgery Section, Weill Cornell Medicine, New York, NY

      The perception that breast cancer in young women is a growing problem in the United States is based on the fact that young women represent a demographic that has enlarged substantially over the past few decades. Population-based data actually reveal relatively stable incidence rates for breast cancer among women in the premenopausal age range. Young women are more likely to be diagnosed with biologically aggressive phenotypes such as triple-negative and HER2/neu-overexpressing breast cancer. Outcomes are optimized by treatment plans focusing on disease stage and targeted to phenotype. Locoregional therapy for breast cancer in young women should be based on patient preferences and disease pattern (as in older patients); young women with breast cancer can be managed safely with breast-conserving surgery.

      This review contains 3 figures, 2 tables, and 50 references.

      Key Words: breast cancer, fertility, ovarian suppression, premenopausal, young women; triple negative breast cancer

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    • 20

      Inflammatory Breast Cancer

      By Janine M Simons, MD; Mediget Teshome, MD; Kelly K Hunt, MD
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      Inflammatory Breast Cancer

      • JANINE M SIMONS, MDResearch Assistant The University of Texas MD Anderson Cancer Center, Houston, TX
      • MEDIGET TESHOME, MDThe University of Texas MD Anderson Cancer Center
      • KELLY K HUNT, MDThe University of Texas MD Anderson Cancer Center, Houston, TX

      Inflammatory breast cancer is a rare but highly aggressive form of breast cancer. It is considered a distinct entity with unique clinicopathologic features. Symptoms of erythema and increase in breast size usually develop over the course of a few weeks. The clinical symptoms result from lymphovascular tumor emboli, which are pathognomonic for inflammatory breast cancer. Timely diagnosis may be challenging, as inflammatory breast cancer can mimic infectious disease such as mastitis or breast abscess. However, timely diagnosis and treatment are very important to provide trimodality management as early as possible. Patients should be imaged for distant metastasis at diagnosis. A combination of neoadjuvant systemic therapy, modified radical mastectomy, and adjuvant radiotherapy is standard of care for inflammatory breast cancer and improves local-regional and systemic control.

      This review contains 7 figures, 3 tables, and 59 references.

      Key Words: clinical presentation, diagnosis, imaging, inflammatory breast cancer, modified radical mastectomy, multimodality treatment, neoadjuvant systemic therapy, radiation therapy, staging

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    • 21

      Management of Locoregional Recurrence of Breast Cancer

      By Devon Livingston-Rosanoff, MD, PhD; Sarah E. Tevis, MD; Lee G. Wilke, MD
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      Management of Locoregional Recurrence of Breast Cancer

      • DEVON LIVINGSTON-ROSANOFF, MD, PHDGeneral Surgery Resident, School of Medicine and Public Health, University of Wisconsin, Madison, WI
      • SARAH E. TEVIS, MDAssistant Professor of Surgery, University of Colorado, Denver, CO
      • LEE G. WILKE, MDProfessor of Surgery, Hendricks Chair in Breast Cancer Research, School of Medicine and Public Health, University of Wisconsin, Madison, WI

      Following treatment for breast cancer, disease can recur locally, regionally, or at distant sites. Locoregional recurrence is defined as recurrence in the ipsilateral breast, skin, chest wall, or regional lymph nodes. Concurrent metastatic disease is common in patients with locoregional recurrence; therefore, patients with recurrence should undergo a complete metastatic work-up. Isolated locoregional recurrence should be approached with curative intent, and patients should undergo resection and adjuvant therapy, as indicated, based on previous treatment and location of recurrence. Following treatment for locoregional recurrence, close monitoring should be performed, as patients are at an increased risk for developing metastatic disease.

      This review contains 5 figures, 1 table, and 50 references

      Key Words: breast cancer, CALOR clinical trial, locoregional recurrence, repeat radiation therapy, repeat sentinel lymph node mapping, adjuvant chemotherapy, repeat breast conserving therapy, mastectomy

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  • Colorectal Cancer
    • 1

      Surgical Treatment of Stage I Rectal Cancer

      By Julio Garcia-Aguilar, MD, PhD
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      Surgical Treatment of Stage I Rectal Cancer

      • JULIO GARCIA-AGUILAR, MD, PHDBenno Schmidt Chair in Surgical Oncology and Chief Attending Surgeon, Colorectal Service, Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, NY

      For treatment of early-stage rectal cancer, local (transanal) excision offers the advantages of lower rates of morbidity, mortality, and functional impairment in comparison with radical surgery such as total mesorectal excision (TME). Minimally invasive platforms facilitate removal of rectal tumors that are beyond the reach of conventional transanal excision techniques. The main drawback of local excision is the higher risk of local recurrence compared with TME. The risk of local recurrence is higher in patients with close resection margins, tumors penetrating beyond the submucosa, or tumors with unfavorable histologic features. In these patients, outcomes for immediate proactive TME are generally better than observation followed by reactive salvage TME in case of local recurrence. The use of neoadjuvant chemoradiotherapy may make local excision a viable option for T2 rectal tumors. As chemoradiation and local excision are being increasingly used for later-stage tumors, advances in imaging technologies will play a crucial role in facilitating careful patient selection.

      This review contains 5 figures, 3 tables and 35 references

      Key words: endocavitary contact radiotherapy, local excision, local recurrence, rectal cancer, salvage surgery, total mesorectal excision, transanal endoscopic operation, transanal excision, transanal minimally invasive surgery

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    • 2

      Locally Advanced Rectal Cancer

      By Martin R Weiser, MD; David A Kleiman, MD, MSc
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      Locally Advanced Rectal Cancer

      • MARTIN R WEISER, MDVice Chair for Education and Faculty Development, Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY
      • DAVID A KLEIMAN, MD, MSCDepartment of Surgery, Memorial Sloan Kettering Cancer Center, New York, NY

      Locally advanced rectal cancer is a complex disease that requires a multidisciplinary treatment team to carefully evaluate each patient before prescribing a treatment plan. The current standard of care in the United States is multimodal therapy, consisting of chemotherapy, radiation, and surgery. Commonly, this involves neoadjuvant long-course chemoradiation, followed by total mesorectal excision and then adjuvant systemic chemotherapy. However, alternative regimens using chemotherapy first, followed by chemoradiation and then surgery (total neoadjuvant therapy), may allow for better tolerance of therapy. Short-course radiation is also acceptable but is rarely used in the United States. Minimally invasive surgical techniques such as laparoscopy, robotic surgery, and transanal total mesorectal excision offer several potential advantages over conventional open surgery, but their oncologic equivalence has not been determined. The role of nonoperative management for locally advanced rectal cancer is still evolving, and additional studies are needed to improve patient selection and evaluate long-term outcomes of a watch-and-wait approach. 

      This review contains 1 figure, 1 table and 57 references

      Key words: colorectal cancer, locally advanced rectal cancer, minimally invasive surgery, nonoperative management, radical proctectomy, robotic surgery, total mesorectal excision, total neoadjuvant therapy, transanal total mesorectal excision, watch and wait

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    • 3

      Epidemiology and Molecular Biology of Colorectal Cancer

      By Tarik Sammour, MBChB, PhD; Miguel A Rodriguez-Bigas, MD
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      Epidemiology and Molecular Biology of Colorectal Cancer

      • TARIK SAMMOUR, MBCHB, PHDAssociate Professor, Colorectal Surgery, Adelaide, South Australia, Australia Hospital & Health Care, Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX
      • MIGUEL A RODRIGUEZ-BIGAS, MDProfessor, Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX

      Colorectal cancer (CRC) is the fourth most common cancer and the second most common cause of cancer-related death in the United States. Nonmodifiable risk factors include age, male sex, African-American ethnicity, and personal or family history of CRC or polyps (especially if these were diagnosed at a younger age), inflammatory bowel disease, or diabetes. Modifiable risk factors include poor physical activity; obesity; high consumption of red meats, processed meats, or alcohol; low total dietary intake of fiber, folate, fruits, or vegetables; and smoking tobacco. There have been several advances in diagnostic techniques, which, when combined with newly discovered genetic pathways, contribute to an expanding knowledge on which to base treatment. There are three known major molecular pathways of CRC carcinogenesis: the chromosomal instability pathway, the microsatellite instability pathway, and the serrated carcinoma pathway. Approximately 5% of all CRC cases will have a specific known genetic mutation associated with a well-characterized familial cancer syndrome with defined features. These syndromes are important to recognize distinctly as their identification facilitates surveillance and management with the aim of prevention, prophylaxis, and surgical cure.

      This review contains 2 figures, 3 tables and 50 references. 

      Key words: colon cancer, colorectal cancer, familial, familial adenomatous polyposis, Lynch syndrome, microsatellite, polyp, polyposis, rectal cancer, serrated

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    • 4

      Polyposis Syndromes

      By Katherine A Kelley, MD; Daniel O Herzig, MD, FACS, FASCRS
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      Polyposis Syndromes

      • KATHERINE A KELLEY, MDResident Physician, Department of Surgery, Oregon Health and Sciences University, Portland, OR
      • DANIEL O HERZIG, MD, FACS, FASCRSAssociate Professor, Division of Gastrointestinal and General Surgery, Department of Surgery, Oregon Health and Sciences University, Portland, OR

      Colorectal cancer (CRC) is the third most common type of cancer and the second leading cause of cancer death in men and women. Although about one third of cancers arise in patients with a family history of CRC, only 5% arise in the setting of mendelian-inherited disorders. Patients without a family history but with a significant polyp burden (> 20 polyps) should be considered to have polyposis syndrome. The field of polyposis syndromes continues to advance, based on new genetic discoveries that define the genetic etiologies of polyposis syndromes. When considered in relation to an individual’s phenotype, these discoveries help guide screening and treatment based on the cancer risk created by specific mutations. Patients with polyposis syndromes carry an increased risk of CRC and some other extracolonic cancers. Future research will provide additional insight into the cause of polyposis syndromes without a currently detectable gene defect and will improve early identification and cancer prevention in affected individuals. Identification of additional molecular characteristics may lead to a more personalized approach to treatment for these individuals.

      This review contains 7 figures, 9 tables and 50 references

      Key words: attenuated familial polyposis, colorectal cancer risk, familial adenomatous polyposis, hamartomatous polyposis syndrome, juvenile polyposis syndrome, MUTYH-associated polyposis, Peutz-Jeghers syndrome, polyposis syndromes, screening guidelines, serrated polyposis syndrome

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    • 5

      Rare Tumors of the Colorectal Region

      By Jonathan S Abelson, MD; Heather L Yeo, MD, MHS
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      Rare Tumors of the Colorectal Region

      • JONATHAN S ABELSON, MDGeneral Surgery Resident, Department of Surgery, New York Presbyterian Hospital – Weill Cornell Medicine, New York, NY
      • HEATHER L YEO, MD, MHSAssistant Professor of Surgery, Assistant Professor of Healthcare Policy and Research, Department of Surgery, New York Presbyterian Hospital – Weill Cornell Medicine, Department of Healthcare Policy and Research, Weill Cornell Medicine, New York, NY

      Rare tumors of the colon and rectum account for less than 5% of all colorectal tumors. The most common of these rare tumors include anorectal melanoma, rectal neuroendocrine tumors, rectal gastrointestinal stromal tumors (GISTs), and retrorectal tumors. Their prognosis is varied and depends on underling pathology. Surgery plays an important role in many of these tumors. Molecularly targeted therapy and immunotherapy have expanding roles in GIST and anal melanoma, respectively. Adjuvant chemotherapy or radiation therapy may be employed in specific circumstances of unresectable or metastatic disease or for symptom control. 

      This review contains 4 figures, 6 tables and 60 references. 

      Key words: anal canal, antineoplastic agents, anus neoplasms, chordoma, gastrointestinal stromal tumors, melanoma, neuroendocrine tumors, operative, radiotherapy, rectal neoplasms, surgical procedures

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    • 6

      Appendiceal Neoplasm

      By Tushar Samdani , MD, FASCRS; Garrett M. Nash, MD, MPH, FACS, FASCRS
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      Appendiceal Neoplasm

      • TUSHAR SAMDANI , MD, FASCRSColorectal Surgeon, Medstar Saint Marys Hospital, Leonardtown, MD
      • GARRETT M. NASH, MD, MPH, FACS, FASCRSAssistant Professor, Colorectal Surgeon, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, NY

      Appendiceal neoplasms are rare and are typically found in the setting of metastatic disease or, less commonly, as an incidental finding in an appendectomy specimen in cases of appendicitis or an unrelated condition. The World Health Organization classifies appendiceal neoplasms as benign or malignant. The management of the mucinous variety of appendiceal neoplasms is influenced by the presence of peritoneal involvement. Selected patients with metastatic appendiceal neoplasms are treated with surgical cytoreduction and intraperitoneal chemotherapy and/or systemic chemotherapy. Nonmetastatic appendiceal adenocarcinoma is typically managed with staging right colectomy and adjuvant fluorouracil-based chemotherapy based on colon cancer guidelines. Neuroendocrine tumors (NETs) constitute 50 to 77% of all appendiceal neoplasms and 19% of all gastrointestinal NETs. Right hemicolectomy is indicated if the size of the NET is more than 2 cm, the base of the appendix is involved, it is a high-grade tumor, or the depth of invasion of the mesoappendix is more than 3 mm.

      This review contains 7 figures, 8 tables and 36 references

      Key Words: appendiceal adenocarcinoma, appendiceal adenoma, appendiceal neoplasm, diffuse peritoneal adenomucinosis, Goblet cell carcinoid of appendix, low-grade appendiceal mucinous neoplasm, mucocele of appendix, neuroendocrine tumor of appendix, peritoneal mucinous carcinomatosis (PMCA), pseudomyxoma peritonei  

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    • 7

      Colon Cancer

      By Richard S Hoehn, MD; Felipe Quezada-Diaz, MD; Jesse J Smith, MD, PhD
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      Colon Cancer

      • RICHARD S HOEHN, MDGeneral Surgery Resident, University of Cincinnati College of Medicine, Cincinnati, OH, United States
      • FELIPE QUEZADA-DIAZ, MDColorectal Surgery Fellow, Memorial Sloan Kettering Cancer Center, New York, NY , United States
      • JESSE J SMITH, MD, PHDAssistant Member, Colorectal Service, Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, NY , United States

      Colon cancer is a leading cause of cancer-related death in the United States and worldwide. Routine screening has led to early diagnosis and improved survival for many patients but is still greatly underused. Complete surgical resection provides the best opportunity for cure of localized disease and requires removal of a defined segment of colon along with its lymphovascular pedicle, including a minimum of 12 lymph nodes. Minimally invasive approaches have been shown to provide better perioperative outcomes and patient recovery, with oncologic outcomes equivalent to those of traditional open surgery. Patients with lymph node metastases are at an increased risk of distant metastases and disease recurrence. Survival for these patients has improved in the recent years with the advent of oxaliplatin-based adjuvant chemotherapy. In addition, surgical resection is increasingly being used to control and sometimes cure distant metastases. In this chapter, we review the current strategies for diagnosing and managing colon cancer.

      This review contains 1 video, 5 figures, 4 tables and 48 references

      Key Words: adjuvant chemotherapy, anastomosis, colectomy, colon cancer, neoadjuvant chemotherapy, surgery, survival, laparoscopic, robotic

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    • 8

      Anal Cancer

      By Miriam Tsao, MD; David Shibata, MD
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      Anal Cancer

      • MIRIAM TSAO, MDClinical Fellow, Division of Surgical Oncology, Department of Surgery University of Tennessee Health Science Center, Memphis, TN
      • DAVID SHIBATA, MDProfessor and Chair of the Department of Surgery, Division of Surgical Oncology, Department of Surgery University of Tennessee Health Science Center, Memphis, TN

      This chapter focuses on anal squamous cell carcinoma and its precursor lesions and summarizes the relevant epidemiology, evaluation, and management. Risk factors including human immunodeficiency virus status and sexual practices as well as the role of human papilloma virus in anal cancer pathogenesis are reviewed. Further understanding of the molecular biology of anal cancer will inform future strategies for targeted therapy, and biomarkers such as p16 and p53 may emerge as important prognostic indicators. Anal low-grade or high-grade squamous intraepithelial lesion histologies are particularly prevalent in at-risk populations, and surgical oncologists should understand the principles that underlie surveillance and management strategies. Finally, the role of chemoradiation and salvage abdominoperineal resection and the options for systemic therapy in the setting of metastatic disease are outlined. 

      This review contains 10 figures, 5 tables, and 77 references.

