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Clinical characteristics of carcinoid tumors

Jonathan R Strosberg, MD
Section Editors
Kenneth K Tanabe, MD
David C Whitcomb, MD, PhD
Deputy Editor
Diane MF Savarese, MD


The term "carcinoid" is generally applied to well-differentiated neuroendocrine tumors originating in the digestive tract, lungs, or rare primary sites, such as the kidneys or ovaries. Use of the term carcinoid implies well-differentiated (low- to intermediate-grade) histology and is not traditionally used to describe high-grade or poorly differentiated neuroendocrine tumors, which are now classified as neuroendocrine carcinomas. In the digestive system, well-differentiated neuroendocrine tumors of the luminal gastrointestinal tract have been designated carcinoid tumors, while those arising in the pancreas have been referred to as pancreatic neuroendocrine tumors (table 1). (See "Pathology, classification, and grading of neuroendocrine tumors arising in the digestive system", section on 'Pathology, tumor classification, and nomenclature'.)

"Carcinoid syndrome" is the term applied to a constellation of symptoms mediated by various humoral factors that are elaborated by some carcinoid tumors (table 2) [1]. Typical carcinoid syndrome, consisting primarily of flushing and diarrhea (table 3), occurs predominantly in patients with metastatic carcinoid tumors originating in the small intestine. (See "Clinical features of the carcinoid syndrome".)

Carcinoid tumors are rare overall, but their age-adjusted incidence in the United States has increased significantly, partly due to increased detection on radiographic imaging and endoscopy.

This topic will cover the epidemiology, classification, and clinical features of primary well-differentiated neuroendocrine tumors (carcinoid tumors) arising in the gastrointestinal and genitourinary systems. The pathology and nomenclature of carcinoid tumors; clinical features and diagnosis of carcinoid syndrome; issues related to tumor localization in well-differentiated neuroendocrine tumors; features and management of high-grade gastroenteropancreatic neuroendocrine carcinoma, bronchial carcinoid tumors, and thymic carcinoid tumors; and the management of patients with nonmetastatic and metastatic carcinoid tumors are discussed separately.

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

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