General Information About Gastrointestinal Carcinoid Tumors
The age-adjusted incidence of carcinoid tumors worldwide is approximately 2 per 100,000 persons.[1,2] The average age at diagnosis is 61.4 years. Carcinoid tumors represent about 0.5% of all newly diagnosed malignancies.[2,3]
Langerhans cell histiocytosis (LCH) usually presents with a skin rash or painful bone lesion. Systemic symptoms of fever, weight loss, diarrhea, edema, dyspnea, polydipsia, and polyuria, relate to specific organ involvement as well as single-system or multisystem disease presentation as noted below.
Specific organs are considered high-risk or low-risk organs when involved with disease presentation. Risk refers to the risk of mortality.
High-risk organs include liver, spleen, lung,...
Carcinoid tumors are rare, slow-growing tumors that originate in cells of the diffuse neuroendocrine system. They occur most frequently in tissues derived from the embryonic gut. Foregut tumors, which account for up to 25% of cases, arise in the lung, thymus, stomach, or proximal duodenum. Midgut tumors, which account for up to 50% of cases, arise in the small intestine, appendix, or proximal colon, with the appendix being the most common site of origin. Hindgut tumors, which account for approximately 15% of cases, arise in the distal colon or rectum. Other sites of origin include the gallbladder, kidney, liver, pancreas, ovary, and testis.[3,4,5]
Gastrointestinal (GI) carcinoid tumors, especially tumors of the small intestine, are often associated with other cancers. Synchronous or metachronous cancers occur in approximately 29% of patients with small intestinal carcinoids. However, it is possible that the association may be due in part to the serendipitous discovery of slow-growing carcinoid tumors, which are found while investigating symptoms from, or staging, other tumors.
The term carcinoid should be used for well-differentiated neuroendocrine tumors (NETs) or carcinomas of the GI tract only; the term should not be used to describe pancreatic NETs or islet cell tumors. (Refer to the PDQ summary on Islet Cell Carcinoma Tumors [Endocrine Pancreas] Treatment for more information.) Data regarding carcinoids and other NETs, such as poorly differentiated neuroendocrine carcinomas, may be combined in some epidemiologic and clinical studies, rendering separate consideration difficult. Occurring nonrandomly throughout the GI tract are more than 14 cell types, which produce different hormones. (Refer to the Cellular and Pathologic Classification of Gastrointestinal Carcinoid Tumors section of this summary for more information.) Although the cellular origin of NETs of the GI tract is uncertain, consistent expression of cytokeratins in NETs and the expression of the caudal-related homeodomain protein 2 (CdX2 protein), an intestinal transcription factor in endocrine tumors of the small intestine, suggests an origin from an epithelial precursor cell.
Most NETs of the small and large intestines occur sporadically, while others may occur within the background of an inherited neoplasia syndrome such as multiple endocrine neoplasia type 1 (MEN1) or neurofibromatosis type 1 (NF1) (e.g., gastrin-producing G-cell tumors and somatostatin-producing D-cell tumors of the duodenum, respectively). Tumor multifocality is the rule within the background of neuroendocrine cell hyperplasia, but multifocality is found in approximately one-third of patients with small enterochromaffin cell tumors in the absence of proliferative or genetic factors; clonality studies suggest that most of these neoplasms are separate primary lesions.[10,11] Gastric carcinoids may be associated with chronic atrophic gastritis.