      Key Words: Anal cancer, Anal HSIL, Anal LSIL, Anal margin cancer, Anal squamous cell, carcinoma, HPV, p16, Perianal cancer

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    • 9

      Systemic Therapy for Colorectal Cancer

      By Anna M. Varghese
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      Systemic Therapy for Colorectal Cancer

      • ANNA M. VARGHESEAssistant Attending, Gastrointestinal Oncology Service, Division of Solid Tumor Oncology, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, NY

      Colorectal cancer remains the second most common cause of cancer-related deaths in this country. Although colorectal cancer is best managed by a multidisciplinary team of surgical, radiation, and medical oncologists, cytotoxic therapy remains the backbone of treatment in the metastatic disease setting. In addition to cytotoxic therapies, vascular-targeted therapies and epidermal growth factor receptor (EGFR)–targeted therapies for selected patients with metastatic colorectal cancer improve outcomes for patients with metastatic colorectal cancer. Growing understanding of various biological subsets of colorectal cancer, including BRAF V600E mutant and mismatch repair–deficient colorectal cancers, is expanding treatment opportunities for patients and is the focus of ongoing research.

      This review contains 4 tables and 57 references.

      Key Words: chemotherapy, colorectal cancer, fluoropyrimidines, immunotherapy, metastatic, mismatch repair–deficient colorectal cancer, targeted therapy, topoisomerase inhibitors

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    • 10

      Radiographic Staging of Colorectal Cancer

      By Maurits P. Engbersen; Max J. Lahaye; Regina G.H. Beets-Tan
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      Radiographic Staging of Colorectal Cancer

      • MAURITS P. ENGBERSENDepartment of Radiology, The Netherlands Cancer Institute, Antoni van Leeuwenhoek Hospital, Amsterdam, The Netherlands
      • MAX J. LAHAYEDepartment of Radiology, The Netherlands Cancer Institute, Antoni van Leeuwenhoek Hospital, Amsterdam, The Netherlands
      • REGINA G.H. BEETS-TANDepartment of Radiology, The Netherlands Cancer Institute, Antoni van Leeuwenhoek Hospital, Amsterdam, The Netherlands

      Imaging increasingly plays an important role in selecting the most optimal treatment for patients with colon and rectal cancer. While in colon cancer, computed tomography (CT) remains the modality of choice for local and distant staging, in patients with rectal cancer magnetic resonance imaging (MRI) is the main modality and mandatory for local staging. Endoluminal rectal ultrasound (ERUS) is the preferred staging method for superficial rectal tumors. This chapter addresses the current role of various imaging modalities in colorectal tumor staging.

      This review contains 4 figures and 50 references.

      Key words: Preoperative imaging, Colorectal cancer, Magnetic resonance imaging, Diffusion weighted MRI, Computed tomography, Mesorectal fascia, TNM staging, Treatment stratification

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  • Endocrine Cancer
    • 1

      Differentiated Thyroid Cancer

      By Rebecca Tuttle, MD; William Cance, MD; James Howe, MD
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      Differentiated Thyroid Cancer

      • REBECCA TUTTLE, MDWright State University Department of Surgery, Division of Surgical Oncology
      • WILLIAM CANCE, MDRoswell Park Cancer Institute, Department of Surgical Oncology
      • JAMES HOWE, MDUniversity of Iowa Carver College of Medicine, Division of Surgical Oncology and Endocrine Surgery

      Differentiated thyroid cancer is a common malignancy with an excellent prognosis. Patients typically present with a thyroid nodule identified on physical exam or imaging. Fine needle aspiration (FNA) is the diagnostic modality of choice; ultrasound of the neck is used preoperatively to evaluate lymphadenopathy. Surgery is the mainstay of treatment, with partial or total thyroidectomy (with or without lymphadenectomy). Intra-operatively, identification of the recurrent laryngeal nerve and preservation of the parathyroid glands is imperative. Postoperatively, patients are considered for adjuvant radioactive iodine ablation. Risk stratification systems are available to assist patient selection for therapy. Surveillance is completed with serial physical exams, laboratory studies, ultrasound, and radioactive iodine scanning. Recurrence can be managed with surgery, thyroid-stimulating hormone suppression, radioactive iodine ablation, radiation, or kinase inhibitors. 

      Key Words: Bethesda classification; Differentiated thyroid cancer; Follicular thyroid cancer; Papillary thyroid cancer; Radioactive Iodine; Thyroid nodule; Thyroidectomy

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    • 2

      Medullary Thyroid Cancer

      By Geeta Lal, MD, MSc, FRCS(C), FACS
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      Medullary Thyroid Cancer

      • GEETA LAL, MD, MSC, FRCS(C), FACSAssociate Professor, Surgery, University of Iowa, Iowa City, IA

      Medullary thyroid cancer (MTC) arises from the thyroid C-cells and accounts for 1 to 2% of thyroid cancers in the United States. Most tumors are sporadic but may occur as a part of the familial syndromes multiple endocrine neoplasia (MEN) 2A, MEN2B, and familial MTC. Surgery is the mainstay of treatment of these tumors, although recent advances in molecular genetics have enabled the development and use of targeted therapies such as tyrosine kinase inhibitors to treat patients with symptomatic metastatic disease.

       This review contains 2 figures, 3 tables and 34 references

      Key Words: genetics, management, medullary thyroid cancer, MEN2A, MEN2B, targeted therapies

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    • 3

      Anaplastic Thyroid Cancer

      By Geeta Lal, MD, MSc, FRCS(C), FACS
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      Anaplastic Thyroid Cancer

      • GEETA LAL, MD, MSC, FRCS(C), FACSAssociate Professor, Surgery, University of Iowa, Iowa City, IA

      Anaplastic thyroid cancer (ATC) is a rare thyroid malignancy with a nearly uniform poor prognosis. Most patients present with advanced disease, and optimal management requires rapid diagnosis, staging, and involvement of multidisciplinary teams. Treatment may include surgery in patients with resectable disease and adjuvant or neoadjuvant radiotherapy and chemotherapy. Improved understanding of molecular pathogenesis has allowed the assessment of tyrosine kinase inhibitors and other targeted treatments in these patients.  The FDA recently approved the combination of dabrafenib (BRAF inhibitor) and trametinib (MEK inhibitor) for the treatment of BRAF V600E mutation positive, unresectable or metastatic ATC. This review summarizes the current state-of-the-art concepts in the management of patients with ATC.

      This review contains 3 figures, 2 tables, and 25 references.

      Key words: anaplastic thyroid cancer, goals of care discussion, management, surgery, radiotherapy, chemotherapy novel therapies, NCCN and ATA guidelines

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    • 4

      Operative Approach to Primary Hyperparathyroidism

      By Anna C Beck, MD
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      Operative Approach to Primary Hyperparathyroidism

      • ANNA C BECK, MDDepartment of Surgery, University of Iowa Hospitals and Clinics, Iowa City, IA, and Sonia L Sugg,1 MD, Department of Surgery, University of Iowa Hospitals and Clinics, Iowa City, IA

      We review the key components of the embryology and anatomy of the parathyroid glands. Here, minimally invasive parathyroidectomy with intraoperative adjuncts such as intraoperative parathyroid hormone monitoring, four-gland exploration, subtotal parathyroidectomy, total parathyroidectomy with autotransplantation, and cyropreservation are described. The incidence of postoperative complications and the technical aspects of parathyroid surgery are delineated.

      This review contains 3 figures, 1 tables, and 17 references.

      Key Words: cryopreservation, hyperparathyroidism, minimally invasive parathyroidectomy parathyroidectomy, pharyngeal pouch, recurrent laryngeal nerve, subtotal parathyroidectomy, total parathyroidectomy


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    • 5

      Secondary Hyperparathyroidism

      By Anna C Beck, MD; Sonia L Sugg, MD
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      Secondary Hyperparathyroidism

      • ANNA C BECK, MDDepartment of Surgery, University of Iowa Hospitals and Clinics, Iowa City, IA, and Sonia L Sugg,1 MD, Department of Surgery, University of Iowa Hospitals and Clinics, Iowa City, IA
      • SONIA L SUGG, MDDepartment of Surgery, University of Iowa Hospitals and Clinics, Iowa City, IA

      Secondary hyperparathyroidism is defined and its pathophysiology, delineated. Key components of the diagnostic work-up, medical management, and indications for surgery are described. The operative approach and controversy on extent of parathyroidectomy are discussed.

      This review contains 3 figures, 1 tables, and 24 references. 

      Key Words: autotransplantation, calciphylaxis, chronic renal failure, cryopreservation, hungry bone syndrome, hypocalcemia, secondary hyperparathyroidism, subtotal parathyroidectomy, total parathyroidectomy

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    • 6

      Tertiary Hyperparathyroidism

      By Anna C Beck, MD; Sonia L Sugg, MD
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      Tertiary Hyperparathyroidism

      • ANNA C BECK, MDDepartment of Surgery, University of Iowa Hospitals and Clinics, Iowa City, IA, and Sonia L Sugg,1 MD, Department of Surgery, University of Iowa Hospitals and Clinics, Iowa City, IA
      • SONIA L SUGG, MDDepartment of Surgery, University of Iowa Hospitals and Clinics, Iowa City, IA

      The definition, pathophysiology, and diagnosis of tertiary hyperparathyroidism is reviewed. Parathyroidectomy is the mainstay of management, and the indications and timing of surgery are explained. The operative approach is described.

       This review contains 1 figure, 1 table, and 19 references.

      Key Words: cinacalcet, hungry bone syndrome, parathyroid adenoma, parathyroidectomy, parathyroid hyperplasia, renal transplantation, tertiary hyperparathyroidism

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    • 7

      Primary Hyperparathyroidism

      By Anna C Beck, MD; Sonia L Sugg, MD
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      Primary Hyperparathyroidism

      • ANNA C BECK, MDDepartment of Surgery, University of Iowa Hospitals and Clinics, Iowa City, IA, and Sonia L Sugg,1 MD, Department of Surgery, University of Iowa Hospitals and Clinics, Iowa City, IA
      • SONIA L SUGG, MDDepartment of Surgery, University of Iowa Hospitals and Clinics, Iowa City, IA

      Primary hyperparathyroidism is defined. Key components of the diagnostic work-up including symptoms, family history, laboratory examination, and imaging techniques are reviewed. Indications for surgery, including a discussion of the latest guidelines, are emphasized. Controversies such as when to operate on asymptomatic primary hyperparathyroidism are discussed.

      This review contains 4 figures, 1 table, and 29 references.

      Key Words: hypercalcemia, hypercalcemic crisis, intraoperative parathyroid hormone monitoring, multiple endocrine neoplasia, normocalcemic primary hyperparathyroidism, parathyroid adenoma, parathyroid hyperplasia, primary hyperparathyroidism, sestamibi scan

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    • 8

      Recurrent and Persistent Hyperparathyroidism

      By Anna C Beck, MD
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      Recurrent and Persistent Hyperparathyroidism

      • ANNA C BECK, MDDepartment of Surgery, University of Iowa Hospitals and Clinics, Iowa City, IA, and Sonia L Sugg,1 MD, Department of Surgery, University of Iowa Hospitals and Clinics, Iowa City, IA

      Persistent and recurrent hyperparathyroidism is defined. Key components of preoperative planning including imaging modalities are discussed. Indications for surgery and emerging techniques to aid identification of abnormal parathyroid tissue in the reoperative neck are described.

      This review contains 2 figures, 2 tables and 22 references

      Key Words: indocyanine green fluorescence angiography, methylene blue injection, near-infrared autofluorescence imaging, parathyroid cryopreservation, parathyromatosis, persistent hyperparathyroidism, recurrent hyperparathyroidism, selective parathyroid hormone venous sampling

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    • 9

      Parathyroid Carcinoma

      By Anna C Beck, MD; Sonia L Sugg, MD
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      Parathyroid Carcinoma

      • ANNA C BECK, MDDepartment of Surgery, University of Iowa Hospitals and Clinics, Iowa City, IA, and Sonia L Sugg,1 MD, Department of Surgery, University of Iowa Hospitals and Clinics, Iowa City, IA
      • SONIA L SUGG, MDDepartment of Surgery, University of Iowa Hospitals and Clinics, Iowa City, IA

      The clinical presentation and genetic mutations associated with parathyroid cancer are reviewed. Surgery, other treatment modalities, and outcomes are discussed.

      This review contains 1 table and 19 references.

      Key Words: CDC73-related parathyroid carcinoma, denosumab, HRPT2 germline mutation, hyperparthyroidism-jaw tumor syndrome, parathyroid carcinoma

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    • 10

      Diseases of the Adrenal Gland and Adrenalectomy

      By Quan-Yang Duh, MD; Frederick Thurston Drake, MD, MPH
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      Diseases of the Adrenal Gland and Adrenalectomy

      • QUAN-YANG DUH, MDProfessor of Surgery, University of California, San Francisco, CA
      • FREDERICK THURSTON DRAKE, MD, MPHAssistant Professor of Surgery, Department of Surgery, Boston University, Boston, MA, Boston Medical Center, Boston, MA

      Evaluation and treatment of adrenal disease encompasses a broad range of disorders, including pheochromocytoma, primary hypercortisolism, primary aldosteronism, and malignancies such as adrenocortical carcinoma and metastases from other primary cancers. This pathophysiologic diversity and the technical challenges of operating on these bilateral organs with the attendant differences in left and right anatomy make adrenal surgery a complex but rewarding endeavor. Adrenal surgeons must be engaged in the physiologic aspects of the disease being treated, the anatomic details of lesion and laterality, and the technical considerations in selecting an operative approach and carrying it out successfully. Guidelines for the management of pheochromocytoma, primary hypercortisolism, and primary aldosteronism are reviewed in this chapter. Consensus is emerging that resection of adrenal metastases from other primary cancers can prolong survival in well-selected patients and that most are suitable for a laparoscopic approach. Most adrenal surgeons agree that large or locally aggressive adrenocortical carcinomas must be resected in an open fashion, though there is some controversy surrounding the operative approach in the setting of small or indeterminate lesions. Minimally invasive techniques have revolutionized adrenal surgery, and surgeons who wish to incorporate adrenal surgery into their practice must master either the laparoscopic or the retroperitoneoscopic approach.

      This review contains 8 figures, 2 tables, and 28 references.

      Key Words: adrenal cortex, adrenalectomy, adrenal incidentaloma, adrenal medulla, adrenocortical cancer, Cushing syndrome, laparoscopic adrenalectomy, pheochromocytoma, primary aldosteronism, retroperitoneoscopic adrenalectomy

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  • Esophageal Cancer
    • 1

      Esophageal Adenocarcinoma

      By Steven N Hochwald, MD, FACS; Rupen Shah, MD
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      Esophageal Adenocarcinoma

      • STEVEN N HOCHWALD, MD, FACSProfessor of Oncology Vice-Chair and Chief of GI Surgery Department of Surgical Oncology Roswell Park Cancer Institute Buffalo, NY
      • RUPEN SHAH, MDDepartment of Surgical Oncology Roswell Park Cancer Institute Buffalo, NY

      Esophageal adenocarcinomas are characterized by advanced stages of disease at presentation and poor survival rates. There is an increased use of multimodal therapy, including neoadjuvant chemotherapy and radiation treatment followed by surgery for locoregional disease. There has been a strong recent emphasis on optimizing morbidity and mortality following surgical therapy. The implementation of nomograms and treatment pathways has improved our ability to predict outcomes and standardize care. Large single-institution retrospective studies demonstrate excellent results with minimally invasive Ivor Lewis and three-field esophagectomy. A recent randomized study supported the use of minimally invasive surgery for the management of esophageal adenocarcinoma.

      Keywords imaging, staging, multimodality therapy, surgical approaches, and controversies in the management of esophageal adenocarcinoma.

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    • 2

      Imaging and Staging of Esophageal Malignancy

      By Steven N Hochwald, MD, FACS; Rupen Shah, MD
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      Imaging and Staging of Esophageal Malignancy

      • STEVEN N HOCHWALD, MD, FACSProfessor of Oncology Vice-Chair and Chief of GI Surgery Department of Surgical Oncology Roswell Park Cancer Institute Buffalo, NY
      • RUPEN SHAH, MDDepartment of Surgical Oncology Roswell Park Cancer Institute Buffalo, NY

      Squamous cell carcinoma (SCC) and adenocarcinoma are the two main cell types of esophageal cancer. Esophageal adenocarcinoma is rapidly increasing in incidence in Western countries, particular in elderly white males. Histopathologic cell type affects the survival of clinically and pathologically staged patients, but less so pathologically staged patients following neoadjuvant therapy. The survival of early- and intermediate-stage patients with SCC is worse than for those with similarly staged adenocarcinoma. Unfortunately, patients often present with either cell type of esophageal carcinoma at an advanced stage. Various imaging modalities are necessary to adequately stage patients with esophageal cancer due to the length of the esophagus and patterns of spread that frequently involve the neck, thorax, and abdomen. Despite advances in imaging, the accuracy of clinical staging is limited, which results in different survival profiles for clinical stage groups compared with pathologic stage groups. Since clinical staging based on imaging remains unpredictable and inaccurate, the eighth edition of the AJCC Cancer Staging Manual has now expanded staging to allow for three different opportunities. Separate classifications now include clinical (cTNM), pathologic (pTNM), and postneoadjuvant pathologic (ypTNM) staging. It is hoped that the use of these three stage groups will improve our ability to provide precise care to patients with esophageal cancer.

      This review contains 2 figures, 9 tables, and 23 references.

