TY - CHAP M1 - Book, Section TI - Neuroendocrine Tumors A1 - Halperin, Daniel M. A1 - Yao, James C. A2 - Kantarjian, Hagop M. A2 - Wolff, Robert A. Y1 - 2016 N1 - T2 - The MD Anderson Manual of Medical Oncology, 3e AB - Neuroendocrine tumors (NETs) originate from enterochromaffin cells distributed throughout the body. This chapter focuses on low- to intermediate-grade gastroenteropancreatic NETs (GEP-NETs), although the term neuroendocrine tumor also denotes diseases such as small cell carcinoma, medullary thyroid carcinoma, neuroblastoma, and Merkel cell tumor. Pancreatic NETs (PNETs), previously known as islet cell carcinomas, arise from pancreatic ductal progenitors. Extrapancreatic low- to intermediate-grade NETs are generally called carcinoids and most often originate along the aerodigestive tract. These tumors share the capacity for hormone production and usually have an indolent clinical course. Presenting symptoms are caused by secreted hormones, local tumor growth, and/or metastasis. Surgical resection is the curative approach for localized disease. In unresectable or metastatic disease, long-acting somatostatin analogues improve quality of life and progression-free survival. In PNETs, recent randomized studies support the use of targeted therapies such as everolimus and sunitinib, with older prospective and retrospective data supporting the use of alkylating chemotherapy such as streptozocin and temozolomide. This chapter presents a comprehensive overview of the diagnosis and management of pancreatic and extrapancreatic NETs. SN - PB - McGraw-Hill Medical CY - New York, NY Y2 - 2024/03/28 UR - hemonc.mhmedical.com/content.aspx?aid=1126742831 ER -