| H00034 | |
| H番号 | H00034 |
| 名称 | カルチノイド |
| 概要 | 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. |
| カテゴリ | がん |
| ネットワーク | - |
| 病因遺伝子 | MEN1 (mutation, LOH) [HSA:4221] [KO:K14970] SDHD (germline mutation, LOH) [HSA:6392] [KO:K00237] |
| 病原体 | - |
| 環境要因 | - |
| 発癌物質 | - |
| 治療薬 | オクトレオチド酢酸塩 [DR:D02250] |
| コメント | - |
| リンク | ICD-11: 2B80 ICD-10: C17 MeSH: D002276 |
| 文献 | PMID:15887161 著者 Modlin IM, Kidd M, Latich I, Zikusoka MN, Shapiro MD. タイトル Current status of gastrointestinal carcinoids. 雑誌 Gastroenterology 128:1717-51 (2005) DOI:10.1053/j.gastro.2005.03.038 PMID:14713256 著者 Leotlela PD, Jauch A, Holtgreve-Grez H, Thakker RV. タイトル Genetics of neuroendocrine and carcinoid tumours. 雑誌 Endocr Relat Cancer 10:437-50 (2003) DOI:10.1677/erc.0.0100437 |