      Key words: Esophageal cancer, Esophagography, Endoscopic ultrasonography, Invasive esophageal cancer, Staging of esophageal cancer, Gastroesophageal junction carcinoma

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    • 3

      A Standardized Clinical Pathway Approach to Esophageal Cancer

      By Andrea Wirsching, MD, MSc; Donald E Low, FACS, FRCSC, FRCSI (Hon), FRCSE (Hon)
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      A Standardized Clinical Pathway Approach to Esophageal Cancer

      • ANDREA WIRSCHING, MD, MSCThoracoesophageal Research Fellow, Department of General, Vascular and Thoracic Surgery, Virginia Mason Medical Center, Seattle, WA
      • DONALD E LOW, FACS, FRCSC, FRCSI (HON), FRCSE (HON)Head of Thoracic Surgery and Thoracic Oncology, Department of General, Vascular and Thoracic Surgery, Director, Digestive Disease Institute Esophageal Center of Excellence, Virginia Mason Medical Center, Seattle, WA

      Clinical pathways associated with the surgical treatment of esophageal cancer patients represent an important development to maximize the opportunity for clinical and cost efficiency in patient care. The main goals implemented in standardized clinical pathways are a comprehensive preoperative workup and tumor board presentation; anesthesia management dedicated to enable enhanced recovery and standardized steps of postoperative recovery, including early mobilization; efficient removal of tubes, catheters, and lines; and early enteral feedings. Recent studies report a decreased length of hospital and intensive care unit stay and decreased costs associated with the routine application of these pathways. Some reports have also shown a decrease in postoperative morbidity and mortality related to the implementation and refinement of standardized clinical pathways. The present review is dedicated to all aspects of standardized clinical pathways for esophagectomy and aims to give an insight into key components of the clinical pathway, which have evolved over the last 20 years at our institution.  

      This review contains 3 figures, 8 tables, and 33 tables.

      Key words: clinical pathway, costs, enhanced recovery, esophageal cancer, esophagectomy, hospital stay, implementation, length of stay, outcome, standardized pathway

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    • 4

      Squamous Cell Carcinoma of the Esophagus

      By Kyle G Mitchell, MD; David B Nelson, MD; Wayne L Hofstetter, MD
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      Squamous Cell Carcinoma of the Esophagus

      • KYLE G MITCHELL, MDResearch Fellow, Department of Thoracic and Cardiovascular Surgery, University of Texas MD Anderson Cancer Center, Houston, Texas
      • DAVID B NELSON, MDResearch Fellow, Department of Thoracic and Cardiovascular Surgery, University of Texas MD Anderson Cancer Center, Houston, Texas
      • WAYNE L HOFSTETTER, MDProfessor, Department of Thoracic and Cardiovascular Surgery, University of Texas MD Anderson Cancer Center, Houston, Texas

      Although its incidence in the Western world is decreasing, squamous cell carcinoma of the esophagus (ESCC) remains a significant cause of cancer mortality worldwide. Recent analyses have identified a number of unique characteristics that differentiate ESCC from esophageal adenocarcinoma at the etiologic, genomic, and epigenetic levels. A thorough diagnostic and staging work-up includes endoscopy, endoscopic ultrasonography, and axial imaging. Applicable therapeutic modalities include definitive endoscopic therapies, resection alone, trimodality therapy, definitive chemoradiation, systemic therapy, and palliative treatment options. Given the complexity of this disease and its management, careful consideration in a multidisciplinary setting is warranted to appropriately individualize therapeutic options.

      This review contains 5 figures, 6 tables, and 51 references.

      Key Words: squamous cell carcinoma, esophageal diseases, esophageal cancer, esophageal neoplasms, gastroesophageal junction, Tylosis, bimodality therapy, trimodality therapy, endoscopic therapy

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    • 5

      Nonepithelial Esophageal Tumors: Leiomyomas and GI Stromal Tumors

      By Urs von Holzen, MD, MBA, FACS; Daniel C Wiener, MD; Jon O Wee, MD
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      Nonepithelial Esophageal Tumors: Leiomyomas and GI Stromal Tumors

      • URS VON HOLZEN, MD, MBA, FACSAssociate Professor of Surgery, Indiana University School of Medicine South Bend, Director of Surgical Oncology, Goshen Center for Cancer Care, Goshen, IN
      • DANIEL C WIENER, MDInstructor in Surgery, Harvard Medical School, Brigham and Women’s Hospital, Boston, MA
      • JON O WEE, MDAssistant Professor in Surgery, Harvard Medical School, Section Chief, Esophageal Surgery, Co-Director, Minimally Invasive Thoracic Surgery, Director, Robotics in Thoracic Surgery, Brigham and Women’s Hospital, Boston, MA

      Nonepithelial esophageal tumors can be classified into two groups. The most common group consists of GI stromal tumors (GISTs). The less common group consists of several tumors similar to tumors arising from soft tissues of other body parts (eg, lipomas, liposarcomas, leiomyomas, leiomyosarcomas, peripheral nerve sheath tumors). Differentiation between these two groups is based on the near-universal expression of CD117 in GISTs, as opposed to other spindle cell tumors. Although some of these tumors are asymptomatic and discovered accidentally during CT or endoscopies for other reasons, they often cause some unspecific symptoms (eg, bloating, dysphagia, early satiety). Contrast-enhanced CT with IV and oral contrast is the examination of choice to evaluate these tumors, as extent of the tumor and presence of metastatic disease can be determined. Surgical resection is generally recommended for tumors larger than 2 cm or symptomatic tumors.


      This review contains 4 figures, 2 tables, and 35 references.

      Key Words: GIST, leiomyoma, leiomyosarcoma, nonepithelial esophageal tumors, esophagus, diagnosis, treatment, follow-up

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    • 6

      Endoscopic Management of Barrett’s Esophagus and Early Esophageal Neoplasia

      By Amanpal Singh, MD, MS; Andrew J Bain, MD; Ramon Rivera, MD
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      Endoscopic Management of Barrett’s Esophagus and Early Esophageal Neoplasia

      • AMANPAL SINGH, MD, MSMercy Medical Group, Sacramento, CA
      • ANDREW J BAIN, MDRoswell Park Cancer Institute, Buffalo, NY
      • RAMON RIVERA, MDUniversity at Buffalo, Buffalo, NY

      Gastroesophageal reflux disease is common in North America, affecting around 25% of the population. A small fraction of these patients develop Barrett esophagus and thus are at a risk of developing esophageal cancer. The progression of Barrett esophagus to esophageal cancer can be prevented with timely examinations and, when necessary, by performing treatments to eradicate it. This review describes the use of endoscopy techniques to diagnose and classify Barrett esophagus among high-risk patients and the use of ablative techniques in patients with dysplasia. The review also describes endoscopic resection techniques for early esophageal cancer, which can provide staging information as well as can be therapeutic in selected cases.

      This review contains 8 figures and 49 references.

      Key Words: acid reflux, Barrett esophagus, cryoablation, endoscopic mucosal resection, endoscopic submucosal dissection, esophageal cancer, esophagus ablation, GERD, radiofrequency ablation

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  • Gastrointestinal Cancer
    • 1

      Endoscopic Management of Polyps and Early Malignancy

      By Nadim Mahmud, MD, MS, MPH; Tyler Berzin, MD, MS
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      Endoscopic Management of Polyps and Early Malignancy

      • NADIM MAHMUD, MD, MS, MPHResident, Department of Internal Medicine, Brigham and Women’s Hospital, Boston, MA
      • TYLER BERZIN, MD, MSAssistant Professor of Medicine, Center for Advanced Endoscopy, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA

      Gastrointestinal endoscopy plays a critical role in the detection, diagnosis, and management of premalignant and malignant conditions. Because of the diversity of polypoid and nonpolypoid abnormalities that may be encountered throughout the gastrointestinal tract, the modern endoscopist must be keenly aware of the differential diagnosis of these lesions, the appropriateness of biopsy or resection, and the subsequent management based on pathology or staging information. A variety of tissue sampling and resection techniques have been developed for this purpose, including forceps biopsy, snare polypectomy, endoscopic mucosal resection, and endoscopic submucosal dissection. Depending on the lesion encountered, these techniques may be both diagnostic and therapeutic. Here we review the endoscopic management of premalignant polyps and early malignancy that may be encountered at different locations in the gastrointestinal tract.


      This review contains 17 figures, 5 tables and 47 references

      Key words: ablation, adenocarcinoma, adenoma, Barrett esophagus, biopsy, endoscopic mucosal resection, endoscopic submucosal dissection, forceps, polypectomy, snare

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    • 2

      Extent of Gastrectomy and Lymphadenectomy for Gastric Adenocarcinoma

      By Sam S. Yoon, MD; Kevin K. Chang, MD
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      Extent of Gastrectomy and Lymphadenectomy for Gastric Adenocarcinoma

      • SAM S. YOON, MDAssociate Attending Surgeon, Gastric and Mixed Tumor Service, Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, NY
      • KEVIN K. CHANG, MDResearch Fellow, Gastric and Mixed Tumor Service, Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, NY

      Gastric adenocarcinoma is one of the most common and lethal cancers worldwide and is associated with a high frequency of nodal metastasis. The value of multimodality therapy is well established, but gastric resection and locoregional lymph node dissection are important mainstays in potentially curative therapy. However, there has been considerable regional variation in surgical approach and debate regarding the ideal extent of gastric resection, gastric reconstruction, and extent of lymphadenectomy. This review outlines the current evidence in the surgical management of gastric adenocarcinoma. The advent of minimally invasive approaches to gastric operations is also discussed.

      This review contains 4 figures, 7 tables, and 50 references.

      Key words: gastrectomy, gastric cancer, lymphadenectomy, lymph node, lymph node staging, stomach

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    • 3

      Imaging and Staging for Stomach Malignancy

      By Moamen M Gabr, MD, MSc; Tyler M Berzin, MD, MS, FASGE
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      Imaging and Staging for Stomach Malignancy

      • MOAMEN M GABR, MD, MSCDigestive Diseases and Nutrition, University of Kentucky, Lexington, KY
      • TYLER M BERZIN, MD, MS, FASGEGastroenterology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA

      Therapeutic decisions regarding surgical, endoscopic, and medical management of gastric tumors depends on tumor type, anatomic location of the tumor, and, in the setting of malignant tumors, the clinical staging of the lesion. Common types of stomach tumors include adenocarcinoma, which accounts of 90% of stomach malignancies, lymphomas, gastrointestinal stromal tumors (GISTs), and neuroendocrine tumors. A detailed physical examination during initial evaluation may help target the approach during malignancy staging. Endoscopy offers a large armamentarium of tools for diagnosis and staging of gastric tumors. EUS is currently the mainstay in local staging and obtaining tissue diagnosis of primary tumor and nodal metastasis. Advances in imaging modalities have provided precise tools for diagnosis and staging of stomach malignancies.

      This review contains 11 figures, 1 table, and 35 references.

      Key Words: endoscopy, endoscopic ultrasonography, gastric malignancy, GIST, MALT lymphoma, neuroendocrine tumors, staging

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    • 4

      Gastric Carcinoids and Polyps

      By George Z. Li, MD; Thomas E. Clancy, MD
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      Gastric Carcinoids and Polyps

      • GEORGE Z. LI, MDSenior Resident in General Surgery, Department of Surgery, Brigham and Women’s Hospital, Boston, MA
      • THOMAS E. CLANCY, MDAssociate Chief, Division of Surgical Oncology, Department of Surgery, Brigham and Women’s Hospital, Boston, MA

      Gastric polyps are commonly found on upper endoscopy. With the increasing use of proton pump inhibitors and decreasing incidence of Helicobacter pylori infection, fundic gland polyps are now the most common gastric polyps in Western countries. Most of the other polyps, such as hyperplastic polyps, gastric adenomas, and gastric neuroendocrine tumors (NETs), are strongly associated with the presence of chronic atrophic gastritis, commonly due to either H. pylori infection or autoimmune gastritis. Gastric NETs, previously termed carcinoids, are rare neoplasms that often present as polypoid lesions and can be subcategorized into three subtypes. The most common subtype, type 1, is associated with chronic atrophic gastritis and generally thought to have low malignant potential. Type 2 NETs behave similarly to type I NETs but are specifically associated with the Zollinger-Ellison syndrome. Type 3 NETs are sporadic and highly malignant. All gastric polypoid lesions require histopathologic examination for diagnosis. The key aspect to the management of gastric NETs and other gastric polyps is to determine the malignant potential of the lesion in question. This then informs whether the patient needs removal of additional polyps if multiple, whether the patient needs further endoscopic surveillance, or whether surgery is indicated.

      This review contains 5 figures, 5 tables, and 50 references.

      Key Words: atrophic gastritis, fundic gland polyp, gastric adenoma, gastric carcinoid, gastric neuroendocrine tumor, gastric polyp, hyperplastic polyp, intestinal metaplasia

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  • Soft Tissue Cancer
    • 1

      Management of Recurrent Retroperitoneal Sarcoma

      By Armen Parsyan, MD, PhD, DSc, MPH, FRCSC; Abha Gupta, MD, MSc, FRCPC; Charles Catton, MD, FRCPC; Rebecca Gladdy, MD, PhD, FRCSC
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      Management of Recurrent Retroperitoneal Sarcoma

      • ARMEN PARSYAN, MD, PHD, DSC, MPH, FRCSCDepartment of Surgical Oncology, Princess Margaret Cancer Centre;, Department of Surgery, Mount Sinai Hospital, University of Toronto, Toronto, ON
      • ABHA GUPTA, MD, MSC, FRCPCAssociate Professor of Pediatrics, Division of Pediatric Hematology/Oncology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto; Department of Medical Oncology, Princess Margaret Cancer Centre, Toronto, ON
      • CHARLES CATTON, MD, FRCPCProfessor of Radiation Oncology, Department of Radiation Oncology, University of Toronto; Radiation Medicine Program, Princess Margaret Cancer Centre, Toronto, ON
      • REBECCA GLADDY, MD, PHD, FRCSCAssistant Professor of Surgery, Department of Surgery, Mount Sinai Hospital; Department of Surgical Oncology, Princess Margaret Cancer Centre; Assistant Professor of Surgery, Department of Surgery, Mount Sinai Hospital, University of Toronto, Toronto, ON

      Retroperitoneal sarcomas (RPSs) are a heterogeneous group of mesenchymal tumors. The mainstay of treatment of RPS is curative surgical resection, which often involves a multivisceral resection. The predominant pattern of failure in RPS is local recurrence, which poses significant management challenges and limits survival. There is a paucity of high-level evidence to guide the management of primary and recurrent RPS, mainly due to the rarity of the disease. This review highlights the challenges and decision making in the diagnosis and management of recurrent RPS. 

      Key words: chemotherapy, distant recurrence, leiomyosarcoma, liposarcoma, local recurrence, radiation therapy, retroperitoneal sarcoma, surgical resection 

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    • 2

      Management of Metastatic Sarcoma

      By Ayana Allard-Picou, MD; Steven C Katz, MD, FACS
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      Management of Metastatic Sarcoma

      • AYANA ALLARD-PICOU, MDSurgical Oncology Fellow, Department of Surgery, Roger Williams Medical Center, Providence, RI
      • STEVEN C KATZ, MD, FACSDivision of Surgical Oncology, Department of Surgery, Roger Williams Medical Center, Providence, RI; Department of Surgery, Boston University School of Medicine, Boston, MA

      Approximately 12,310 soft tissue sarcomas (STSs) were diagnosed in 2016. These tumors originate from mesenchymal cells throughout the body and account for less than 1% of all newly diagnosed cancers in adults. STSs comprise a heterogeneous group of tumors, with over 50 different histologic subtypes categorized based on the presumptive tissue of origin, each with varying individual characteristics and varying patterns of behavior. Most commonly, they originate in the extremity followed by the trunk as the second most common site of primary lesions. The likelihood of metastatic spread is highly variable among histologic subtype and primary disease site. Distant hematogenous metastases occur in up to 50% of patients with high-grade sarcomas but in only 5% of patients with low-grade sarcomas. Most metastases occur within 2 years following initial diagnosis. The risk of metastasis is dependent on several prognostic factors, including tumor depth, tumor size, and mitotic rate. The role of surgery in the management of metastatic disease is governed by careful patient selection in the context of a multidisciplinary plan including systemic treatment. Selection of patients for resection of sarcoma metastases is most appropriately based on consideration of factors associated with long-term survival. We review the role of surgery in the management of STS metastases, rational selection of patients for resection, and strategies for integrating surgery into multidisciplinary treatment planning for patients with metastatic STS. 

