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NCCN Guidelines Insights: Neuroendocrine and Adrenal Tumors, Version 2.2018

Manisha H. Shah, Whitney S. Goldner, Thorvardur R. Halfdanarson, Emily Bergsland, Jordan D. Berlin, Daniel Halperin, Jennifer Chan, Matthew H. Kulke, Al B. Benson III, Lawrence S. Blaszkowsky, Jennifer Eads, Paul F. Engstrom, Paul Fanta, Thomas Giordano, Jin He, Martin J. Heslin, Gregory P. Kalemkerian, Fouad Kandeel, Sajid A. Khan, Wajih Zaheer Kidwai, Pamela L. Kunz, Boris W. Kuvshinoff II, Christopher Lieu, Venu G. Pillarisetty, Leonard Saltz, Julie Ann Sosa, Jonathan R. Strosberg, Craig A. Sussman, Nikolaos A. Trikalinos, Nataliya A. Uboha, Jonathan Whisenant, Terence Wong, James C. Yao, Jennifer L. Burns, Ndiya Ogba, and Griselda Zuccarino-Catania

The NCCN Guidelines for Neuroendocrine and Adrenal Tumors provide recommendations for the management of adult patients with neuroendocrine tumors (NETs), adrenal gland tumors, pheochromocytomas, and paragangliomas. Management of NETs relies heavily on the site of the primary NET. These NCCN Guidelines Insights summarize the management options and the 2018 updates to the guidelines for locoregional advanced disease, and/or distant metastasis originating from gastrointestinal tract, bronchopulmonary, and thymus primary NETs.

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The Pancreatic Cancer Early Detection (PRECEDE) Study is a Global Effort to Drive Early Detection: Baseline Imaging Findings in High-Risk Individuals

George Zogopoulos, Ido Haimi, Shenin A. Sanoba, Jessica N. Everett, Yifan Wang, Bryson W. Katona, James J. Farrell, Aaron J. Grossberg, Salvatore Paiella, Kelsey A. Klute, Yan Bi, Michael B. Wallace, Richard S. Kwon, Elena M. Stoffel, Raymond C. Wadlow, Daniel A. Sussman, Nipun B. Merchant, Jennifer B. Permuth, Talia Golan, Maria Raitses-Gurevich, Andrew M. Lowy, Joy Liau, Joanne M. Jeter, James M. Lindberg, Daniel C. Chung, Julie Earl, Teresa A. Brentnall, Kasmintan A. Schrader, Vivek Kaul, Chenchan Huang, Hersh Chandarana, Caroline Smerdon, John J. Graff, Fay Kastrinos, Sonia S. Kupfer, Aimee L. Lucas, Rosalie C. Sears, Randall E. Brand, Giovanni Parmigiani, Diane M. Simeone, and on behalf of the PRECEDE Consortium

Background: Pancreatic adenocarcinoma (PC) is a highly lethal malignancy with a survival rate of only 12%. Surveillance is recommended for high-risk individuals (HRIs), but it is not widely adopted. To address this unmet clinical need and drive early diagnosis research, we established the Pancreatic Cancer Early Detection (PRECEDE) Consortium. Methods: PRECEDE is a multi-institutional international collaboration that has undertaken an observational prospective cohort study. Individuals (aged 18–90 years) are enrolled into 1 of 7 cohorts based on family history and pathogenic germline variant (PGV) status. From April 1, 2020, to November 21, 2022, a total of 3,402 participants were enrolled in 1 of 7 study cohorts, with 1,759 (51.7%) meeting criteria for the highest-risk cohort (Cohort 1). Cohort 1 HRIs underwent germline testing and pancreas imaging by MRI/MR-cholangiopancreatography or endoscopic ultrasound. Results: A total of 1,400 participants in Cohort 1 (79.6%) had completed baseline imaging and were subclassified into 3 groups based on familial PC (FPC; n=670), a PGV and FPC (PGV+/FPC+; n=115), and a PGV with a pedigree that does not meet FPC criteria (PGV+/FPC–; n=615). One HRI was diagnosed with stage IIB PC on study entry, and 35.1% of HRIs harbored pancreatic cysts. Increasing age (odds ratio, 1.05; P<.001) and FPC group assignment (odds ratio, 1.57; P<.001; relative to PGV+/FPC–) were independent predictors of harboring a pancreatic cyst. Conclusions: PRECEDE provides infrastructure support to increase access to clinical surveillance for HRIs worldwide, while aiming to drive early PC detection advancements through longitudinal standardized clinical data, imaging, and biospecimen captures. Increased cyst prevalence in HRIs with FPC suggests that FPC may infer distinct biological processes. To enable the development of PC surveillance approaches better tailored to risk category, we recommend adoption of subclassification of HRIs into FPC, PGV+/FPC+, and PGV+/FPC– risk groups by surveillance protocols.

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Neuroendocrine and Adrenal Tumors, Version 2.2021, NCCN Clinical Practice Guidelines in Oncology

Manisha H. Shah, Whitney S. Goldner, Al B. Benson III, Emily Bergsland, Lawrence S. Blaszkowsky, Pamela Brock, Jennifer Chan, Satya Das, Paxton V. Dickson, Paul Fanta, Thomas Giordano, Thorvardur R. Halfdanarson, Daniel Halperin, Jin He, Anthony Heaney, Martin J. Heslin, Fouad Kandeel, Arash Kardan, Sajid A. Khan, Boris W. Kuvshinoff II, Christopher Lieu, Kimberly Miller, Venu G. Pillarisetty, Diane Reidy, Sarimar Agosto Salgado, Shagufta Shaheen, Heloisa P. Soares, Michael C. Soulen, Jonathan R. Strosberg, Craig R. Sussman, Nikolaos A. Trikalinos, Nataliya A. Uboha, Namrata Vijayvergia, Terence Wong, Beth Lynn, and Cindy Hochstetler

The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Neuroendocrine and Adrenal Gland Tumors focus on the diagnosis, treatment, and management of patients with neuroendocrine tumors (NETs), adrenal tumors, pheochromocytomas, paragangliomas, and multiple endocrine neoplasia. NETs are generally subclassified by site of origin, stage, and histologic characteristics. Appropriate diagnosis and treatment of NETs often involves collaboration between specialists in multiple disciplines, using specific biochemical, radiologic, and surgical methods. Specialists include pathologists, endocrinologists, radiologists (including nuclear medicine specialists), and medical, radiation, and surgical oncologists. These guidelines discuss the diagnosis and management of both sporadic and hereditary neuroendocrine and adrenal tumors and are intended to assist with clinical decision-making. This article is focused on the 2021 NCCN Guidelines principles of genetic risk assessment and counseling and recommendations for well-differentiated grade 3 NETs, poorly differentiated neuroendocrine carcinomas, adrenal tumors, pheochromocytomas, and paragangliomas.