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The American Society of Colon and Rectal Surgeons, Clinical Practice Guidelines for the Management of Appendiceal Neoplasms

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The American Society of Colon and Rectal Surgeons, Clinical Practice Guidelines for the Management of Appendiceal Neoplasms CLINICAL PRACTICE GUIDELINES The American Society of Colon and Rectal Surgeons, Clinical Practice Guidelines for the Management of Appendiceal Neoplasms 12/16/2019 on BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AWnYQp/IlQrHD3bhnalqTQiPuOeErObcGkYc0C+sRFRc02Kq2wNkH0jKo= by https://journals.lww.com/dcrjournal from Downloaded Sean C. Glasgow, M.D.1 • Wolfgang Gaertner, M.D.2 • David Stewart, M.D.3 4 4 5 Downloaded Jennifer Davids, M.D. • Karim Alavi, M.D. • Ian M. Paquette, M.D. Scott R. Steele, M.D., M.B.A.6 • Daniel L. Feingold, M.D.7 from https://journals.lww.com/dcrjournal 1 Department of Surgery, Washington University School of Medicine, St. Louis, Missouri 2 Department of Surgery, University of Minnesota, Minneapolis, Minnesota 3 Department of Surgery, University of Arizona, Phoenix, Arizona 4 Department of Surgery, University of Massachusetts, Worcester, Massachusetts 5 Department of Surgery, University of Cincinnati, Cincinnati, Ohio 6 Department of Surgery, Cleveland Clinic, Cleveland, Ohio 7 Department of Surgery, Rutgers University, New Brunswick, New Jersey by BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AWnYQp/IlQrHD3bhnalqTQiPuOeErObcGkYc0C+sRFRc02Kq2wNkH0jKo= PREPARED ON BEHALF OF THE CLINICAL PRACTICE GUIDELINES COMMITTEE OF THE AMERICAN SOCIETY OF COLON AND RECTAL SURGEONS. he American Society of Colon and Rectal Surgeons It should be recognized that these guidelines should is dedicated to ensuring high-quality patient care not be deemed inclusive of all proper methods of care nor Tby advancing the science, prevention, and manage- exclusive of methods of care reasonably directed toward ment of disorders and diseases of the colon, rectum, and obtaining the same results. The ultimate judgment regard- anus. The Clinical Practice Guidelines Committee is com- ing the propriety of any specific procedure must be made posed of society members who are chosen because they by the physician in light of all the circumstances presented have demonstrated expertise in the specialty of colon and by the individual patient. rectal surgery. This Committee was created to lead interna- tional efforts in defining quality care for conditions related to the colon, rectum, and anus. This is accompanied by the STATEMENT OF THE PROBLEM development of clinical practice guidelines based on the Historically, the estimated incidence of appendiceal tu- best available evidence. These guidelines are inclusive but mors was 0.12 cases per 1,000,000 people per year; how- not prescriptive. Their purpose is to provide information ever, based on recent large database studies, the incidence to support decision-making rather than to dictate a spe- may be as high as 0.97 per 100,000 population.1–3 It is on cific form of treatment. These guidelines are intended for 12/16/2019 unclear whether this increase reflects an actual change the use of all practitioners, healthcare workers, and patients in the disease occurrence or simply greater recognition who desire information about the management of the con- and reporting. Although tumors of the appendix are rare, ditions addressed by the topics covered in these guidelines. surgeons should be familiar with the implications of ap- pendiceal pathology, because almost 300,000 appendec- Funding/Support: None reported. tomies are performed annually in the United States, and 4–6 Financial Disclosure: None reported. neoplasia is found in ≈1% to 2% of these specimens. Correspondence: Daniel L. Feingold, M.D., Rutgers Robert Wood John- son Medical School New Brunswick, 125 Paterson St, New Brunswick, CLASSIFICATION BY HISTOPATHOLOGY NJ 08901. E-mail: [email protected] Given the rarity and multiple different terms used to de- Dis Colon Rectum 2019; 62: 1425–1438 scribe appendiceal neoplasms, consistency regarding their DOI: 10.1097/DCR.0000000000001530 classification not only allows for improved reporting but © The ASCRS 2019 also for more precise management. In general terms, ap- DISEASES OF THE COLON & RECTUM VOLUME 62: 12 (2019) 1425 Copyright © The American Society of Colon & Rectal Surgeons, Inc. Unauthorized reproduction of this article is prohibited. 