NANETS GUIDELINES 2020 TABLE OF CONTENTS Introduction ............................................................................................................................. 3 NANETS Overview .................................................................................................................... 4 Consensus Guidelines for Surveillance and Medical Management of Pancreatic Neuroendocrine Tumors (2020)................................................. 5 Consensus Paper on the Surgical Management of Pancreatic Neuroendocrine Tumors (2020) ..................................................................... 34 NANETS/SNMMI Consensus Statement on Patient Selection and Appropriate Use of 177Lu-DOTATATEPeptide Receptor Radionuclide Therapy (2020) ............................. 83 NANETS/SNMMI Procedure Standard for Somatostatin Receptor Based Peptide Receptor Radionuclide Therapy with 177Lu-DOTATATE (2019) ............................... 94 The Surgical Management of Small Bowel Neuroendocrine Tumors (2017) ..................................................................... 105 Consensus Guidelines for Surveillance and Medical Management of Midgut Neuroendocrine Tumors (2017) ................................................... 131 Appropriate Use Criteria for Somatostatin Receptor PET Imaging in Neuroendocrine Tumors (Joint with SNMMI, 2017) .................................. 143 Consensus Guidelines for the Management and Treatment of Neuroendocrine Tumors (2013)............................................................. 157 NANETS Guidelines: Mission, Goals and Process (2010) .................................................... 195 2 INTRODUCTION Dear NET Community, It is with great pleasure that we present this inaugural edition of NANETS’ Consensus Guidelines. NANETS is a leader in NET education and is actively involved in the development of consensus guidelines to support the medical treatment and management of NET disease. NANETS’ Consensus Guidelines serve as important references for practicing physicians. The Guidelines and Publications Committee coordinates the process of developing consensus guidelines using rigorous literature review conducted by content-specific NET experts serving on consensus panels. Since 2013, NANETS has published 8 guidelines on pancreatic NETs, small bowel NETs, peptide receptor radionuclide therapy with 177Lu-dotatate, and somatostatin receptor PET imaging. Two panels are completing their work on guidelines for pheochromocytoma/paraganglioma and high-grade neuroendocrine neoplasms. We invite you to visit our online library at https://nanets.net/net-guidelines-library as we continue to publish and post new consensus guidelines. We wish to thank all of the committee members and panelists who have contributed to this important work over the years. Sincerely, James R. Howe, MD Jennifer A. Chan, MD, MPH Board President Chair, Guidelines and University of Iowa Carver Publications Committee College of Medicine Dana-Farber Cancer Institute Lauren Fishbein, MD, PhD, MTR Thorvardur R. Halfdanarson, MD Co-Chair, Guidelines and Past Chair, Guidelines and Publications Committee Publications Committee University of Colorado Mayo Clinic School of Medicine NANETS 2020 GUIDELINES 3 NANETS OVERVIEW The North American Neuroendocrine Tumor Society’s (NANETS) mission is to improve outcomes for patients with neuroendocrine tumors through multidisciplinary medical education, NET related medical research, publication of guidelines and the exchange of knowledge and innovation. The Exchange of Knowledge and Innovation — The Cornerstone of NANETS’ Mission NANETS is committed to furthering neuroendocrine tumor disease diagnosis, treatment and management through research and education. With over 15,000 new patients diagnosed with NETs each year, there is a continuing need to support NET research and advance the dissemination of vital research findings. NANETS offers a robust educational programming series through regional NET education confer- ences, an annual symposium, and an online webinars. These educational opportunities are presented by well-known leaders and experts in the field, exclusively for medical professionals. NANETS supports NET related medical research through annual research grants to early career professionals in basic/translational science, clinical investigation and theranostics. These annual grants support innovative research initiatives and encourage young researchers and scientific clinicians to become NET specialists. NANETS’ Annual Multidisciplinary NET Medical Symposium provides a forum for the exchange of knowledge and innovation among researchers and clinicians within the NET field as well as related fields. NANETS curates abstracts from researchers at institutions