无症状患者13公斤的胃间质瘤:一例罕见的巨大间质瘤并文献复习

F. Silano, R. Amaral, V. Neves, Rodolfo Santana, Paulo Cézar, Galvão do Amaral
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引用次数: 0

摘要

虽然胃肠道间质瘤(gist)是最常见的发生于胃肠道间质的肿瘤,但在所有消化道肿瘤中所占比例不到1%。其发病率近年来有所增加,可能是由于诊断方法的改进。目前已知GIST是最常见的肉瘤。它起源于Cajal的间质细胞,依赖于转录因子ETV-1。这些肿瘤与分子改变和一些突变相关:超过75%的病例中有KIT突变(cd117)[3], 10%的病例有血小板衍生生长因子受体α (PDGFRA)突变,野生型gist有SDH通路[3]突变。两性之间似乎没有偏好。它们影响的年龄范围很广,但在大约75%的病例中,50岁以上的人更常见。胃肠道间质瘤可发生在消化道的任何器官,但超过50%的病例发生在胃,25%发生在小肠,少数发生在结肠、直肠、腹膜后、网膜等部位[5-9]。它的大小可以从毫米级病变到所谓的巨型胃肠道间质瘤,这是文献中对直径超过10厘米的肿瘤的定义。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Gastric GIST with 13 kg in Asymptomatic Patient: A Rare Case of Giant GIST and Literature Review
Although gastrointestinal stromal tumors, GISTs, are the most common neoplasms arising from the gastrointestinal mesenchyme, they represent less than 1% of all digestive tumors [1]. Its incidence has increased in recent years, probably due to improved diagnostic methods [2]. It is currently known that GIST is the most common sarcoma [3]. It originates from interstitial cells of Cajal and depends on the transcription factor ETV-1. These are neoplasms associated with molecular alterations and some mutations: In more than 75% of cases there is the KIT mutation (CD 117) [3], 10% have mutation in platelet-derived growth factor receptor alpha (PDGFRA) and wild-type GISTs have a mutation in the SDH pathway [3]. There does not seem to be a predilection between the sexes. They affect a wide age range, but in around 75% of cases, it is more common over 50 years of age [4]. Any organ of the digestive tract can be affected by GIST, however more than 50% of cases are seen in the stomach, 25% in the small intestine and a minority in the colon, rectum, retroperitoneum, omentum, among others [5-9]. Its size can vary from millimetric lesions to the so-called giant GISTs, a definition in the literature for those with more than 10 cm in diameter [10].
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