GASTROENTEROPANCREATIC NEUROENDOCRINE TUMORS: EPIDEMIOLOGIC AND CLINICAL FEATURES

Sercan Ön

Tepecik Training and Research Hospital, Department of Medical Oncology, İzmir, Türkiye

Ön S. Gastroenteropancreatic Neuroendocrine Tumors: Epidemiologic and Clinical Features. In: Gönüllü E, Karaman K, editors. Modern Approaches and Recent Advances in Neuroendocrine Tumors. 1st ed. Ankara: Türkiye Klinikleri; 2025. p.9-14.

ABSTRACT

Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are relatively rare. Their frequency has been increasing over time. These tumors are typically diagnosed in older patients and can originate from the tubular gastrointestinal system or the pancreas. The ileum is the most common site of origin, followed by the rectum, appendix, colon, and stomach. The exact etiology remains unclear, but they can be associated with inherited syndromes such as multiple endocrine neoplasia type 1 (MEN1). Tumor location affects the clinical course and tumor behavior. GEP-NETs are heterogeneous, with the risk of metastasis, bioactive amine synthesis capacity, and prognosis varying significantly. GEP-NETs are graded according to Ki-67 and mitotic index, with clinical progression becoming more aggressive as the tumor grade increases. While it is difficult to describe common characteristics for such a heterogeneous group of tumors, the liver is the most frequent site of metastasis. Due to the release of bioactive amines from tumor cells, GEP-NETs can lead to carcinoid syndrome. Functional pancreatic neuroendocrine tumors secrete various hormones such as insulin, gastrin, and glucagon, resulting in unique clinical manifestations.

Keywords: Gastroenteropancreatic neuroendocrine tumor; Carcinoid tumors; Malignant carcinoid syndrome; Epidemiology; Neoplasm metastasis

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