RT Book, Section A1 Maxwell, Jessica E. A1 Yao, James C. A1 Halperin, Daniel M. A2 Kantarjian, Hagop M. A2 Wolff, Robert A. A2 Rieber, Alyssa G. SR Print(0) ID 1190836105 T1 Neuroendocrine Tumors T2 The MD Anderson Manual of Medical Oncology, 4e YR 2022 FD 2022 PB McGraw Hill Education PP New York, NY SN 9781260467642 LK accessmedicine.mhmedical.com/content.aspx?aid=1190836105 RD 2024/03/28 AB KEY CONCEPTSGastroenteropancreatic neuroendocrine tumors (GEP-NETs) are increasing in incidence, but delay in diagnosis is common. Patients may present with specific symptoms related to a functional tumor or vague gastrointestinal symptoms, emphasizing the importance of maintaining a high index of suspicion for this disease.The majority of GEP-NETs arise sporadically. Multiple endocrine neoplasia type 1 is the most common inherited syndrome in which nonfunctional pancreatic neuroendocrine tumors (PNETs) predominate.68Ga tetraazacyclododecanetetraacetic acid-DPhe1-Tyr3-octreotate (68Ga-DOTATATE) has excellent sensitivity and specificity for GEP-NETs. It is used to detect the location of unknown primary tumors and to assess for distant disease. It is not used for routine surveillance.Surgical resection is the standard of care for localized GEP-NETs. In advanced disease, surgery may be appropriate when R0 resection can be achieved, or cytoreduction is performed for palliation of symptoms.In cases in which hepatic metastases are unresectable, hepatic arterial embolotherapies may be used. Peptide receptor radionuclide therapy is efficacious for hepatic and extrahepatic metastases.Systemic therapy for advanced extrapancreatic NETs includes somatostatin analogues and the targeted therapy everolimus. In PNETs, FAS (5-fluorouracil, doxorubicin, and streptozocin), capecitabine and temozolomide, and sunitinib are additional options.