      This review contains 3 figures, 3 tables and 60 references

      Key words: gastrointestinal stromal tumor, hepatic sarcoma metastases, metastatic sarcoma, pulmonary sarcoma metastases, retroperitoneal sarcoma, sarcoma, soft tissue sarcoma, surgical resection of sarcoma metastases

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    • 3

      Dermatofibrosarcoma Protuberans

      By Benjamin Deschner, MD; Jeffrey Wayne, MD, FACS
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      Dermatofibrosarcoma Protuberans

      • BENJAMIN DESCHNER, MDResident physician at Northwestern Memorial Hospital, Chicago, IL.
      • JEFFREY WAYNE, MD, FACSProfessor of surgery and dermatology, chief of melanoma and soft tissue surgical oncology, and associate director for clinical affairs at the Robert H. Lurie Comprehensive Cancer Center, Northwestern University Feinberg School of Medicine, and chief of staff of Northwestern Memorial Hospital, Chicago, IL.

      Dermatofibrosarcoma protuberans (DFSP) is a rare malignant cutaneous tumor with an annual incidence of 4.2 per million. The tumor typically grows slowly and has a proclivity toward local invasiveness and away from distant metastasis. The mainstay of treatment is complete surgical resection, which is curative in almost all cases. The tumor’s characteristics make this difficult, however, and reexcision to negative margins can be challenging. DFSP can also present with or develop fibrosarcomatous change (DFSP-FS), a more aggressive variant. Thus, these tumors require thoughtful management. In this review, we discuss the biologic background of DFSP, describe the tumor’s behavior and presentation, and discuss current concepts in management.

      This review contains 5 figures, 4 tables and 28 references

      Key words: dermatofibrosarcoma protuberans, fibrosarcomatous change, imatinib, local recurrence, micrographic surgery, nonmelanoma, radiation, sarcoma, skin cancer, surgical excision

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    • 4

      Pathologic Evaluation of Soft Tissue Sarcoma

      By Narasimhan P. Agaram, MBBS
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      Pathologic Evaluation of Soft Tissue Sarcoma

      • NARASIMHAN P. AGARAM, MBBSAssociate Attending Pathologist, Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY

      Soft tissue sarcomas are rare tumors and are mostly managed in tertiary care centers. They are broadly classified based on their differentiation into multiple different types. They affect patients of all ages and mostly occur in the extremities. Pathologic examination of the biopsy or resection of the tumor is an extremely important aspect in the diagnosis of these tumors and appropriately guiding the clinical team in the management of these rare tumors. This review focuses on the aspects of pathologic evaluation of these tumors and discusses the morphologic aspects of the predominant soft tissue sarcomas based on differentiation. The review also highlights the latest molecular diagnostic studies that are used in the accurate subtyping of these tumors. 

      This review contains 12 figures, 2 tables, and 28 references.

      Key words: angiosarcoma, fibromatosis, fibrous, liposarcoma, myxoid, nerve sheath tumor, sarcoma, synovial sarcoma

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    • 5

      Surgical Management of Extremity and Truncal Sarcoma

      By Christina L Roland, MD; Janice N Cormier, MD, MPH
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      Surgical Management of Extremity and Truncal Sarcoma

      • CHRISTINA L ROLAND, MDAssistant Professor, Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX
      • JANICE N CORMIER, MD, MPHProfessor, Departments of Surgical Oncology and Biostatistics and Biomathematics, The University of Texas MD Anderson Cancer Center, Houston, TX

      Soft tissue sarcomas are a heterogeneous group of neoplasms that arise throughout the body, but most commonly in the extremity and trunk. A multidisciplinary treatment approach has resulted in local control rates exceeding 90% and 5-year survival rates exceeding 70%. For patients with localized soft tissue sarcoma, limb-sparing surgical resection is the mainstay of therapy, with radiation therapy and chemotherapy used as adjuncts to reduce local and distant recurrences. Amputation is reserved for patients with primary or recurrent tumors that cannot be grossly resected with a limb-sparing procedure and preservation of function, which should occur in less than 5% of patients. Most patients who die of soft tissue sarcoma die of metastatic disease, which becomes evident within 2 to 3 years of initial diagnosis in 80% of cases, highlighting the need for improved systemic therapy options and optimal treatment at the time of diagnosis.

      This review contains 13 figures, 4 tables and 53 references

      Key words: amputation, multimodality, radiation therapy, soft tissue sarcoma, staging, surgery, treatment, wide resection

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    • 6

      Principles in Reconstruction After Resection of Extremity and Truncal Sarcomas

      By Jonas A. Nelson, MD; Evan Matros, MD, MPH
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      Principles in Reconstruction After Resection of Extremity and Truncal Sarcomas

      • JONAS A. NELSON, MDAssistant Professor, Plastic and Reconstructive Surgery Service, Memorial Sloan Kettering Cancer Center, New York, NY
      • EVAN MATROS, MD, MPHAssistant Professor, Plastic and Reconstructive Surgery Service, Memorial Sloan Kettering Cancer Center, New York, NY

      Reconstruction of sarcoma defects of the trunk or extremity requires detailed understanding of a variety of techniques for wound closure. Advances in reconstruction and oncologic treatment have led to higher rates of limb salvage in patients who would have previously undergone amputation. Such defects often require consideration of the reconstructive ladder, moving from the most simplistic closure to free flaps. As previous surgeries or radiation therapy can create a challenging wound environment with scar and fibrosed tissues, at times the more complex up-front option will be a better long-term solution for a patient following sarcoma resection. A multidisciplinary approach to the initial management, resection, and reconstruction is certainly warranted to attain the best overall outcome for the patient from the oncologic and aesthetic standpoint. This review provides an overview of the reconstructive techniques employed in addressing defects of the trunk and extremities following sarcoma resection.

      This review contains 20 Figures and 30 References. 

      Key words: abdominal wall reconstruction, chest wall reconstruction, free flap, lower extremity reconstruction, pedicled flap, propeller flap, reconstructive elevator, reconstructive ladder, upper extremity reconstruction 

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    • 7

      Adjuvant Radiation for Tissue Extremity and Truncal Sarcomas

      By Nabil Wasif, MD, MPH; Melody Qu, MD, MPH
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      Adjuvant Radiation for Tissue Extremity and Truncal Sarcomas

      • NABIL WASIF, MD, MPH
      • MELODY QU, MD, MPHClinical and Research Fellow, Department of Radiation Oncology, Princess Margaret Cancer Centre, Toronto, ON, Canada

      In this review, we cover the clinical use of radiation therapy in the treatment of soft tissue extremity and truncal sarcomas. After a brief overview of the seminal trials establishing the efficacy of radiation therapy as a component of limb-preserving therapy, we cover current indications for use and discuss when radiation therapy can be withheld without compromising oncologic outcomes. Controversies in treatment sequencing with surgery are fleshed out with pros and cons for both pre- and postoperative treatment. Finally, different modalities of radiation therapy are discussed and common toxicities are covered.


      This review contains 3 figures, 1 table and 41 references

      Key words:brachytherapy, limb preservation, neoadjuvant therapy, radiation therapy, soft tissue sarcoma

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    • 8

      Management of Locally Recurrent Sarcoma in the Extremity

      By Lauren Krumeich, MD; Madalyn G Neuwirth, MD; Giorgos Karakousis, MD
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      Management of Locally Recurrent Sarcoma in the Extremity

      • LAUREN KRUMEICH, MDResident, Department of Surgery, Hospital of the University of Pennsylvania, Philadelphia, PA
      • MADALYN G NEUWIRTH, MDResident, Department of Surgery, Hospital of the University of Pennsylvania, Philadelphia, PA
      • GIORGOS KARAKOUSIS, MDAssociate Professor of Surgery, Department of Surgery, Hospital of the University of Pennsylvania, Philadelphia, PA

      Extremity sarcomas are a heterogeneous group of malignant tumors with a varied propensity for local recurrence. This review focuses on factors associated with local recurrence and survival, diagnostic workup, management, outcomes, and surveillance. Local recurrence is more common in patients with previous local recurrence, positive margins, high-grade histology, and deep tumors. In the absence of metastases, the mainstay of treatment is limb-sparing surgery, with radiation to improve local control. Modalities such as brachytherapy or proton therapy may be valuable in the setting of previous irradiation. Systemic chemotherapy is typically limited to the treatment of distant disease, although chemotherapy can be delivered locally via limb perfusion or infusion for locally advanced or recurrent disease. Amputation is used if local control cannot be achieved while preserving adequate limb function or as a palliative option for pain, bleeding, or fungating tumors. Prognostic factors associated with poor survival include tumors that recur with high-grade histology, with a large size (> 5 cm), or within a short interval (< 16 months). Reports of 5-year overall survival in patients with locally recurrent sarcomas vary from 36 to 65%. Surveillance includes physical examination, cross-sectional imaging, and chest x-ray. Genetic profiling and intratumoral injections provide novel therapeutic targets.

      This review contains 1 figure, 4 tables and 40 references. 

      Key words: chemotherapy, hyperthermic isolated limb perfusion, intratumoral injection, isolated limb perfusion, local recurrence, margin status, radiation, soft tissue sarcoma, wide local excision 

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    • 9

      Leiomyosarcoma

      By Marco Fiore, MD; Sandro Pasquali, MD; Chiara Colombo, MD
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      Leiomyosarcoma

      • MARCO FIORE, MDSurgical Oncologist, Sarcoma Service, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
      • SANDRO PASQUALI, MDSurgical Oncologist, Sarcoma Service, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
      • CHIARA COLOMBO, MD Surgical Oncologist, Sarcoma Service, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy

      Leiomyosarcoma of soft tissue is a rare tumor affecting one new patient in every 100,000 people yearly. The most frequent sites of origin are the retroperitoneum and abdomen, followed by the limbs and trunk, skin, major vessels, and, rarely, organs. Increasing evidence suggests that all of these tumors originate from the smooth cell muscles of blood vessels. Surgery is the main treatment for localized disease. The risk of disease progression at distant sites outweighs that of local recurrence, suggesting that extensive resections are not recommended, particularly for retroperitoneal tumors. Moreover, leiomyosarcomas are sensitive to various chemotherapy regimens. The eighth edition of the AJCC TNM staging system introduced new accurate nomograms for predicting patient survival that are improving prognostic stratification for identifying those patients at higher risk for disease progression who are likely to benefit from perioperative treatments. More research for this rare tumor is needed to identify innovative predicting and prognostic biomarkers and new effective treatment strategies.

      This review contains 14 figures, 3  tables and 56 references

      Key words: inferior vena cava leiomyosarcoma, leiomyosarcoma, retroperitoneal sarcoma

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    • 10

      Evaluation of Intra-abdominal and Retroperitoneal Soft Tissue Masses

      By Jeffrey M Farma, MD, FACS; Neha Goel, MD
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      Evaluation of Intra-abdominal and Retroperitoneal Soft Tissue Masses

      • JEFFREY M FARMA, MD, FACSFox Chase Cancer Center, Philadelphia, PA, Associate Professor of Surgical Oncology, Program Director Surgical, Oncology Fellowship, Surgical Director Melanoma and Skin Cancer Program, Fox Chase Cancer Center
      • NEHA GOEL, MDSurgical Oncology Fellow, Fox Chase Cancer Center, Department of Surgical Oncology, Philadelphia, PA

      Proper workup of intra-abdominal and retroperitoneal sarcomas cannot be overstated in the management of these rare tumors. In particular, evaluation should begin with a thorough history and physical examination, including careful consideration of genetic syndromes. Next, computed tomography (CT) of the abdomen and pelvis with oral and intravenous contrast should be performed to determine whether a tumor is completely resectable as complete resection is the mainstay of sarcoma management. Completion staging studies with CT of the chest should also be performed. Following imaging, a core-needle biopsy, usually an interventional radiology–guided biopsy, is performed to obtain a tissue diagnosis. In certain large, intermediate- to high-grade tumors involving other vital organs, neoadjuvant chemotherapy or neoadjuvant radiation is considered to try to decrease the tumor in the hope of improving the chance of obtaining an R0 resection and to limit the extent of surgery. Overall, it is important to remember that prior to the initiation of any therapy, all patients should be evaluated and managed by a multidisciplinary team with expertise and experience in sarcoma.

      This review contains 3 figures, 3 tables and 39 references

      Key words: biopsy, genetic testing, intra-abdominal and retroperitoneal sarcoma, neoadjuvant chemotherapy, neoadjuvant radiotherapy

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    • 11

      Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma

      By Ann Y Lee, MD
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      Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma

      • ANN Y LEE, MDNYU School of Medicine, Assistant Professor, Department of Surgery, New York, NY 10016.

      Both myxofibrosarcoma and undifferentiated pleomorphic sarcoma (UPS) are sarcomas that primarily occur in the extremities of older patients. UPS (previously known as malignant fibrous histiocytoma) is now considered a diagnosis of exclusion, representing a group of high-grade, heterogeneous tumors with no clear line differentiation and characterized by high rates of distant metastases. Myxofibrosarcoma (formerly myxoid variant of malignant fibrous histiocytoma) is a distinct histologic entity that is largely distinguished from UPS by its myxoid stroma and curvilinear vascular pattern. Myxofibrosarcoma tends to have a locally infiltrative behavior that can often be seen as T2 hyperintense “tails” on magnetic resonance imaging. Local recurrence rates can be as high as 61% and are independent of grade. Unlike other sarcomas, it is recommended that tumors be excised with at least a 2 cm margin around both clinically and radiographically evident tumor. Low-grade tumors do not tend to metastasize but can recur as high-grade tumors with increased metastatic potential. Surgery is the mainstay of treatment for localized disease. For high-grade tumors, this may require complex plastics and vascular reconstruction. Selecting cases for multimodality therapy, including radiation and chemotherapy, should be done on a case-by-case basis after review by a multidisciplinary sarcoma team with particular attention to a patient’s risk of local recurrence and distant metastases. Further studies into histology-specific therapeutic targets will help inform future clinical trials.

      This review contains 4 figures, 4 tables and 54 references

      Key words: chemotherapy, distant metastases, extremity sarcoma, local recurrence, malignant fibrous histiocytoma, myxofibrosarcoma, radiation, sarcoma, surgical margins, Thyperintense tails, undifferentiated pleomorphic sarcoma

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    • 12

      Sarcoma: Vascular Tumors (EHE and Angiosarcoma)

      By Anne Grand'Maison, MD; Kilian Salerno, MD; John M Kane III, MD
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      Sarcoma: Vascular Tumors (EHE and Angiosarcoma)

      • ANNE GRAND'MAISON, MD
      • KILIAN SALERNO, MD
      • JOHN M KANE III, MD

      Angiosarcoma (AS) and epithelioid hemangioendothelioma (EHE) are rare but clinically disparate vascular sarcomas. Common AS scenarios include the head and neck/scalp region in older patients or in association with chronic lymphedema or radiation therapy (often in the setting of breast cancer treatment). AS behaves like a high-grade tumor with a propensity for multifocality and hematogenous metastases. The potentially curative treatment is negative margin wide resection. Adjuvant radiation may be considered to does not reduce the risk of local recurrence. Unresectable localized tumor can be treated with definitive radiation therapy. Although data are limited, neoadjuvant and adjuvant chemotherapy are occasionally used. Extensive primary tumor or metastatic disease is treated with systemic therapy. EHE can arise in the liver, bone, lung, pleura, or skin/soft tissue and can also metastasize hematogenously. The tumor is frequently asymptomatic and can be clinically indolent. Therefore, specific therapy is reserved for documented progression. Limited primary tumor is treated with surgical resection and occasionally adjuvant radiation. There is no proven benefit to adjuvant chemotherapy. Liver-directed therapy and occasionally transplantation are used for unresectable hepatic disease. Metastatic disease is treated with systemic therapy, but only in the setting of clinical progression.

      Key words: angiosarcoma, breast, cutaneous, epithelioid hemangioendothelioma, lymphedema, neoadjuvant chemotherapy, radiation associated, visceral

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    • 13

      Evaluation of a Soft Tissue Mass in the Extremity or Trunk

      By Daniel C Thomas, MD, MPH; Dale Han, MD
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      Evaluation of a Soft Tissue Mass in the Extremity or Trunk

      • DANIEL C THOMAS, MD, MPHResident, General Surgery, Section of Surgical Oncology, Department of Surgery, Yale University School of Medicine, New Haven, CT
      • DALE HAN, MDAssistant Professor of Surgery, Section of Surgical Oncology, Department of Surgery, Yale University School of Medicine, New Haven, CT

      Soft tissue sarcoma (STS) represents a diverse group of malignant tumors derived from mesenchymal elements. Evaluation of a soft tissue mass of the trunk or extremity starts with a detailed history and physical examination, which will help guide further diagnostic evaluation.  Imaging should then be obtained for an STS, with MRI being the preferred modality for the trunk or extremities. Imaging can assist with diagnosis and delineate the extent of the primary lesion, including the relationship to contiguous structures, for treatment planning and assessing resectability. Pathology diagnosis is most commonly performed via examination of a specimen obtained through image-guided percutaneous core-needle biopsy. This requires multidisciplinary planning between Surgery and Radiology to place the needle within the anticipated definitive surgical incision to allow for en bloc resection of the needle tract. Once the diagnosis of an STS is made, appropriate staging studies should be performed based on the history and physical examination and tumor histology, grade, and size. Clinical and pathology data are used to stage STS patients using the American Joint Committee on Cancer staging system, which incorporates the STS site, size and grade of the primary tumor, and absence or presence of nodal and distant metastatic disease.

