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胃肠道基质肿瘤(Gastrointestinal stromal tumor,简称GISTs),为最常见的消化道间质肿瘤。GIST源自于卡氏间质细胞等平滑肌节律细胞[1]。本疾病一般是因为KIT基因突变所致(85%)[1],少数则由PDGFRA(10%)[1]、BRAF激酶基因突变引起[1]。95%的GIST在c-Kit(CD117)免疫染色下会呈阳性[1][2]。肿瘤最常见于胃部(66%),但源发于胃部的GIST一般相较于源发于消化道以外的GIST,恶性度会较低[2]。GIST的影响范围不限定于肠道,而可以遍及食道、胃、小肠、大肠及直肠;而随着GIST在消化道的不同部位发生,病者亦会出现不同的病征[3]。
胃肠道基质肿瘤 | |
---|---|
胃部胃肠道基质肿瘤之组织病理影像(以苏木精伊红着色显示) | |
类型 | Stromal tumor[*]、rare genetic intestinal disease[*]、inherited digestive tract tumor[*]、mesenchymal tumor of small intestine[*]、胃肠癌 |
分类和外部资源 | |
医学专科 | 肿瘤学 |
ICD-10 | D37 |
ICD-O | M8936/0 - M8936/3 |
OMIM | 606764 |
DiseasesDB | 33849 |
MeSH | D046152 |
Orphanet | 44890 |
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