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Treatment of the carcinoid syndrome

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


Carcinoid tumors are neuroendocrine tumors that originate in the digestive tract, lungs, or rare primary sites, such as kidneys or ovaries. The term "carcinoid" usually implies a well-differentiated histology and is rarely used to describe high-grade or poorly differentiated neuroendocrine cancers. (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 that are mediated by various hormones that are secreted by some carcinoid tumors (table 1) [1]. Two of the most common manifestations are flushing and diarrhea (table 2). Symptoms are associated with elevations in serum serotonin or its metabolite urinary 5-hydroxyindoleacetic acid (5-HIAA). (See "Clinical features of the carcinoid syndrome".)

Carcinoid crisis is a life-threatening form of carcinoid syndrome that results from the release of an overwhelming amount of biologically active compounds from the tumor that may be triggered by tumor manipulation (biopsy or surgery) or by anesthesia.

More than 90 percent of patients with the carcinoid syndrome have metastatic disease, typically involving the liver, with primary tumors in the distal small intestine or proximal colon (midgut). Rare exceptions are bronchial and ovarian carcinoids, which can rarely release hormones directly into the systemic circulation, thereby producing symptoms without metastases. When it does occur, carcinoid syndrome associated with bronchial carcinoids is often atypical; episodes of flushing and/or diaphoresis may be accompanied by other symptoms, such as tremor, periorbital edema, lacrimation, salivation, and edema. (See "Clinical features of the carcinoid syndrome", section on 'Lung NET variant syndrome'.)

This topic provides an overview of treatment for patients with symptoms of the carcinoid syndrome. Diagnosis of the carcinoid syndrome, radiologic staging, tumor localization, histologic assessment of carcinoid tumors, and presentation, treatment, prognosis, and posttreatment surveillance of patients with localized and metastatic carcinoid are discussed in detail elsewhere. (See "Diagnosis of the carcinoid syndrome and tumor localization" and "Pathology, classification, and grading of neuroendocrine tumors arising in the digestive system", section on 'Pathology, tumor classification, and nomenclature' and "Staging, treatment, and posttreatment surveillance of nonmetastatic, well-differentiated gastrointestinal tract neuroendocrine (carcinoid) tumors" and "Metastatic well-differentiated gastroenteropancreatic neuroendocrine tumors: Presentation, prognosis, imaging, and biochemical monitoring" and "Metastatic gastroenteropancreatic neuroendocrine tumors: Local options to control tumor growth and symptoms of hormone hypersecretion", section on 'Surgical resection'.)

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