Clinical characteristics of carcinoid tumors
- Jonathan R Strosberg, MD
Jonathan R Strosberg, MD
- Associate Professor
- Department of Gastrointestinal Oncology
- H. Lee Moffitt Cancer Center
- Section Editors
- Kenneth K Tanabe, MD
Kenneth K Tanabe, MD
- Section Editor — Gastrointestinal Malignancies
- Professor of Surgery
- Harvard Medical School
- David C Whitcomb, MD, PhD
David C Whitcomb, MD, PhD
- Section Editor — Pancreatic Diseases
- Professor of Medicine
- University of Pittsburgh School of Medicine
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 (GI) tract have been designated carcinoid tumors, while those arising in the pancreas (and rarely in the duodenum) have been referred to as pancreatic neuroendocrine (islet cell) 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) . The 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 GI and genitourinary systems. The pathology and nomenclature of carcinoid tumors, clinical features and diagnosis of the 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 non-metastatic and metastatic carcinoid tumors are discussed separately.
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