      This review contains 6 figures, 6 tables and 50 references

      Key words: core-needle biopsy, extremity, histology, imaging, risk factors, soft tissue mass, soft tissue sarcoma, staging evaluation, trunk, tumor grade

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    • 14

      Pediatric Tumors: Rhabdomyosarcoma

      By John Groundland, MD, MS; Sara Shaw, BS; R L Randall, MD, FACS
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      Pediatric Tumors: Rhabdomyosarcoma

      • JOHN GROUNDLAND, MD, MSSarcoma Advanced Research & Clinical Fellow, Primary Children’s Hospital & Huntsman Cancer Institute, The University of Utah, Salt Lake City, UT
      • SARA SHAW, BSSarcoma Research Coordinator, Huntsman Cancer Institute, University of Utah, Salt Lake City, UT
      • R L RANDALL, MD, FACSThe L.B. & Olive S. Young Presidential Endowed Chair for Cancer Research, Director, Sarcoma Services & Chief, SARC Lab and Fellowships, Co-Leader, Sarcoma Disease Oriented Research Team, Co-Director, Huntsman-Intermountain Adolescent & Young Adult Oncology Program, Professor of Orthopaedics, Primary Children’s Hospital & Huntsman Cancer Institute, The University of Utah, Salt Lake City, UT

      Rhabdomyosarcoma (RMS) is a group of soft tissue sarcomas that share a common feature of primitive skeletal muscle differentiation. Three main subtypes have been characterized: embryonal, alveolar, and pleomorphic. Presentation and prognosis of RMS are highly variable, depending on anatomic location, subtype, and risk stratification. Currently, treatment centers on systemic control with cytotoxic chemotherapy and local control with surgery and/or therapeutic radiation.

      This review contains 3 figures, 10 tables and 50 references

      Key words: alveolar rhabdomyosarcoma, embryonal rhabdomyosarcoma, pediatric soft tissue sarcoma, pleomorphic rhabdomyosarcoma, rhabdomyosarcona, t(2;13), t(1;13)

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    • 15

      Rare Fibroblastic and Myofibroblastic Tumors

      By Vasilena Zheleva, MD; Yu Liang, MD, PhD; Laleh Melstrom, MD, MS, FACS
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      Rare Fibroblastic and Myofibroblastic Tumors

      • VASILENA ZHELEVA, MD
      • YU LIANG, MD, PHD
      • LALEH MELSTROM, MD, MS, FACS

      This review discusses the clinical presentation, histopathologic characteristics, and diagnostic and treatment algorithms for several distinct fibroblastic and myofibroblastic tumor subtypes. Low-grade fibromyxoid sarcomas typically arise in the deep soft tissues of the proximal extremities or trunk in young adults. They tend to have a high local recurrence rate, although some may have distant metastases. Histologically, they consist of bland fibroblasts with a whorled or linear arrangement alternating with hypocellular myxoid stroma. Sclerosing epithelioid fibrosarcomas typically arise in the deep soft tissues of the lower extremities of middle-aged adults. They tend to be slow growing but clinically aggressive, with a high propensity for local recurrence and distant metastases. Classic histologic features include the presence of small, uniform, ovoid epithelioid cells with sparse cytoplasm, arranged in cords within a sclerotic fibrous stroma. Myxoinflammatory fibroblastic sarcomas mainly affect the hands and feet. They are prone to local recurrence, with a very low likelihood of distant metastasis. Histologically, they have nodular architecture and highly cellular areas with an abundance of inflammatory cells, among which characteristic large Reed-Sternberg–like and lipoblast-like cells and hypocellular areas rich in myxoid stroma can be seen. Inflammatory myofibroblastic tumors typically occur in children and young adults, with common anatomic locations being the omentum, mesentery, retroperitoneum, and lung. Histologically, they are composed of myofibroblastic spindle cells accompanied by a lymphoplasmacytic inflammatory infiltrate. Surgical resection is the mainstay of treatment for all of the above tumor types. Radiation therapy may be used as adjuvant therapy for tumors with a high risk of local recurrence, in the setting of positive margins, or for unresectable primary or metastatic disease. There is no definite role for standard chemotherapy, but in certain cases, targeted therapy may be of use, such as ALK inhibitors in ALK-positive inflammatory myofibroblastic tumors. 

      Key words: inflammatory myofibroblastic sarcoma, low-grade fibromyxoid sarcoma, myxoinflammatory fibroblastic sarcoma, sclerosing epithelioid fibrosarcoma

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    • 16

      Pediatric Tumors: Extraskeletal Ewing Sarcoma

      By John Groundland, MD, MS; Sara Shaw, BS
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      Pediatric Tumors: Extraskeletal Ewing Sarcoma

      • JOHN GROUNDLAND, MD, MSSarcoma Advanced Research & Clinical Fellow, Primary Children’s Hospital & Huntsman Cancer Institute, The University of Utah, Salt Lake City, UT
      • SARA SHAW, BSSarcoma Research Coordinator, Huntsman Cancer Institute, University of Utah, Salt Lake City, UT

      Extraskeletal Ewing sarcoma is a rare clinical entity under the umbrella of the Ewing sarcoma family of tumors. The pathogenesis of the tumor has yet to be fully described, but a gene rearrangement and the resultant fusion protein characterize the molecular basis of the disease. Current therapy centers on cytotoxic chemotherapy and local control, either through surgical resection or therapeutic radiation. Survival data specific to extraskeletal Ewing sarcoma are limited due to the rare nature and varied presentation of the disease but parallel those of skeletally based Ewing sarcoma.

      This review contains 14 figures, 5 tables and 50 references. 

      Key words: CD 99, Ewing sarcoma, EWS-FLI1, extraskeletal Ewing sarcoma, pediatric soft tissue sarcoma, t(11;22) 

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    • 17

      Basics of Imaging Techniques in Evaluation of Soft Tissue Tumors

      By Emily Alanna Aherne, MB BCh, BAO, BMed Sci, MRCPI, FFR, RCSI,; Sinchun Hwang, MD
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      Basics of Imaging Techniques in Evaluation of Soft Tissue Tumors

      • EMILY ALANNA AHERNE, MB BCH, BAO, BMED SCI, MRCPI, FFR, RCSI,Combined Modality Imaging Fellow, Department of Musculoskeletal Radiology, Thomas Jefferson University Hospital, Philadelphia, PA; Body Oncology Imaging Fellow, Department of Radiology, Memorial Sloan Kettering Cancer Center, New York, NY
      • SINCHUN HWANG, MD Associate Attending Radiologist, Department of Radiology, Memorial Sloan Kettering Cancer Center, New York, NY

      Medical imaging plays a pivotal role in the detection, diagnosis, and clinical management of primary soft tissue tumors. Various imaging modalities have been used, and each modality offers unique advantages in the workup of soft tissue tumors by localizing the lesions in different compartments of the body and characterizing macroscopic tissue composition of the lesions in a noninvasive and safe manner. We review the clinical role and technical aspects of the frequently used imaging modalities, including magnetic resonance imaging (MRI), computed tomography, ultrasonography, positron emission tomography, and plain radiography with an emphasis on MRI as a mainstay imaging modality and a brief discussion of advanced techniques. We also review imaging features of common soft tissue tumors that can be detected and characterized using current imaging techniques.

      Key words: computed tomography, diffusion-weighted imaging, imaging, magnetic resonance imaging, positron emission tomography, soft tissue, technique, tumor, ultrasonography

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    • 18

      Solitary Fibrous Tumors

      By William W Tseng, MD; Shefali Chopra, MD; Dakshesh Patel, MD; Gino K In, MD; Omar Ragab, MD; James S Hu, MD; Lawrence R Menendez, MD; Stephen F Sener, MD
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      Solitary Fibrous Tumors

      • WILLIAM W TSENG, MDAssistant Professor, Section of Surgical Oncology, Department of Surgery, Keck School of Medicine, University of Southern California, Los Angeles, CA
      • SHEFALI CHOPRA, MDAssistant Professor, Department of Pathology, Keck School of Medicine, University of Southern California, Los Angeles, CA
      • DAKSHESH PATEL, MDAssociate Professor, Department of Radiology, Keck School of Medicine, University of Southern California, Los Angeles, CA
      • GINO K IN, MDAssistant Professor, Division of Medical Oncology, Department of Medicine, Norris Comprehensive Cancer Center, Keck School of Medicine, University of Southern California, Los Angeles, CA
      • OMAR RAGAB, MDAssistant Professor, Department of Radiation Oncology, Norris Comprehensive Cancer Center, Keck School of Medicine, University of Southern California, Los Angeles, CA
      • JAMES S HU, MDAssociate Professor, Division of Medical Oncology, Department of Medicine, Norris Comprehensive Cancer Center, Keck School of Medicine, University of Southern California, Los Angeles, CA
      • LAWRENCE R MENENDEZ, MDProfessor, Department of Orthopedics, Keck School of Medicine, University of Southern California, Los Angeles, CA
      • STEPHEN F SENER, MDProfessor, Section of Surgical Oncology, Department of Surgery, Keck School of Medicine, University of Southern California, Los Angeles, CA

      Solitary fibrous tumors (SFTs) are a subtype of soft tissue sarcoma with fibroblastic differentiation. SFTs typically occur in patients between ages 50 and 70 and most commonly develop in the pleural space or abdomen, although they have been described in virtually every body site. Cross-sectional imaging (CT or MRI) is useful to help characterize the tumor, and core-needle biopsy is critical to obtain tissue for diagnosis. Definitive diagnosis is now made by immunohistochemical expression of STAT6, which results from a key genetic aberration (NAB2-STAT6 fusion) recently identified in SFTs. For patients with SFT, surgical resection is the mainstay of treatment. Current data suggest some benefit with adjuvant radiation therapy and systemic therapy; however, further studies are needed. After treatment, surveillance in SFT patients is important, and late recurrences have been reported. Although these tumors are generally indolent, with low metastatic potential, a subset of cases will demonstrate aggressive behavior. Simple risk assessment models have been developed to help predict clinical behavior. Novel predictive biomarkers and disease biology–driven targeted therapy options are needed.

      This review contains 4 figures, 2 tables and 37 references

      Key words: cellular angiofibroma, characteristic chromosomal translocation, dermatofibrosarcoma protuberans, Doege-Potter syndrome, hemangiopericytoma, perineuroma, preoperative angioembolization, schwannoma, solitary fibrous tumors

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    • 19

      Regional Therapies for Extremity Sarcoma

      By Chiara Colombo, MD; Sandro Pasquali, MD
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      Regional Therapies for Extremity Sarcoma

      • CHIARA COLOMBO, MDDepartment of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
      • SANDRO PASQUALI, MDDepartment of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy

      Several regional therapies are used for the local treatment of patients with soft tissue sarcomas (STS), especially for tumors at high risk for local recurrence. Surgery with negative tumor resection margins is the main treatment for primary STS. External-beam radiation therapy is considered for deeply seated, large, and high-grade disease to lower the risk of local recurrence. A combination of preoperative chemo-radiation is associated with improved local control. TNF-α-based isolated limb perfusion is another regional chemotherapy strategy available at specialized surgical oncology units for unresectable STS. Other strategies suitable for management of advanced STS include cryoablation and radiofrequency. This review discusses these and other current regional treatment strategies. 

      This review contains 10 figures, 6 tables and 64 references

      Key words: cryoablation, extremity, hyperthermia, isolated limb perfusion, limb infusion, radiotherapy, regional therapy, sarcoma

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    • 20

      Liposarcoma

      By Joseph G Crompton, MD, PhD; Arun S Singh, MD; Sarah M Dry, MD; Fritz C Eilber, MD
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      Liposarcoma

      • JOSEPH G CROMPTON, MD, PHDResident, Department of Surgery, Sarcoma Research Fellow, Division of Surgical Oncology, University of California, Los Angeles, Los Angeles, CA
      • ARUN S SINGH, MDAssistant Professor, UCLA – JCC Sarcoma Program, Division of Hematology-Oncology, University of California, Los Angeles, Los Angeles, CA
      • SARAH M DRY, MDProfessor, Department of Pathology, University of California, Los Angeles, Los Angeles, CA
      • FRITZ C EILBER, MDDirector, UCLA – JCCC Sarcoma Program, Professor of Surgery, Professor of Molecular & Medical Pharmacology, Division of Surgical Oncology, University of California, Los Angeles, Los Angeles, CA

      Liposarcoma is a mesenchymal malignancy that predominantly arises in the retroperitoneum, trunk, and extremities. Histologic subtypes of liposarcoma include well-differentiated/dedifferentiated, myxoid/round cell, and pleomorphic liposarcoma. Although liposarcoma is rare and can occur in almost any anatomic location, it is the most common soft tissue sarcoma in the retroperitoneum, and surgery remains the only potentially curative therapy. This review discusses liposarcoma pathogenesis, classification, clinical-diagnostic evaluation, treatment, and surveillance. The cornerstone of treatment is surgery, with the main therapeutic goals of improving survival, limiting local recurrence, maximizing function, and minimizing morbidity. Perioperative radiation therapy may be indicated in patients with large high-grade liposarcomas. Systemic treatment—whether traditional chemotherapy or molecularly targeted therapy—may benefit patients depending on their histologic subtype, location, and extent of disease. For the majority of patients, however, systemic treatment is not curative. Controversial issues in the management of liposarcoma, such as surgical margins and extent of resection, are also discussed.   

      This review contains 2 figures, 5 tables and 39 references

      Key words: atypical lipomatous liposarcoma, liposarcoma, myxoid liposarcoma, pleomorphic liposarcoma, retroperitoneal sarcoma, soft tissue sarcoma, well-differentiated/dedifferentiated liposarcoma

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    • 21

      The Use of Adjuvant or Neoadjuvant Chemotherapy in Extremity and Truncal Sarcoma

      By Robert J Canter, MD, MAS
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      The Use of Adjuvant or Neoadjuvant Chemotherapy in Extremity and Truncal Sarcoma

      • ROBERT J CANTER, MD, MASProfessor, Department of Surgery, Division of Surgical Oncology, University of California, Davis School of Medicine, Sacramento, CA 95817, United States

      Although neoadjuvant chemotherapy has been an established component of multimodality cancer care for patients with pediatric sarcomas for the past 25 years, the role of adjuvant or neoadjuvant chemotherapy in the management of adult patients with soft tissue sarcoma (STS) amenable to treatment with curative intent remains controversial. Overall, meta-analyses have revealed modest improvements in survival outcomes with the use of adjuvant or neoadjuvant chemotherapy, but individual trials have demonstrated inconsistent results leading some to question the robustness and external validity of the results. A recent randomized trial using anthracycline- and ifosfamide-based chemotherapy has provided further positive evidence in support of neoadjuvant chemotherapy for adult STS patients, but concerns persist regarding the risks of chemotherapy-related toxicities and the generalizability of the findings. Given the substantial risk of distant recurrence and disease-specific death for adult STS patients with tumors greater than 10 cm, especially those with synovial sarcoma and myxoid or round liposarcoma histologies, these patients should be strongly considered for neoadjuvant chemotherapy as part of a combined modality approach. The impact of recent level I data on the broader implementation of adjuvant or neoadjuvant chemotherapy in adult STS remains to be seen.

       This review contains 5 figures and 34 references

      Key Words: chemotherapy, limb salvage, myxoid/round cell liposarcoma, multimodality therapy, soft tissue sarcoma, surgery, survival, synovial sarcoma, undifferentiated pleomorphic sarcoma

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    • 22

      Nerve Sheath Tumors: Malignant Peripheral Nerve Sheath Tumors, Schwannomas, and Neurofibromas

      By Keila E Torres, MD, PhD; Wei-Lien Wang, MD; Alexander Lazar, MD, PhD; Ian E McCutcheon
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      Nerve Sheath Tumors: Malignant Peripheral Nerve Sheath Tumors, Schwannomas, and Neurofibromas

      • KEILA E TORRES, MD, PHDAssociate Professor, Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX
      • WEI-LIEN WANG, MDAssociate Professor, Division of Pathology and Laboratory Medicine, Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, TX
      • ALEXANDER LAZAR, MD, PHDAssociate Professor, Division of Pathology and Laboratory Medicine, Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, TX
      • IAN E MCCUTCHEONProfessor, Department of Neurosurgery, The University of Texas MD Anderson Cancer Center, Houston, TX

      Peripheral nerve sheath tumors represent a spectrum of entities ranging from benign tumors, such as schwannoma, to high-grade malignant tumors termed malignant peripheral nerve sheath tumors. In this chapter, we discuss current concepts and problematic areas in the diagnosis, treatment, and management of peripheral nerve sheath tumors. We discuss the major categories of nerve sheath tumors including schwannomas, neurofibromas, and malignant peripheral nerve sheath tumors. The different benign variants and their propensity for malignant transformation are highlighted. We suggest useful guidelines for the surgical management of these challenging cases. Finally, we discuss some of the advances in molecular biology that have provided new insights into the nature of the various peripheral nerve sheath tumors and suggest potential novel targeted therapeutic strategies for these tumors.

