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Sujata Narayanan and Pamela L. Kunz

Neuroendocrine tumors (NETs) are rare epithelial neoplasms with neuroendocrine differentiation that most commonly originate in the lungs and gastrointestinal tract. Many patients have advanced disease not amenable to surgery or local management. Some tumors also secrete amines, such as serotonin, that lead to syndromes of hormone excess, such as diarrhea and flushing. Thus, management of patients with NETs often requires a dual approach, including hormone symptom management and systemic tumor control. Somatostatin analogues have long been a mainstay of managing the hormone-related symptoms, and increasing evidence also supports their use for tumor control in patients with well-differentiated NETs. This article reviews the role of somatostatin analogues in the treatment of NETs.

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M. Zach Koontz, Brendan M. Visser and Pamela L. Kunz

A 36-year-old woman presented to the emergency department with black stools and syncope. Her hemoglobin was 7.0 and her red blood cells were microcytic. Upper endoscopy did not identify a clear source of bleeding, but a bulge in the third portion of the duodenum was noted. A CT scan showed a large extraintestinal mass, and follow-up esophagogastroduodenoscopy/endoscopic ultrasound with biopsy revealed a spindle cell neoplasm, consistent with gastrointestinal stromal tumor (GIST). Because of the size of the lesion and association with the superior mesenteric vein and common bile duct, she was referred to medical oncology for consideration of neoadjuvant imatinib. Neoadjuvant tyrosine kinase inhibitor therapy for GISTs is emerging as a viable treatment strategy for borderline resectable tumors, although the dose, duration, and optimal imaging modalities have not been clearly established. Recent pathologic and radiographic data have provided insight into the mechanism and kinetics of this approach. This case report presents a patient for whom surgery was facilitated using neoadjuvant imatinib.

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Somasundaram Subramaniam, Jason A. Zell and Pamela L. Kunz

Everolimus is an mTOR inhibitor commonly used to treat metastatic pancreatic neuroendocrine tumors (pNETs) and renal cell carcinoma, and for posttransplant immunosuppression. This report presents a case of a 36-year-old man being treated with everolimus for a metastatic pNET who developed severe hypertriglyceridemia and acute pancreatitis. The incidence of hypertriglyceridemia reported in large prospective randomized trials is reviewed and the management of hypertriglyceridemic pancreatitis is discussed. Careful monitoring of triglyceride levels and dose adjustments of everolimus together with lipid-lowering therapy can allow patients to continue this medication. Because there are increasing indications for the use of everolimus, prescribing oncologists must be cognizant of the common and serious side effects.

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Matthew H. Kulke, Manisha H. Shah, Al B. Benson III, Emily Bergsland, Jordan D. Berlin, Lawrence S. Blaszkowsky, Lyska Emerson, Paul F. Engstrom, Paul Fanta, Thomas Giordano, Whitney S. Goldner, Thorvardur R. Halfdanarson, Martin J. Heslin, Fouad Kandeel, Pamela L. Kunz, Boris W. Kuvshinoff II, Christopher Lieu, Jeffrey F. Moley, Gitonga Munene, Venu G. Pillarisetty, Leonard Saltz, Julie Ann Sosa, Jonathan R. Strosberg, Jean-Nicolas Vauthey, Christopher Wolfgang, James C. Yao, Jennifer Burns and Deborah Freedman-Cass

Neuroendocrine tumors (NETs) comprise a broad family of tumors that may or may not be associated with symptoms attributable to hormonal hypersecretion. The NCCN Clinical Practice Guidelines in Oncology for Neuroendocrine Tumors discuss the diagnosis and management of both sporadic and hereditary NETs. This selection from the guidelines focuses on sporadic NETs of the pancreas, gastrointestinal tract, lung, and thymus.

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Matthew H. Kulke, Al B. Benson III, Emily Bergsland, Jordan D. Berlin, Lawrence S. Blaszkowsky, Michael A. Choti, Orlo H. Clark, Gerard M. Doherty, James Eason, Lyska Emerson, Paul F. Engstrom, Whitney S. Goldner, Martin J. Heslin, Fouad Kandeel, Pamela L. Kunz, Boris W. Kuvshinoff II, Jeffrey F. Moley, Venu G. Pillarisetty, Leonard Saltz, David E. Schteingart, Manisha H. Shah, Stephen Shibata, Jonathan R. Strosberg, Jean-Nicolas Vauthey, Rebekah White, James C. Yao, Deborah A. Freedman-Cass and Mary A. Dwyer

Neuroendocrine tumors comprise a broad family of tumors, the most common of which are carcinoid and pancreatic neuroendocrine tumors. The NCCN Neuroendocrine Tumors Guidelines discuss the diagnosis and management of both sporadic and hereditary neuroendocrine tumors. Most of the recommendations pertain to well-differentiated, low- to intermediate-grade tumors. This updated version of the NCCN Guidelines includes a new section on pathology for diagnosis and reporting and revised recommendations for the surgical management of neuroendocrine tumors of the pancreas.

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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.