in North America and around the world. Through abstract presentations and interactive poster sessions, NANETS showcases cutting edge topics in neuroendocrine tumor research and current clinical trials. Abstracts are compiled in a yearly publication and serve as a comprehensive guide to the latest in NET research. Guidelines Form the Foundation of NANETS To begin educating medical professionals on NET disease, founding members of NANETS understood the importance of consensus guidelines. The NANETS Guidelines Working Group was created and from 2008-2010, hours of effort were poured into launching this much-lauded initiative. The first consensus guidelines focused on critical elements of NET disease management by organ – including the develop- ment of position papers and collaborative feedback, with the first consensus conference held on October 2, 2008. This meeting generated intense discussions resulting in the publication of NANETS’ first consen- sus guidelines: The NANETS Consensus Guidelines for the Diagnosis of Neuroendocrine Tumors (p. 157).1 Since that initial meeting and publication, NANETS’ has published 7 additional consensus guidelines includ- ing joint consensus guidelines with the Society of Nuclear Medicine and Molecular Imaging (SNMMI). Several more guidelines will be published in 2021. The Guidelines and Publications Committee seeks the input from members on guidelines topics through an annual survey. To submit topics, email [email protected]. 1See p. 195 for more history of the formation of NANETS and the guidelines process. 4 The North American Neuroendocrine Tumor Society Consensus Guidelines for Surveillance and Medical Management of Pancreatic Neuroendocrine Tumors Thorvardur R. Halfdanarson, MD,* Jonathan R. Strosberg, MD,† Laura Tang, MD, PhD,‡ Andrew M. Bellizzi, MD,§ Emily K. Bergsland, MD,|| Thomas M. O’Dorisio, MD,¶ Daniel M. Halperin, MD,# Lauren Fishbein, MD, PhD,** Jennifer Eads, MD,†† Thomas A. Hope, MD,‡‡ Simron Singh, MD,§§ Riad Salem, MD,|||| David C. Metz, MD,¶¶ Boris G. Naraev, MD, PhD,## Diane L. Reidy-Lagunes, MD,*** James R. Howe, MD,††† Rodney F. Pommier, MD,§§§ Yusuf Menda, MD,|||||| and Jennifer A. Chan, MD, MPH¶¶¶ From the *Mayo Clinic Cancer Center, Division of Medical Accelerator Applications, Lexicon, Curium, and TERUMO, and Oncology, Rochester, MN; †Department of Gastrointestinal research support from Thermo Fisher Scientific, and Ipsen. J.R.S. Oncology, Moff itt Cancer Center, Tampa, FL; ‡Department has received consulting fees from Novartis, and is a member of of Pathology, Memorial Sloan Kettering Cancer Center, New the speaker’s bureau of Lexicon and Ipsen. E.K.B. has received York, NY; §Department of Pathology, University of Iowa Carver research support from Novartis and Merck and consulting fees College of Medicine, Iowa City, IA; ||Department of Medicine, from AAA, Ipsen, and Hutchison MediPharma (all unpaid). D.M.H. University of California, San Francisco, San Francisco, CA; has received consulting fees from Ipsen, Advanced Accelerator ¶Department of Medicine, Division of Endocrinology, University Applications, Lexicon, Novartis, AbbVie, and Curium, and research of Iowa Carver College of Medicine, Iowa City, IA; #Department support from Genentech/Roche, Tarveda Therapeutics, Thermo of Gastrointestinal Medical Oncology, University of Texas MD Fisher Scientific, Incyte, Lexicon, and Advanced Accelerator Anderson Cancer Center, Houston, TX; **Department of Medicine, Applications. J.E. has received consulting fees from Lexicon Division of Endocrinology, Metabolism and Diabetes, Division of (ongoing), Novartis (within the past 2 years), has an employment Biomedical Informatics and Personalized Medicine, University status in Bristol Meyers Squibb (spouse), has stocks in Bristol of Colorado School of Medicine, Aurora, CO; ††Division of Meyers Squibb (pending), and has received honoraria from Hematology and Oncology, Abramson Cancer Center, University Pfizer (talk). T.A.H. has received consulting fees from and is a of Pennsylvania, Philadelphia, PA; ‡‡Department of Radiology member of the advisory board of Ipsen, and Curium; has received and Biomedical Imaging, University of California, San Francisco, research support from Clovis Oncology, Philips, and Advanced San Francisco, CA; §§Department of Medical Oncology, Odette Accelerator Applications. S.S. has received consulting fees Cancer Centre, Sunnybrook Health Sciences Centre, Toronto, from Novartis and Ipsen and research support from Novartis. Ontario, Canada; ||||Department of Radiology, Section of R.S. has received consulting fees from Cook, Boston Scientific, Interventional Radiology, Northwestern University, Chicago Genentech, Eisai, and Becton-Dickinson. D.C.M. has received IL; ¶¶Division of Gastroenterology,