ASO Author Reflections: Low-Grade Appendiceal Mucinous Neoplasms: An Appeal for Standardization and Collaborative Care

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ASO AUTHOR REFLECTIONS

ASO Author Reflections: Low-Grade Appendiceal Mucinous Neoplasms: An Appeal for Standardization and Collaborative Care Alexandra C. Istl, MD, MPH

, Jonathan B. Greer, MD, and Fabian M. Johnston, MD, MHS

Department of Surgery, Johns Hopkins University, Baltimore, MD

PAST Although typically indolent, the spectrum of presentation for low-grade appendiceal mucinous neoplasms (LAMN) presents a challenge for community surgeons and experts alike. LAMN are often first identified by community general surgeons, but evidence to guide clinical decision making in this setting has been unavailable until recently. It is established that all appendiceal neoplasms should be reviewed at high-volume centers, in part because changes commonly occur in both the pathologic diagnosis and management plan following expert review.1 However, there is still widespread misconception that all appendiceal neoplasms have a poor prognosis, which may lead to overtreatment in the form of aggressive upfront resection in the community. Unfortunately, even following referral to specialists, the employment of additional resections, cytoreductive surgery (CRS), and hyperthermic intraperitoneal chemotherapy (HIPEC) is neither standardized nor consistent. As was recently highlighted by the European consensus guidelines (Online ahead of print), there is a conspicuous lack of high-quality, multi-institutional studies to guide management.2 Furthermore, these guidelines demonstrate that even among experts, opinions still vary significantly with respect to some facets of treatment. The goal of our study was to better describe the state of LAMN

Ó Society of Surgical Oncology 2020 First Received: 26 October 2020 Accepted: 26 October 2020 F. M. Johnston, MD, MHS e-mail: [email protected]

management nationally and internationally and identify areas of practice where greater consensus and consistency are needed. PRESENT In our survey of national and international surgeons performing CRS and HIPEC, we evaluated management preferences for patients with LAMN. At least half of respondents’ referrals had undergone prior surgery for their LAMN, of which 20% had undergone a right hemicolectomy (RH) that would not have been recommended by either the study respondents or published guidelines.2–4 For referred LAMN that had not undergone surgery in the community, 85.7% of surgeon respondents reported they would perform an appendectomy alone if LAMN was confined to the appendix. However, when probed on management in the setting of a positive margin, one-third of respondents reported they would proceed with RH.4 While margin status was not addressed in the European guidelines, the Chicago Consensus on peritoneal surface malignancies recommended against RH when a positive margin is identified.3 Instead, they advocated taking an additional cuff at the base of the appendix or performing a partial cecectomy in an effort to avoid an anastomosis.3 This divergence between the new consensus guidelines and specialist practice in our study’s findings further de