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Staging, treatment, and posttreatment surveillance of nonmetastatic, well-differentiated gastrointestinal tract neuroendocrine (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 (NETs) originating in the digestive tract, lungs, or rare primary sites such as kidneys or ovaries. Use of the term "carcinoid" implies well-differentiated histology; the term "neuroendocrine carcinoma" is applied to the rare, high-grade, usually poorly-differentiated NETs. (See "Pathology, classification, and grading of neuroendocrine tumors arising in the digestive system", section on 'Pathology, tumor classification, and nomenclature' and "High-grade gastroenteropancreatic neuroendocrine carcinoma".)

"Carcinoid syndrome" is the term applied to a constellation of symptoms mediated by various humoral factors that are elaborated by some carcinoid tumors (table 1) [1]. Two of the most common manifestations are flushing and diarrhea (table 2). (See "Clinical features of the carcinoid syndrome".)

Most NETs are associated with carcinoid syndrome only when they have metastasized to the liver. Exceptions are bronchial and ovarian carcinoids, which can release hormones directly into the systemic circulation, thereby producing symptoms without metastases (table 3). When it occurs, carcinoid syndrome associated with foregut tumors such as bronchial NETs is often atypical with episodes of flushing and/or diaphoresis, occasionally accompanied by other symptoms such as lacrimation, salivation, and edema. (See "Clinical features of the carcinoid syndrome", section on 'Lung NET variant syndrome'.)

In general, the basic principles of evaluation and management of patients with NETs include:

Radiographic staging and tumor localization – Common imaging modalities include computed tomography (CT) or magnetic resonance imaging (MRI) scans as well as somatostatin receptor-based diagnostic imaging (Indium-111 pentetreotide single-photon emission computed tomography [SPECT] imaging [OctreoScan] or Gallium Ga-68 DOTATATE positron emission tomography [PET] scanning). Upper and lower endoscopy (with attention to the terminal ileum) should be performed for the evaluation of metastatic NET with an unknown primary site. CT enterography can also be used for this purpose. While video capsule endoscopy allows for evaluation of the entire small intestine, routine use of capsule endoscopy cannot be recommended due to the risk of bowel obstruction from retention of the capsule at the site of an intestinal NET. (See "Neuroendocrine neoplasms of unknown primary site", section on 'Well-differentiated neuroendocrine tumor'.)

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Literature review current through: Nov 2017. | This topic last updated: May 11, 2017.
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