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Metastatic well-differentiated gastroenteropancreatic neuroendocrine tumors: Presentation, prognosis, imaging, and biochemical monitoring

Jennifer Ang Chan, MD, MPH
Matthew Kulke, MD
Section Editor
Richard M Goldberg, MD
Deputy Editor
Diane MF Savarese, MD


Neuroendocrine cells are distributed widely throughout the body, and neuroendocrine neoplasms can arise at many sites. While there are differences in terminology and grading for these tumors arising at different sites, all commonly used classification systems reflect a basic separation between more indolent well-differentiated tumors (which in the digestive system have been traditionally referred to as carcinoid tumors [now termed gastrointestinal neuroendocrine tumors (NETs)] or pancreatic islet cell tumors [pancreatic NETs]) and far more aggressive poorly differentiated types that behave clinically more like small cell carcinoma of the lung (termed neuroendocrine carcinomas). (See "High-grade gastroenteropancreatic neuroendocrine carcinoma" and "Pathobiology and staging of small cell carcinoma of the lung".)

The clinical presentation, prognosis, radiographic imaging, and tumor marker evaluation of patients with well-differentiated NETs arising in the digestive system are discussed in this topic review. Pathology and nomenclature of gastroenteropancreatic NETs (GEP-NETs), treatment options for advanced or metastatic NETs, as well as issues relating to the presentation, localization, and treatment of early stage gastrointestinal NETs and pancreatic NETs are presented elsewhere, as is small cell lung cancer, high-grade gastroenteropancreatic neuroendocrine carcinoma, and neuroendocrine carcinoma of unknown primary site.

(See "Pathology, classification, and grading of neuroendocrine tumors arising in the digestive system".)

(See "Metastatic gastroenteropancreatic neuroendocrine tumors: Local options to control tumor growth and symptoms of hormone hypersecretion".)

(See "Clinical characteristics of carcinoid tumors".)

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Literature review current through: Oct 2017. | This topic last updated: Oct 20, 2017.
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