1426 GLASGOW ET AL: ASCRS GUIDELINES FOR APPENDICEAL NEOPLASMS pendiceal neoplasms can be broadly described as epithelial, TABLE 1. Histologic classification of PMP such as adenomas or adenocarcinomas, or nonepithelial (eg, neuroendocrine or lymphoma). The epithelial group is Pathologic lesion Criteria often further subdivided based on mucin production, be- Acellular mucin • Mucin within peritoneal cavity cause mucinous tumors have distinctly different biologic without neoplastic epithelial cells behavior and oncologic outcomes from nonmucinous Low-grade mucinous • Epithelial component typically scanty 4 carcinoma peritonei • Minimal cytological atypia neoplasms. The World Health Organization classifies the (DPAM) • Strips, gland-like structures or small majority of noninvasive epithelial lesions as low-grade ap- cell clusters pendiceal mucinous neoplasms (LAMNs).7 Histologically, High-grade mucinous • Relatively more cellular LAMNs are characterized by well-differentiated adenomas carcinoma peritonei • Cribriform growth pattern that can proliferate outside the appendix in a malignant (PMCA) • High-grade cytological atypia • Numerous mitoses fashion. Acellular or cellular extra-appendiceal mucin may High-grade mucinous • Any lesion with a signet ring cell be associated with LAMNs, although this is not a require- carcinoma peritonei component, that is, round cells with ment. The LAMN terminology includes lesions that were with signet rings cells intracytoplasmic mucin pushing described previously as mucoceles or mucinous cystadeno- (PMCA-S) nucleus against cell membrane mas, which are terms no longer in use. Some authors have Adapted from Carr et al.9 suggested an intermediate grouping between traditional DPAM = disseminated peritoneal adenomucinosis; PMCA = peritoneal mucinous carcinomatosis; PMCA-S = peritoneal mucinous carcinomatosis with signet ring 8 LAMNs and invasive carcinoma. These LAMNs of un- cells; PMP = pseudomyxoma peritonei. certain malignant potential may exhibit gross perforation, mural fibrosis, mucin dissecting within the appendiceal porting classification was published recently for both PMP wall, or acellular mucin in the periappendiceal soft tissues. and appendiceal neoplasia.14 The authors recommended High-grade appendiceal neoplasms (HAMNs) share some categorizing PMP based on the degree of cellularity within histologic features with LAMNs but exhibit more aggres- the mucin as follows: acellular, low-grade histologic fea- sive cytologic atypia. The distinct biological and clinical tures, high-grade histologic features, and PMP with sig- behaviors of HAMNs are poorly characterized.9 net ring cells (Table 1). The low-grade group includes Appendiceal adenocarcinomas may be either muci- the commonly reported term of disseminated peritoneal nous or nonmucinous. Mucinous adenocarcinomas are adenomucinosis, whereas peritoneal mucinous carcino- characterized by invasive glands containing high-grade matosis is designated as high grade. Because the group- cytologic atypia and extracellular mucin in >50% of the ing is based on histology, clinical features such as omental lesion.7 Appendiceal adenocarcinomas resemble their co- caking or ovarian involvement may represent either low- lorectal counterparts histologically, regularly expressing or high-grade PMP. This classification aligns with other p53, CD44, and CDX2. They often demonstrate signet schemes and helps determine treatment and prognosis.17,18 ring cells if poorly differentiated, are prone to lymphatic Nonepithelial appendiceal neoplasms include NETs. spread, and are staged according to the TNM classifica- These lesions are histologically similar to those found else- tion. Goblet cell carcinoid tumors represent a variant of where in the GI tract.7 Appendiceal NETs are frequently adenocarcinoma that demonstrates some features similar asymptomatic and identified incidentally after routine to traditional neuroendocrine tumors (NETs; eg, positive appendectomy. Staging remains controversial and may be chromogranin A staining).7 However, these mixed adeno- based on tumor size, depth of invasion, or degree of differ- neuroendocrine carcinomas are more aggressive than tra- entiation. Other rare nonepithelial appendiceal neoplasms ditional NETs and should generally be treated in a similar include GI stromal tumors, lymphomas, and neural prolif- manner to classic appendiceal adenocarcinomas.10,11 erations, which are not considered in this guideline. Appendiceal neoplasms may perforate and spread 12 throughout the peritoneal cavity. When this spread in- METHODOLOGY cludes abundant mucin production, the term pseudo- myxoma peritonei (PMP) is used. Some authors make the Selected members of the ASCRS Clinical Practice Guide- distinction that PMP represents a clinical finding rather lines committee drafted de novo position statements after than a diagnosis and should be reserved for diffuse spread performing a thorough search and review of the relevant of mucin throughout the abdomen as opposed to mucin literature. With input from the authors, a professional deposits that are confined adjacent to the appendix.9 Be- librarian conducted a systematic literature search en- cause PMP often recurs after treatment and the 10-year compassing January 1, 1997, to April 30, 2019, inclusive, overall survival (OS) rate after surgery for PMP is 63%, across the Ovid Medline, Embase, and Scopus medical
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