摘要
背景:胃肠间质瘤(GIST)是肠间叶细胞瘤的一个独特亚组,具有侵袭性特点,在成人GIST常被描述过,但在儿童很少被观察到。方法:作者对3例原发于胃的GIST患儿的临床表现、诊断性检查方法、手术记录、病理标本及最后结果进行了回顾。结果:3例患儿均因胃肿瘤引起的上消化道出血而就诊。第1例为10岁女孩, 接受了胃部分切除术,8年后因复发需进行第2次切除术,再8年后又有肝转移进行第3次切除术。第2例为9 岁女孩,接受了胃窦切除术(Bilroth I式重建术)并发现同时有肝转移而接受了肝肿瘤切除,尽管医生对其采用了甲磺酸伊马替尼治疗,但患者还是再次出现了肝转移。第3例为4岁男孩,于近期接受了胃部分切除术,此次未发现有转移表现。结论:GIST在儿童为罕见肿瘤, 并很少被描述过,当其发生于胃时,常在上消化道出血后就诊,可通过内镜和活检做出诊断。GIST需要进行切除并严密观察有无肝脏转移,对应用甲磺酸伊马替尼治疗儿童GIST的潜在作用目前正进行着研究。
Background Gastrointestinal stromal tumors (GIST) are a unique subset of intestinal mesenchynud tumors that behave in an aggressive fashion. They have been commonly described in adults but have been rarely observed in children. Methods The authors review the presentation, diagnostic workup, operative records, pathologic specimens, and outcomes of 3 children with GISTs that originated from the stomach. Results All 3 children presented after upper gastrointestinal bleeding from the gastric tumor. The first was a 10 - year-old girl who underwent partial gastrectomy but had recurrence 8 years later requiring a second resection. She subsequently had a hepatic metastasis 8 years later requiring a third resection. The second patient was a 9 - year-old girl who had an antrectomy with a Bilroth I reconstruction and was noted to have a synchronous liver metastasis that was also resected. Despite Imatinib Mesy-late, she had further hepatic metastases. The third child was a 4 - year-old boy who recently underwent a partial gastrectomy and has no signs of metastatic disease at this time. Conclusions GISTs are unusual tumors that have been rarely described in children. When they arise in the stomach, they often present after upper gastrointestinal bleeding. Diagnosis can be made by endoscopy and biopsy. GISTs require resection and close observation for hepatic metastases. Current studies are ongoing for the potential role of Imatinib Mesylate for GISTs in children.