      This review contains 11 figures, 5 tables and 66 references.

      Key Words: malignant peripheral nerve sheath tumors, nerve sheath tumor, neurofibroma, neurofibromatosis, schwannoma, schwannomatosis, NF1, NF2

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    • 23

      Desmoplastic Small Round Cell Tumor

      By James A Saltsman III, MD; Todd E Heaton, MD, MPH
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      Desmoplastic Small Round Cell Tumor

      • JAMES A SALTSMAN III, MDDepartment of Surgery, Mount Sinai School of Medicine, New York, NY
      • TODD E HEATON, MD, MPHDivision of Pediatric Surgery, Memorial Sloan Kettering Cancer Center, New York, NY

      Desmoplastic small round cell tumor (DSRCT) is a relatively recently identified, rare, aggressive cancer that arises from the peritoneal lining of the abdominal cavity and predominantly affects male adolescents and young adults. DSRCT is included in the Ewing family of tumors and harbors a unique translocation between EWSR and WT1 genes. Patients characteristically present with advanced disease, including widespread involvement of the abdominal cavity and pelvis, with frequent extraperitoneal metastases involving liver, spleen, and thoracic lymph nodes. Five-year overall survival remains approximately 15 to 20% in recent series despite aggressive multimodal therapy. Neoadjuvant chemotherapy, complete resection, defined as less than 1 cm3 of residual tumor, and consolidative whole abdominopelvic radiotherapy (WAP-RT) appear to play important role in curative treatment. The rarity of DSRCT makes large randomized trials difficult and consensus statements about clinical approach impossible. However, this chapter summarizes the best available data on the pathogenesis, diagnosis, and treatment of DSRCT; highlights the major advances made in these areas; and discusses the addition of local therapy to the treatment paradigm.

      This review contains 3 figures, 1 table, and 51 references.  

      Key Words: DSRCT, peritoneal malignancy, desmoplastic round cell tumor, surface malignancies of the peritoneum, desmoplastic small round cell tumor of the abdomen, surgical oncology, rare malignancies, surgical therapy of abdominal malignancies

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    • 24

      Lymphotropic Sarcomas (epithelioid Sarcoma and Clear Cell Sarcoma)

      By Alexandra G Lopez-Aguiar, MD; Kenneth Cardona, MD, FACS
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      Lymphotropic Sarcomas (epithelioid Sarcoma and Clear Cell Sarcoma)

      • ALEXANDRA G LOPEZ-AGUIAR, MDDepartment of Surgery, Emory University School of Medicine, Atlanta, GA
      • KENNETH CARDONA, MD, FACSAssistant Professor of Surgery, Division of Surgical Oncology, Department of Surgery, Winship Cancer Institute, Emory University, Atlanta, GA

      Lymphotropic sarcomas encompass a rare subset of soft tissue sarcoma histotypes known for their distinct ability to metastasize to locoregional lymph nodes. Epithelioid and clear cell sarcomas are the predominant lymphotropic subtypes and the focus of this review. The utility of lymph node assessment, whether clinical, radiographic, and/or surgical, in patients with these sarcomas is presented. The role of sentinel lymph node biopsy as well as completion lymphadenectomy in patients with these lymphotropic sarcoma histotypes is discussed. Surgery remains the only potential curative option for these tumors, and advancement in histology-specific chemotherapeutic regimens and biologic therapy options is necessary.

       This review contains 6 tables and 50 references.

      Key words: clear cell sarcoma, completion lymphadenectomy, epithelioid sarcoma, EWS-ATF1, limb-sparing surgery, lymph node metastasis, sentinel lymph node biopsy, soft tissue sarcoma

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    • 25

      Desmoid-type Fibromatosis

      By Aimee M. Crago, MD; Timothy M. Loftus, MD
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      Desmoid-type Fibromatosis

      • AIMEE M. CRAGO, MDAssistant Attending Surgeon, Sarcoma Disease Management Team, Gastric and Mixed Tumor Service, Memorial Sloan Kettering Cancer Center, New York, NY
      • TIMOTHY M. LOFTUS, MDEmergency Medicine Resident, Department of Emergency Medicine, University of Cincinnati, Cincinnati, OH

      Desmoid-type fibromatosis is a rare disease that does not have metastatic potential. It can be locally aggressive or regress spontaneously. For this reason, and because many of these tumors are inoperable or surgery is associated with significant morbidity, nonoperative treatment modalities are now more commonly employed to manage these lesions. Active observation, ablative therapies, and systemic treatments such as doxorubicin-based chemotherapy or sorafenib can be prescribed to minimize morbidities associated with the disease and surgical intervention. A thorough understanding regarding desmoid biology and the relative indications for multimodality therapies is essential to triage patients for appropriate interventions.

      This review contains 5 figures, and 29 references.

      Key Words: active observation, β-catenin, desmoid, familial adenomatous polyposis, fibromatosis, soft tissue tumors

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  • Cancer of the Small Bowel and Spleen
    • 1

      Small Intestine: Adenocarcinoma

      By Joel M Baumgartner, MD, MAS; Sudeep Banerjee, MD; Jason K Sicklick, MD
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      Small Intestine: Adenocarcinoma

      • JOEL M BAUMGARTNER, MD, MASAssistant Professor of Surgery, Division of Surgical Oncology, Department of Surgery, University of California, San Diego, La Jolla, CA
      • SUDEEP BANERJEE, MDResearch Fellow, Division of Surgical Oncology, Department of Surgery, University of California, San Diego, La Jolla, CA; Surgery Resident, Department of Surgery, University of California, Los Angeles, Los Angeles , CA
      • JASON K SICKLICK, MDAssociate Professor of Surgery, Division of Surgical Oncology, Department of Surgery, University of California, San Diego, La Jolla, CA

      Adenocarcinoma is the second most common nonduodenal small bowel tumor. Small bowel adenocarcinoma has risk factors similar to those of colorectal adenocarcinoma but is rarer and less well understood. Diagnosis relies on advanced imaging techniques as well as endoscopy or enteroscopy for tissue diagnosis. Aggressive biology and vague symptoms in early disease cause a majority of patients to present with late-stage disease. Adenocarcinomas with lymph node involvement should be treated with resection and systemic chemotherapy. In contrast, systemic chemotherapy alone should be employed in cases with distant metastases unless the primary tumor is bleeding, perforated, or causing a bowel obstruction.

      This review contains 4 figures, 5 tables and 17 references

      Key words: adenocarcinoma, chemotherapy, enteroscopy, hereditary syndrome, inflammatory bowel disease, lymph node, mesentery, small bowel

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    • 2

      Small Intestine: Neuroendocrine and Miscellaneous Tumors

      By Joel M Baumgartner, MD, MAS; Sudeep Banerjee, MD; Jason K Sicklick, MD
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      Small Intestine: Neuroendocrine and Miscellaneous Tumors

      • JOEL M BAUMGARTNER, MD, MASAssistant Professor of Surgery, Division of Surgical Oncology, Department of Surgery, University of California, San Diego, La Jolla, CA
      • SUDEEP BANERJEE, MDResearch Fellow, Division of Surgical Oncology, Department of Surgery, University of California, San Diego, La Jolla, CA; Surgery Resident, Department of Surgery, University of California, Los Angeles, Los Angeles , CA
      • JASON K SICKLICK, MDAssociate Professor of Surgery, Division of Surgical Oncology, Department of Surgery, University of California, San Diego, La Jolla, CA

      Carcinoid tumors are the most common nonduodenal small bowel tumors. Although the diagnosis of any small bowel tumor is challenging, serum tumor markers and specialized imaging can aid in the diagnosis of carcinoid. Localized carcinoids are treated with surgical resection, whereas metastatic tumors are treated with somatostatin analogues, although liver-directed therapies can improve disease-related symptoms. In contrast, small bowel lymphomas are primarily treated with chemotherapy and sometimes radiation, although surgical intervention may be necessary for diagnosis and resection for palliation of symptoms. Furthermore, there are many benign etiologies of small bowel tumors, including adenoma, leiomyoma, and lipoma. The small bowel can also be a site of distant metastases for which surgical management is reserved for the treatment of complications such as bleeding, obstruction, perforation, or pain.

      This review contains 3 figures, 3 tables and 17 references. 

      Key words: carcinoid tumor, desmoid tumor, locoregional therapy, mesentery, neuroendocrine tumor, small bowel, small bowel lymphoma, somatostatin

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    • 3

      Malignant Tumors of the Spleen

      By Alessandro Paniccia, MD; Brandon Chapman, MD; Ana Gleisner, MD, PhD
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      Malignant Tumors of the Spleen

      • ALESSANDRO PANICCIA, MDgeneral surgery resident, Department of Surgery, University of Colorado School of Medicine, Aurora, CO
      • BRANDON CHAPMAN, MDgeneral surgery resident, Department of Surgery, University of Colorado School of Medicine, Aurora, CO
      • ANA GLEISNER, MD, PHDAssistant Professor, Department of Surgery, University of Colorado School of Medicine, Aurora, CO

      Malignant tumors of the spleen are rare lesions that can be divided into three broad categories: lymphoproliferative diseases, metastatic lesions, and primary splenic (nonlymphoid) malignant neoplasms. Additionally, although myeloproliferative diseases are not tumors of the spleen, they do have the potential to either directly or indirectly affect splenic function, often manifesting as splenomegaly, hypersplenism, or both. Lymphoproliferative and myeloproliferative diseases are almost always systemic diseases. Consequently, medical therapy is the primary treatment, and splenectomy is currently reserved for patients with hematologic disorders refractory to medical management—for palliation of symptoms caused by splenomegaly or hypersplenism—and in the rare case of the pure splenic form of the disease. Patients affected by malignant splenic lesions often present with vague symptomatology, including generalized malaise, fatigue, weight loss, and fever. These symptoms may be accompanied by abdominal pain, a palpable left upper quadrant mass, or overt splenomegaly. Radiologic imaging, including ultrasonography, computed tomography, and magnetic resonance imaging, is mandatory in the evaluation of splenic tumors and may narrow the differential diagnosis. Management of splenic metastases should be individualized and, whenever possible, determined in a multidisciplinary setting. Splenectomy is the treatment of choice for patients with primary malignant tumors of the spleen in the absence of metastatic disease. 

      This review contains 10 figures, 8 tables and 51 references

      Key words: angiosarcoma, leukemia, malignant splenic tumor, metastatic splenic tumor, spleen, splenectomy, splenic lymphoma

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    • 4

      Gastrointestinal Stromal Tumors of the Small Intestine

      By Joel M Baumgartner, MD, MAS; Sudeep Banerjee, MD; Jason K Sicklick, MD
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      Gastrointestinal Stromal Tumors of the Small Intestine

      • JOEL M BAUMGARTNER, MD, MASAssistant Professor of Surgery, Division of Surgical Oncology, Department of Surgery, University of California, San Diego, La Jolla, CA
      • SUDEEP BANERJEE, MDResearch Fellow, Division of Surgical Oncology, Department of Surgery, University of California, San Diego, La Jolla, CA; Surgery Resident, Department of Surgery, University of California, Los Angeles, Los Angeles , CA
      • JASON K SICKLICK, MDAssociate Professor of Surgery, Division of Surgical Oncology, Department of Surgery, University of California, San Diego, La Jolla, CA

      Gastrointestinal stromal tumor (GIST) is the most common type of sarcoma and the third most common malignant small bowel tumor. After the stomach, the small bowel is the second most common location for GISTs. Although a majority of GISTs are driven by KIT oncogene mutations, there is increasing evidence that GIST is a genetically heterogeneous disease. Noninvasive imaging provides useful anatomic information but is not adequate to diagnose GIST. Endoscopic ultrasonography–guided tissue biopsy is the preferred method of diagnosis. GISTs are primarily treated with surgical resection, whereas adjuvant tyrosine kinase inhibitor therapy with imatinib is reserved for high-risk cases or in the setting of metastatic disease. Minimally invasive surgical approaches are safe and do not compromise oncologic outcomes in experienced centers. Selective cytoreductive surgery for metastatic GIST remains controversial but may have a role in appropriately selected patients.

      This review contains 10 figures, 9 tables and 65 references

      Key words: cytoreductive surgery, gastrointestinal stromal tumor, imatinib, interstitial cell of Cajal, KIT oncogene, minimally invasive surgery, molecular profile, small bowel

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    • 5

      Benign Tumors of the Spleen

      By Alessandro Paniccia, MD; Brandon Chapman, MD
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      Benign Tumors of the Spleen

      • ALESSANDRO PANICCIA, MDgeneral surgery resident, Department of Surgery, University of Colorado School of Medicine, Aurora, CO
      • BRANDON CHAPMAN, MDgeneral surgery resident, Department of Surgery, University of Colorado School of Medicine, Aurora, CO

      Primary benign splenic tumors are uncommon and manifest with variable presenting signs and symptoms yet are frequently asymptomatic and discovered incidentally. Primary benign tumors involving the spleen can be broadly categorized as vascular/lymphatic tumors, tumorlike lesions, and cystic tumors. Although laboratory tests have little impact on the diagnosis of benign tumors of the spleen, radiologic imaging is mandatory and may narrow the differential diagnoses. However, it is often difficult to distinguish benign from malignant disease. Surgical management is based on the clinical presentation of the patient, disease etiology, and suspicion for malignancy. Surgical options include complete and partial splenectomy, and
      the laparoscopic approach is favored.

      This review contains 6 figures, 6 tables and 66 references

      Key words: cystic, mass, spleen, splenectomy, tumor, tumorlike, vascular neoplasm

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  • Skin Cancer
    • 1

      Primary Cutaneous Melanoma

      By Clara R Farley, Resident; Keith A Delman, Carlos Professor of Surgery; Michael C Lowe, Assistant Professor
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      Primary Cutaneous Melanoma

      • CLARA R FARLEY, RESIDENTDepartment of General Surgery, Emory University School of Medicine, Atlanta, GA
      • KEITH A DELMAN, CARLOS PROFESSOR OF SURGERYDivision of Surgical Oncology, Emory University School of Medicine, Atlanta, GA
      • MICHAEL C LOWE, ASSISTANT PROFESSORDivision of Surgical Oncology, Department of Surgery, Emory University School of Medicine, Atlanta, GA

      Melanoma presents a significant health burden as its incidence continues to rise in both sexes and remains the most common cause of skin cancer–related death. Risk factors for the development of melanoma include sun exposure, fair complexion, increasing age, previous melanoma, multiple dysplastic nevi, and familial syndromes. Wide local excision is the standard of care for those with early forms of melanoma, with sentinel lymph node biopsy in appropriate populations. Sentinel lymph node status contributes to the discussion as to whether to pursue completion lymphadenectomy. This review outlines surgical treatment of primary cutaneous melanoma, including wide local excision, sentinel lymph node biopsy, and completion lymphadenectomy.

      This review contains 10 figures, 4 tables and 33 references

      Key words: biopsy, cutaneous, lymphadenectomy, margin, melanoma, pathology, primary, sentinel node, surgery, treatment

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    • 2

      Merkel Cell Carcinoma

      By Giorgos Karakousis, MD; Charles C Vining, MD; Andrew J Sinnamon, MD
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      Merkel Cell Carcinoma

      • GIORGOS KARAKOUSIS, MDAssociate Professor of Surgery, Department of Surgery, Hospital of the University of Pennsylvania, Philadelphia, PA
      • CHARLES C VINING, MDResident in Surgery, Division of Endocrine and Oncologic Surgery, Department of Surgery, Perelman School of Medicine, The University of Pennsylvania, Philadelphia,
      • ANDREW J SINNAMON, MDResident in Surgery, Division of Endocrine and Oncologic Surgery, Department of Surgery, Perelman School of Medicine, The University of Pennsylvania, Philadelphia, PA

      Merkel cell carcinoma (MCC) is a rare and highly aggressive carcinoma of the skin with a high recurrence rate and propensity for metastasis. It was first described in 1972and subsequently reclassified as a neuroendocrine tumor. Merkel cells are now thought to arise from the epidermal stem cells, not neural crest progenitor cells, as originally thought, and are the only cutaneous cells that form electron-dense neurosecretory granules. Although rare, the incidence of MCC is rapidly increasing. MCC is more often than not associated with Merkel cell polyomavirus, which may be involved in the malignant transformation and pathogenesis of MCC. Mortality exceeds that of the more commonly occurring skin cancer melanoma, with an overall 5-year survival rate ranging from 30 to 64%. Large reviews have documented recurrence rates ranging from 25 to 50% for local recurrence, 52 to 59% for regional recurrence, and 34 to 36% for distant metastases. Early-stage disease can sometimes be cured with surgery and radiation. Due to the rarity of MCC, statistically significant prospective data are lacking to validate prognostic features and treatment. Therefore, recommendations are largely based on small studies, meta-analyses, and expert consensus. Moreover, no consistent molecular activator in MCC has been identified, resulting in large variability in targeted therapies used in its treatment. New therapies to treat advanced MCC, including immunotherapy, hold promise for improved outcomes in the future. This review summarizes current knowledge regarding the etiology, prognosis, and management of MCC.


