KEGG   DISEASE: CarcinoidHelp
Entry
H00034                      Disease                                

Name
Carcinoid
Description
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.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of the digestive system
   H00034  Carcinoid
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  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
       H00034  Carcinoid
Tumor markers [br08442.html]
 H00034
BRITE hierarchy
Pathway
hsa05202  Transcriptional misregulation in cancer
Network
N00290  Mutation-inactivated MEN1 to transcription
Gene
MEN1 (mutation, LOH) [HSA:4221] [KO:K14970]
SDHD (germline mutation, LOH) [HSA:6392] [KO:K00237]
Drug
Octreotide acetate [DR:D06495]
Comment
ICD-O: 8240/3, Tumor type: Carcinoid (well differentiated endocrine neoplasm)
Other DBs
ICD-11: 2B80
ICD-10: C17
MeSH: D002276
Reference
PMID:15887161 (gene, tumor type)
  Authors
Modlin IM, Kidd M, Latich I, Zikusoka MN, Shapiro MD.
  Title
Current status of gastrointestinal carcinoids.
  Journal
Gastroenterology 128:1717-51 (2005)
DOI:10.1053/j.gastro.2005.03.038
Reference
  Authors
Leotlela PD, Jauch A, Holtgreve-Grez H, Thakker RV.
  Title
Genetics of neuroendocrine and carcinoid tumours.
  Journal
Endocr Relat Cancer 10:437-50 (2003)
DOI:10.1677/erc.0.0100437

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