Carcinoid tumors are relatively uncommon neoplasms that nonetheless comprise up to 85% of neuroendocrine gastrointestinal neoplasms. They most frequently occur in the midgut and develop from neuroendocrine cells that are normally and diffusely present in this location. Most carcinoids are sporadic but epidemiological studies report a familial risk. Moreover, carcinoids can occur within the multiple endocrine neoplasia (MEN) syndrome, a rare familiar tumor syndrome in which mutations in the MEN1 gene are manifested. Recently, it has been shown that a majority (78%) of sporadic carcinoids display loss of heterozygosity for markers around the MEN 1 region, thus suggesting involvement of this gene in the pathogenesis of both familial and sporadic carcinoids.
Human diseases [BR:
Cancers of the digestive system
Human diseases in ICD-11 classification [BR:
Malignant neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues
Malignant neoplasms, stated or presumed to be primary, of specified sites, except of lymphoid, haematopoietic, central nervous system or related tissues
Malignant neoplasms of digestive organs
Malignant neoplasms of intestine
2B80 Malignant neoplasms of small intestine
Tumor markers [
Transcriptional misregulation in cancer
Mutation-inactivated MEN1 to transcription
MEN1 (mutation, LOH) [HSA:
SDHD (germline mutation, LOH) [HSA:
Telotristat ethyl [DR:
Octreotide acetate [DR:
ICD-O: 8240/3, Tumor type: Carcinoid (well differentiated endocrine neoplasm)
(gene, tumor type)
Modlin IM, Kidd M, Latich I, Zikusoka MN, Shapiro MD.
Current status of gastrointestinal carcinoids.
Gastroenterology 128:1717-51 (2005)
Leotlela PD, Jauch A, Holtgreve-Grez H, Thakker RV.
Genetics of neuroendocrine and carcinoid tumours.
Endocr Relat Cancer 10:437-50 (2003)
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