      This review contains 6 figures, 4 tables and 52 references

      Key words: Merkel cell carcinoma, Merkel cell polyomavirus, Merkel cell tactile receptor

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    • 3

      Approach to Metastatic Melanoma

      By Alexandra Gangi, MD; Jonathan S Zager, MD, FACS
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      Approach to Metastatic Melanoma

      • ALEXANDRA GANGI, MD
      • JONATHAN S ZAGER, MD, FACS

      Over several decades, the incidence of melanoma has steadily risen. Although a minority of newly diagnosed melanoma patients present with distant metastasis at initial diagnosis, approximately 30% of patients eventually develop metastatic disease as a consequence of disease progression. Although almost all organs can be involved, the most frequent sites of disease are either local or regional recurrences. This review outlines surgical treatment of recurrent or metastatic melanoma, including regional therapies, as well as management with systemic therapy. 

      Key words: in-transit disease, limb infusion, metastatectomy, metastatic melanoma, percutaneous hepatic perfusion, regional therapy, systemic therapy

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    • 4

      Squamous Cell Carcinoma: Epidemiology, Risk Factors, and Treatment Options

      By Jeffrey M Farma, MD, FACS; Elena P Lamb, MD, MS
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      Squamous Cell Carcinoma: Epidemiology, Risk Factors, and Treatment Options

      • JEFFREY M FARMA, MD, FACSFox Chase Cancer Center, Philadelphia, PA, Associate Professor of Surgical Oncology, Program Director Surgical, Oncology Fellowship, Surgical Director Melanoma and Skin Cancer Program, Fox Chase Cancer Center
      • ELENA P LAMB, MD, MSFox Chase Cancer Center, Philadelphia, PA, Complex General Surgical Oncology Fellow, Fox Chase Cancer Center

      Ultraviolent (UV) solar radiation is considered to be the dominant risk factor for development of squamous cell carcinoma (SCC). The development of SCC appears to be linked to the cumulative dose of UV radiation over time. Identifying patients with high-risk factors for developing SCC includes chronic immunosuppression, exposure to ionizing radiation, and certain genetic syndromes. Primary treatment goals of cutaneous SCC include cure of tumor with maximal preservation of function. Treatment options should be stratified for low- and high-risk SCC. Primary treatment options for low-risk SCC include (1) curettage and electrodesiccation, (2) excision with postoperative margin assessment (POMA) with 4 to 6 mm margins, and (3) radiation therapy (RT) for nonsurgical candidates. Options for high-risk SCC lesions include (1) Mohs surgery or resection with complete circumferential peripheral and deep margin assessment, (2) excision with POMA with wider surgical margins and primary or delayed repair, and (3) RT for nonsurgical candidates.    

      This review contains 4 figures, 5 tables, and 32 references.

      Key words: cutaneous squamous cell carcinoma, Mohs micrographic surgery, pathologic risk factors, radiation therapy, surgical margins 

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    • 5

      Basal Cell Carcinoma: Epidemiology, Risk Factors, and Treatment Options

      By Jeffrey M Farma, MD, FACS; Elena P Lamb, MD, MS
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      Basal Cell Carcinoma: Epidemiology, Risk Factors, and Treatment Options

      • JEFFREY M FARMA, MD, FACSFox Chase Cancer Center, Philadelphia, PA, Associate Professor of Surgical Oncology, Program Director Surgical, Oncology Fellowship, Surgical Director Melanoma and Skin Cancer Program, Fox Chase Cancer Center
      • ELENA P LAMB, MD, MSFox Chase Cancer Center, Philadelphia, PA, Complex General Surgical Oncology Fellow, Fox Chase Cancer Center

      Ultraviolent (UV) solar radiation is considered to be the dominant risk factor for development of basal cell carcinoma (BCC). The development of BCC is thought to arise from intense, intermittent sun exposure leading to burns. Identifying patients with high-risk factors for developing BCC includes chronic immunosuppression, exposure to ionizing radiation, and certain genetic syndromes. Primary treatment goals of BCC include cure of tumor with maximal preservation of function. Although rarely metastatic, BCC can produce substantial local destruction. Treatment modalities can be divided into surgical and nonsurgical therapies, although surgical therapy is the mainstay of treatment. Superficial therapies, such as topical imiquimod or 5-fluorouracil, photodynamic therapy, or cryotherapy, may be effective for anatomically challenging locations where surgery or radiation is contraindicated, but the cure rates of these approaches are lower compared with surgery. Recent FDA-approved hedgehog pathway inhibitors include vismodegib and sonidegib for patients who have exhausted surgical and radiation options for treating advanced BCC.

      This review contains 4 figures, 5 tables, and 25 references.

      Key words: cryosurgery, cutaneous basal cell carcinoma, hedgehog pathway inhibitors, Mohs micrographic surgery, pathologic risk factors, photodynamic therapy, radiation therapy, surgical margins, topical therapies 

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  • Pancreatic and Duodenal Cancer
    • 1

      Management of Pancreatic Adenocarcinoma

      By Alexandra G Lopez-Aguiar, MD; Shishir K Maithel, MD, FACS; David A Kooby, MD, FACS
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      Management of Pancreatic Adenocarcinoma

      • ALEXANDRA G LOPEZ-AGUIAR, MDDepartment of Surgery, Emory University School of Medicine, Atlanta, GA
      • SHISHIR K MAITHEL, MD, FACSDepartment of Surgery, Emory University School of Medicine, Atlanta, GA
      • DAVID A KOOBY, MD, FACSDepartment of Surgery, Emory University School of Medicine, Atlanta, GA

      Pancreatic ductal adenocarcinoma is a lethal disease with a poor overall survival. Surgery remains the only potential curative option, and multimodal therapy is typically indicated. Pancreatic cancer can be categorized into four groups according to presentation: resectable disease, borderline resectable disease, locally advanced unresectable disease, and metastatic unresectable disease. Pancreaticoduodenectomy is the most common operation performed for patients with resectable and borderline resectable disease, followed by distal pancreatectomy, and, rarely, total pancreatectomy, with the goal of obtaining an R0 resection. The morbidity associated with these procedures remains high, and thoughtful patient selection is paramount. Postoperative chemotherapy protocols, with or without radiotherapy, are standard, and neoadjuvant therapy is gaining momentum. This review summarizes appropriate management of resectable pancreatic adenocarcinoma.

      This review contains 12 figures, 5 tables and 49 references

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    • 2

      Understanding the Genetics of Pancreatic Adenocarcinoma

      By Apoorve Nayyar, MBBS, Post-Doctoral Research Associate,; Jen Jen Yeh, MD
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      Understanding the Genetics of Pancreatic Adenocarcinoma

      • APOORVE NAYYAR, MBBS, POST-DOCTORAL RESEARCH ASSOCIATE,Lineberger Comprehensive Cancer Center, University of North Carolina at Chapel Hill, Chapel Hill, NC
      • JEN JEN YEH, MDProfessor, Departments of Surgery and Pharmacology, Vice Chair of Research, Department of Surgery, University of North Carolina at Chapel Hill, Chapel Hill, NC

      The underlying molecular basis of pancreatic ductal adenocarcinoma (PDAC) is complex, with multiple genetic, epigenetic, and transcriptomic changes. Five to 10% of PDAC cases have a hereditary basis, with multiple risk-conferring genetic mutations running in the family. All PDAC tumors develop from noninvasive precursor lesions that reflect the temporal accumulation of mutations. Recent advances in high-throughput sequencing technologies have facilitated a better understanding of the diverse mutational landscape of PDAC, reaffirming the role of genes previously known to be mutated (K-ras, CDKN2A, TP53, SMAD4, SLIT/ROBO, SWI/SNF) and revealing novel insights into the differential gene expression patterns and structural variations involved. K-ras mutation, an early event in disease pathogenesis, plays a pivotal role in the initiation, progression, and maintenance of PDAC, with the mutation type impacting disease pathophysiology and prognosis. The subsequent loss of tumor suppressors (CDKN2A, TP53, SMAD4) promotes genetic instability and uncontrolled cellular proliferation. Molecular subtypes with potential prognostic and therapeutic relevance have been identified. The challenge now is to translate this wealth of knowledge to the clinic.

      This review contains 3 figures, 3 tables and 50 references

      Key words: familial pancreatic cancer, heterogeneity, molecular subtypes, pancreatic ductal adenocarcinoma, whole genome/exome sequencing

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    • 3

      Adjuvant and Neoadjuvant Therapy for Pancreatic Adenocarcinoma

      By Gregory C Wilson, MD; Brent T Xia, MD; Syed A Ahmed, MD
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      Adjuvant and Neoadjuvant Therapy for Pancreatic Adenocarcinoma

      • GREGORY C WILSON, MD
      • BRENT T XIA, MD
      • SYED A AHMED, MD

      Despite decades of advancement and research into the multimodal care of pancreatic cancer, mortality after the diagnosis of pancreatic ductal adenocarcinoma remains grim. The role of adjuvant therapy following surgical resection has been well established in the literature. However, adjuvant therapy is imperfect, and outside of a clinical trial, there are high rates of omission or delayed initiation of therapy. Neoadjuvant treatment strategies continue to be explored in the management of resectable, borderline-resectable, and locally advanced unresectable pancreatic adenocarcinoma. With improved resection rates and the possibility for tumor downstaging, neoadjuvant therapy has become standard for patients with borderline-resectable and locally advanced unresectable tumors. Additional benefits of neoadjuvant therapy in the treatment of resectable tumors include improved completion rates of systemic therapy and R0 resection rates. Future clinical trials, including the use of novel treatment agents and combination treatment strategies in both neoadjuvant and adjuvant regimens, will add value to the treatment of pancreatic adenocarcinoma.

      Key words: adjuvant therapy, borderline-resectable pancreatic cancer, locally advanced pancreatic cancer, neoadjuvant therapy, pancreatic adenocarcinoma, resectable disease 

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    • 4

      Management of Neuroendocrine Tumors of the Pancreas

      By Robert CG Martin II, MD, PhD, FACS
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      Management of Neuroendocrine Tumors of the Pancreas

      • ROBERT CG MARTIN II, MD, PHD, FACSSam & Lolita Weakley Endowed Chair, Surgical Oncology; Director, Division of Surgical Oncology; Professor of Surgery; Director, Upper GI & HPB Multidisciplinary Clinic; Academic Advisory Dean, University of Louisville, 315 E. Broadway ­ M10 - Rm#312, Louisville, Ky 40202, United States

      Neuroendocrine tumors (NETs) comprise a heterogeneous group of malignancies, with differences in prognosis and effective therapies. Traditionally, NETs have been characterized by tumor grade, site of primary tumor, functional status, and presence of underlying familial syndrome. However, increased feasibility and use of next-generation sequencing and other methodologies have revealed new genomic and epigenetic aberrations. In the past decade, treatment options available for metastatic well-differentiated gastroenteropancreatic NETs have expanded, with the approval of antiangiogenic and mTOR-directed targeted therapies, and our armamentarium of active therapies is likely to further increase. Optimal surgical therapy both for nonfunctional and functional PNETs has become more personalized and strategic to optimize long-term quality of life time. Management of localized metastatic PNET has become a true multidisciplinary management opportunity that all treating physicians should use. Peptide receptor radioligand therapy is an emerging treatment that uses functional imaging to personalize dosimetry to the tumor and avoid nephrotoxicity. Nevertheless, there is a critical need for further biomarkers, particularly multianalyte biomarkers, to aid in prognostication and predict efficacy of therapies.

      This review contains 1 figure, 1 table, and 72 references.

      Key Words: carcinoid tumor, islet cell carcinoma, neuroendocrine tumors, pancreatic, surgical management, medical management, metastatic, localized.

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    • 5

      Imaging of Malignant and Benign Tumors of the Pancreas

      By Ersan Altun, MD; Richard C Semelka, MD
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      Imaging of Malignant and Benign Tumors of the Pancreas

      • ERSAN ALTUN, MDAssistant Professor of Radiology, Department of Radiology, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC
      • RICHARD C SEMELKA, MDProfessor of Radiology, Director of MRI, Director of Quality and Safety, Department of Radiology, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC

      Pancreatic cross-sectional imaging has been widely used to diagnose and stage pancreatic neoplasms. The most commonly used techniques include multidetector CT, MRI, and endoscopic ultrasonography. Hybrid imaging including positron emission tomography combined with CT has a limited role. Dedicated imaging applications of these modalities for the evaluation of pancreatic neoplasms and their accuracies for different neoplasms are summarized in this review. Critical and differential imaging findings of the most common neoplasms of the pancreas, including adenocarcinoma, neuroendocrine tumors, cystic neoplasms, lymphoma, and metastases, emphasizing the most accurate imaging techniques are also discussed. Additionally, the most common mimics of the pancreatic neoplasms and their imaging findings are reviewed.

      This review contains 32 references, 13 figures, and 5 tables.

      Key words: adenocarcinoma, CT, EUS, intraductal papillary mucinous neoplasm, mucinous cystic neoplasm, MRI, neuroendocrine tumors, PET-CT, serous cystadenoma

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    • 6

      Duodenal Malignancies

      By Emily R Newton, MD; Benjamin H Schmidt, MD; Michael O Meyers, MD
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      Duodenal Malignancies

      • EMILY R NEWTON, MDGeneral Surgery Resident, Department of Surgery, Division of Surgical Oncology, University of North Carolina
      • BENJAMIN H SCHMIDT, MDSurgical Oncology Fellow, Department of Surgery, Division of Surgical Oncology, University of North Carolina
      • MICHAEL O MEYERS, MDProfessor of Surgery, Department of Surgery, Division of Surgical Oncology, University of North Carolina, Lineberger Comprehensive Cancer Center, University of North Carolina

      Although malignancies involving the small bowel are rare, one-third of these are located in the duodenum. The majority of duodenal tumors are adenocarcinoma but also may include gastrointestinal stromal tumors (GIST), carcinoid or neuroendocrine tumors, sarcomas, and lymphoma. These commonly present with nonspecific symptoms, but obstructive patterns predominate when symptoms are present. Preoperative diagnosis is made via endoscopy and/or cross-sectional imaging. This section focuses on treatment and surgical management for adenocarcinoma, carcinoid tumors, and GISTs of the duodenum. Surgical resection is the primary treatment of for all three of these, but all have significant nuances in surgical planning and decision-making as well as variability in the role of adjunctive treatment in their management. Functional carcinoid tumors can have hormone-driven symptoms and are associated with an increase in risk of carcinoid crisis, which may be prophylactically treated with intravenous octreotide. Resection of these tumors relies heavily on tumor relationship to the ampulla. Key anatomic distinctions and clinical tips to identify the ampulla to ensure an appropriate duodenal resection are discussed in this review.

      This review contains 12 figures, 5 tables, and 54 references.

      Key Words: carcinoid, duodenal carcinoma, duodenal adenocarcinoma, duodenal resection, duodenal tumors, neuroendocrine tumor, gastrointestinal stromal tumor, small bowel tumors

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    • 7

      Management of Borderline Resectable and Locally Advanced Pancreatic Adenocarcinoma

      By Francis Igor Macedo, MD; Danny Yakoub, MD, PhD; Vikas Dudeja, MD; Nipun B. Merchant, MD
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      Management of Borderline Resectable and Locally Advanced Pancreatic Adenocarcinoma

      • FRANCIS IGOR MACEDO, MDSurgical Oncology Fellow, Division of Surgical Oncology, Dewitt-Daughtry Department of Surgery, Sylvester Comprehensive Cancer Center, University of Miami Miller School of Medicine, Miami, FL
      • DANNY YAKOUB, MD, PHDAssociate Professor of Surgery, Division of Surgical Oncology, Dewitt-Daughtry Department of Surgery, Sylvester Comprehensive Cancer Center, University of Miami Miller School of Medicine, Miami, FL
      • VIKAS DUDEJA, MDAssistant Professor of Surgery, Division of Surgical Oncology, Dewitt-Daughtry Department of Surgery, Sylvester Comprehensive Cancer Center, University of Miami Miller School of Medicine, Miami, FL
      • NIPUN B. MERCHANT, MDAlan S. Livingstone Professor of Surgery, Chief, Division of Surgical Oncology, Executive Director of Perioperative Services, Director Surgical Oncology Programs, SCCC, Division of Surgical Oncology, Dewitt-Daughtry Department of Surgery, Sylvester Comprehensive Cancer Center, University of Miami Miller School of Medicine, Miami, FL

      The incidence of pancreatic cancer continues to rise, and it is now the third-leading cause of cancer-related deaths in the United States. Only 15 to 20% of patients are eligible to undergo potentially curative resection, as most tumors are deemed unresectable at the time of diagnosis because of either locally advanced disease or distant metastases. Improvements in preoperative CT imaging have enabled better determination of the extent of disease and allowed for better operative planning. Based on their relationship to the surrounding vasculature and structures and presence or absence of distant disease, pancreatic tumors are classified into four categories: resectable, borderline resectable pancreatic cancer (BRPC), locally advanced pancreatic cancer (LAPC), and metastatic. With the recent advent of more effective chemotherapy regimens, efforts have focused on using neoadjuvant therapy approaches to increase the likelihood of achieving an R0 in patients with BRPC and possibly convert unresectable, locally advanced tumors to potentially resectable tumors. Response with neoadjuvant therapy regimens has resulted in increased number of patients eligible for resection, many times requiring vascular resection. Herein, we describe recent changes in the classification, important surgical and pathologic considerations and updated multimodal therapeutic options in the complex management of BRPC and LAPC.

       This review contains 5 figures, 2 tables, and 78 references.

      Key Words: borderline resectable pancreatic cancer, CA 19-9, FOLFIRINOX, locally advanced pancreatic cancer, nab-paclitaxel, neoadjuvant chemotherapy, pancreatectomy, portal vein resection, radiation therapy, gemcitabine

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  • Breast
    • 1

      Diagnostic Breast Imaging and Breast Cancer Staging

      By Nidhi Sharma, MD
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      Diagnostic Breast Imaging and Breast Cancer Staging

      • NIDHI SHARMA, MDStaff, Interventional Breast Imaging, Cleveland Clinic, Cleveland, OH

      This review is geared to provide surgeons practical insight on breast imaging, intended to improve breast cancer detection and staging. Breast cancer is a leading cause of death in women in the United States. The American Joint Committee on Cancer staging system provides a tumor-node-metastasis classification that helps in determining prognosis and patient treatment. There is an increasing role of radiologists in ascertaining the correct cancer stage. Screening mammography is the basic tool and most widely used modality to detect breast cancer. The diagnostic work-up of a patient recalled from screening is the next step in assessing the artifacts and benign findings from more suspicious lesions. Additional mammographic views, tomosynthesis, and ultrasonography play an important role in determining if the finding represents a true lesion and if so, to localize and determine its level of suspicion to be cancer. Breast MRI is used both as a screening tool and a diagnostic modality to help in cancer detection and treatment planning. Recognizing patterns of benign masses, malignant calcifications, architectural distortion, and masses via a multimodality approach is the essential first step in further diagnosis. A quick overview of common interventional breast procedures may serve as a practical reference for the readers.

      This review contains 10 figures, 8 tables, and 39 references.

      Key Words: breast cancer, breast MRI, breast ultrasonography, fibroadenoma, invasive ductal staging, male breast, mammograms, postoperative breast, screening

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    • 2

      Triple-negative Breast Cancer

      By Diane M. Radford, MD; Jame Abraham, MD; Stephen R. Grobmyer, MD, FACS
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      Triple-negative Breast Cancer

      • DIANE M. RADFORD, MDAssociate Professor of Surgery, Lerner College of Medicine, Cleveland Clinic Foundation, Cleveland, OH
      • JAME ABRAHAM, MDDirector, Breast Oncology Program, Taussig Cancer Center, Professor of Medicine, Lerner College of Medicine, Cleveland Clinic Foundation, Cleveland, Ohio
      • STEPHEN R. GROBMYER, MD, FACSProfessor of Surgery, Lerner College of Medicine, Cleveland Clinic Foundation, Cleveland, OH

      Triple-negative breast cancers (TNBCs), negative for estrogen receptor, progesterone receptor, and human epidermal growth factor receptor 2, account for 15 to 20% of all female breast cancers. TNBC is heterogeneous based on gene expression microarray, and identification of TNBC subtypes and their behavior has the potential to enable more targeted, neoadjuvant, and adjuvant interventions. TNBCs usually are higher grade (Nottingham score 3) and are more common in younger, Hispanic, and African American women. They are more aggressive, have an increased likelihood of distant disease and mortality, are larger at presentation, and are more likely to be associated with lymph node metastases. Patients with TNBC are at a higher risk for visceral metastases early in the course of the disease. Genetic risk evaluation is recommended for patients with TNBC diagnosed at or before 60 years of age. Surgical management may be influenced by gene testing results. Standard adjuvant chemotherapy is anthracycline or taxane based.

      This review contains 5 figures, 8 tables, and 51 references.

      Key Words: adjuvant, BRCA, chemotherapy, hormone receptor negative, neoadjuvant, genetics, triple-negative breast cancer, breast neoplasm.

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  • Hepato-pancreato-biliary
    • 1

      Neuroendocrine Liver Metastasis

      By Sean M Ronnekleiv-Kelly, MD; Amir A Rahnemai-Azar, MD; Mary Dillhoff, MD; Carl Schmidt, MD; Sharon M. Weber, MD; Timothy M. Pawlik, MD, MPH, PhD
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      Neuroendocrine Liver Metastasis

      • SEAN M RONNEKLEIV-KELLY, MDAssistant Professor, Division of Surgical Oncology, Department of Surgery, University of Wisconsin School of Medicine and Public Health, Madison, WI
      • AMIR A RAHNEMAI-AZAR, MDDepartment of Surgery, Division of Surgical Oncology, University of Wisconsin Hospital, Madison, WI
      • MARY DILLHOFF, MDDepartment of Surgery, Division of Surgical Oncology, The Ohio State University Wexner Medical Center, Columbus, OH
      • CARL SCHMIDT, MDDepartment of Surgery, Division of Surgical Oncology, The Ohio State University Wexner Medical Center, Columbus, OH
      • SHARON M. WEBER, MDDepartment of Surgery, Division of Surgical Oncology, University of Wisconsin Hospital, Madison, WI
      • TIMOTHY M. PAWLIK, MD, MPH, PHDProfessor and Chair, The Urban Meyer III and Shelley Meyer Chair for Cancer Research, Department of Surgery Wexner Medical Center, Ohio State University

      Liver is the most common site of distant metastasis in patients with advanced gastroenteropancreatic neuroendocrine tumors. Presence of liver disease and grade and location of primary tumor are major determinants of prognosis. The clinical presentation of patients with neuroendocrine liver metastasis (NELM) depends on functional status of the primary tumor as well as severity of the liver disease. Various treatment modalities are available for management of patients with NELM, depending on pattern of metastasis and anatomical origin. Complete surgical resection remains the mainstay of treatment and only hope for cure. Tumor-debulking surgery might be considered in patients with extreme hormonal symptoms refractory to other treatments, with some survival benefits. Orthotopic liver transplantation also has been shown to be an accepted alternative in highly selected patients. Liver-directed treatment strategies such as radiofrequency ablation and hepatic artery-based therapies can be offered to patients who are ineligible for surgical resection. Somatostatin analogs, peptide receptor radiotherapy, cytotoxic chemotherapeutics, and novel molecular-targeted drugs embrace the mainstay of systemic therapy in NELM. In this chapter, we review the clinical presentation, diagnosis, and recent developments in management of patients with NELM.

      This review contains 9 figures, 5 tables, and 50 references.

      Key Words: ablation, chemotherapy, hepatic artery-based therapy, liver metastasis, liver transplantation, molecular-targeted therapy, neuroendocrine tumor, surgical resection

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    • 2

      Colorectal Liver Metastasis

      By Brian E Kadera, MD; Michael D’Angelica, MD
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      Colorectal Liver Metastasis

      • BRIAN E KADERA, MDDivision of Surgical Oncology, Department of Surgery, David Geffen School of Medicine at UCLA, Los Angeles, CA
      • MICHAEL D’ANGELICA, MDAttending Surgeon, Hepatopancreatobiliary Service, Memorial Sloan Kettering Cancer Center, Professor of Surgery, Cornell University, Weill School of Medicine, New York City, NY

      Metastatic colorectal cancer isolated to the liver is a common clinical presentation in the United States, occurring in an estimated 50,000 patients per year. Unlike most stage IV malignancies, surgery is an effective mainstay of therapy. In the past several decades, novel surgical approaches, improved systemic chemotherapy, and locoregional therapies such as ablation and hepatic arterial infusion chemotherapy have broadened the indications for resection. At the same time, advances in perioperative care and adoption of parenchymal-sparing surgical techniques have lowered the perioperative mortality of liver resection to approximately 1%. Surgical cure is possible and using 10-year disease-free survival as a definition, this can be achieved in approximately 20 to 30% of well-selected patients. The majority of patients recur; thus, active surveillance is appropriate to identify patients for potential salvage therapy, including in some cases repeat resections and/or ablation, which is associated with prolonged survival and potential cure. More research is needed in biomarker drivers of prognosis, as there are few reliable clinicopathologic indicators to identify those in whom surgery will not benefit.

      This review contains 7 figures, 7 tables, and 90 references.

      Key Words: colorectal cancer, FOLFOX, FOLFIRI, hepatic arterial infusion, hepatic resection, liver remnant, microwave ablation, portal vein ligation, ALPPS

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    • 3

      Intrahepatic Cholangiocarcinoma

      By Timothy M. Pawlik, MD, MPH, PhD; Jordan M Cloyd, MD
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      Intrahepatic Cholangiocarcinoma

      • TIMOTHY M. PAWLIK, MD, MPH, PHDProfessor and Chair, The Urban Meyer III and Shelley Meyer Chair for Cancer Research, Department of Surgery Wexner Medical Center, Ohio State University
      • JORDAN M CLOYD, MDDepartment of Surgery, The Ohio State University, Columbus, OH

      Intrahepatic cholangiocarcinoma is an aggressive malignancy. For patients who present with localized disease, surgical resection remains the only potentially curative treatment. Similar to the treatment of other liver malignancies, the principle surgical approach for iCCA should be a margin-negative hepatic resection with preservation of a liver remnant of adequate size and function. Regional lymphadenectomy is recommended at the time of hepatectomy due to the importance of nodal involvement on staging and prognosis. Given the substantial recurrence rates observed even after curative-intent resection, perioperative systemic therapy may have value, with recent prospective data suggesting adjuvant capecitabine may be the standard therapy recommended for most patients. For those with metastatic or unresectable disease, systemic chemotherapy and locoregional modalities are recommended. In the future, improved understanding of the genetic and molecular underpinnings of iCCA tumorigenesis will lead to improved targeted therapies and better outcomes for these patients.

      This review contains 4 figures, 1 table and 35 references.

      Key Words: biliary tract cancer, chemotherapy, hepatectomy, intrahepatic cholangiocarcinoma, Klatskin, liver cancer, liver resection, lymphadenectomy, vascular resection

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  • Soft Tissue Sarcoma
    • 1

      Synovial Sarcoma

      By Marcus Tan, MBBS, FACS
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      Synovial Sarcoma

      • MARCUS TAN, MBBS, FACSAssistant Professor, Division of Surgical Oncology, Vanderbilt University Medical Center, Nashville TN

      Synovial sarcomas are a common extremity soft tissue sarcoma, typically arising in the para-articular regions of young adults. All synovial sarcomas are considered high grade. Distinctive clinical features are the two common histologic variants (monophasic and biphasic) and more frequent incidence of nodal metastasis. In addition to the sheets of spindle cells seen in monophasic tumors, biphasic tumors have interspersed areas of glandular differentiation. Synovial sarcomas are characterized by the SS18-SSX translocation. Most biphasic tumors contain the SS18-SSX1 translocation, whereas almost all SS18-SSX2 tumors are monophasic. Radical resection is the mainstay of therapy. Synovial sarcomas are chemosensitive, and ifosfamide-doxorubicin is often used neoadjuvantly. Adjuvant radiation should be considered for tumors greater than 5 cm.

      This review contains 7 figures, 2 tables, and 35 references.

      Key Words: E-cadherin, extremity sarcoma, ifosfamide, neoadjuvant chemotherapy, nomogram, SS18-SSX fusion, synovial sarcoma, translocation-associated sarcoma

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  • Heptobiliary Cancer
    • 1

      Surgical Treatment for Hepatocellular Carcinoma in the Era of Multiple Staging and Algorithm

      By Suguru Yamashita, MD, PhD; Katharina Joechle, MD; Jean-Nicolas Vauthey, MD, FACS
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      Surgical Treatment for Hepatocellular Carcinoma in the Era of Multiple Staging and Algorithm

      • SUGURU YAMASHITA, MD, PHDDepartment of Surgical Oncology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Unit 1484, Houston, TX 77030, USA
      • KATHARINA JOECHLE, MDDepartment of Surgical Oncology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Unit 1484, Houston, TX 77030, USA
      • JEAN-NICOLAS VAUTHEY, MD, FACSDepartment of Surgical Oncology, University of Texas MD Anderson Cancer Center, Houston, TX, USA

      A plethora of staging systems for hepatocellular carcinoma (HCC) has existed, as the management for HCC is made complex by the interplay of tumor characteristics and the health and underlying functions of both the patient and the liver. The majority of patients with HCC have nonsurgical HCC. The Barcelona Clinic Liver Cancer (BCLC) classification has been regarded as the optimal staging system and treatment algorithm for HCC. However, even in patients with intermediate or advanced stage in BCLC classification, who had not been originally recommended to undergo surgery, some could benefit in terms of long-term survival by surgical treatments. An expert panel on HCC has stated that the American Joint Committee on Cancer staging system, whose predictive power on the outcome have been improved by continuous amendments, should be applied for patients undergoing surgery. Herein, we review the recent staging system focusing on patients with HCC undergoing surgery.

      This review contains 5 figures, 3 tables and 34 references

      Key Words: American Joint Committee on Cancer, Barcelona Clinic Liver Cancer, hepatic resection, hepatocellular carcinoma, orthotopic liver transplantation

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    • 2

      Noncolorectal, Nonneuroendocrine Liver Metastasis

      By Lindsay A. Bliss, MD, MPH; Thomas Clark Gamblin, MD, MS, MBA
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      Noncolorectal, Nonneuroendocrine Liver Metastasis

      • LINDSAY A. BLISS, MD, MPHInstructor, Division of Surgical Oncology, Department of Surgery, Medical College of Wisconsin, Milwaukee, WI
      • THOMAS CLARK GAMBLIN, MD, MS, MBAProfessor of Surgery, Chief, Division of Surgical Oncology, Department of Surgery, Medical College of Wisconsin, Milwaukee, WI

      Noncolorectal, nonneuroendocrine metastases represent a heterogeneous group with variable tumor biology. Successes in the use of hepatectomy for colorectal liver metastases has increased interest in resection of noncolorectal, nonneuroendocrine metastases. Cure rates equivalent to those of colorectal liver metastases have not been achieved, but resection of noncolorectal, nonneuroendocrine metastases can often provide improved overall survival compared with systemic therapy alone or exclusively palliative care. Overall survival outcomes can be achieved with acceptable perioperative morbidity and mortality relative to rates for hepatectomies in general. Prospective data regarding the utilization of resection for noncolorectal, nonneuroendocrine metastases are lacking, but retrospective data are encouraging. In appropriately selected patients with favorable tumor biology and excellent performance status, resection of noncolorectal, nonneuroendocrine may offer a survival benefit.

      This review contains 1 figure, 2 tables, and 31 references.

      Key Words: breast cancer, cytoreduction, gastric cancer, GIST, hepatectomy, melanoma, metastasis, ovarian cancer, pancreatic cancer, renal cell carcinoma

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    • 3

      Perihilar Cholangiocarcinoma

      By Michele M. Gage, MD; Matthew J. Weiss, MD
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      Perihilar Cholangiocarcinoma

      • MICHELE M. GAGE, MDDepartment of Surgery, Johns Hopkins University, Baltimore, MA
      • MATTHEW J. WEISS, MDNorthwell Health Cancer Institute, Lake Success, NY

      Hilar cholangiocarcinomas (HCs) are a diverse group of aggressive tumors often diagnosed late in disease due to their typically longitudinal spread along the biliary system. Only approximately one-third of HCs are the candidates for resection, which offers the only chance for cure. However, even following resection, recurrence is common, and 5-year survival rates after surgery remain dismal at 20 to 42%. A thorough preoperative work-up and risk stratification for major surgery are critical to treatment success and maximizing the likelihood of an R0 resection. Due to the relatively few large phase III randomized controlled trials for HC, data are lacking on the optimal adjuvant treatment. Recent results of the BILCAP trial are promising for improved survival after surgery with adjuvant therapy, particularly capecitabine. When resection is not an option, prognosis is poor with median survival of less than 12 months. However, there are multiple chemotherapy-based treatment options that have demonstrated prolonging survival, with combined gemcitabine and cisplatin as first-line therapy. Liver transplantation should be considered on protocol for unresectable HC in the absence of metastatic disease. Palliative options, such as photodynamic therapy or radiation, may also be considered in advanced disease.


      This review contains 2 figures, 5 tables, and 41 references.

      Key Words: adjuvant therapy in cholangiocarcinoma, hilar cholangiocarcinoma, Klatskin tumor, metastatic cholangiocarcinoma, perihilar cholangiocarcinoma, portal dissection, surgery for hilar cholangiocarcinoma, outcomes in hilar cholangiocarcinoma

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