Extra-gastrointestinal stromal tumor of the pelvic cavity : case report
Extra-gastrointestinal stromal tumor of the pelvic cavity : case report
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盆腔胃肠外间质瘤：病例报告
DOI：
10.1007/s00795-011-0568-x
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发表时间：
2012
期刊：
影响因子：
1.8
通讯作者：
Nishigami T
中科院分区：
文献类型：
--
作者：
Hirano H;Yoshida T;Yoshimura H;Fukuoka M;Ohmura N;Nishizawa Y;Tachibana S;Hirota S;Zozumi M;Nishigami T
摘要发生在骨盆腔与消化道无关的胃肠道外间质瘤是非常罕见的。我们治疗了一名49岁的日本男性，他在盆腔内患有E-GIST，接受了盆腔内肿瘤切除术，包括全直肠切除术和部分直肠切除术。大体检查标本显示一个8.1 × 5 × 4 cm的灰白色肿块。组织学检查显示梭形细胞均匀，排列整齐，呈束状或螺旋状。这些肿瘤细胞不会侵入邻近器官，包括肠道。免疫组化结果显示，肿瘤细胞c-kit，CD 34和波形蛋白阳性。分子生物学分析显示c-kit突变位于第9外显子，并伴有Ala和Tyr的重复。我们的诊断是E-GIST，属于GIST的中间组。手术后，我们服用甲磺酸伊马替尼6个月。停药5个月后，因局部复发再次给药。我们正在计划我们未来的策略，包括必要的手术切除。
Extra-gastrointestinal stromal tumors (E-GISTs) not associated with the alimentary tract in the pelvic cavity are extremely rare. We treated a 49-year-old Japanese man with such an E-GIST in the pelvic cavity who underwent an intrapelvic tumorectomy with a total prostatectomy and partial rectum resection. Gross examination of the specimen revealed an 8.1 × 5 × 4 cm white-grayish mass. Histological findings showed uniform spindle cells with scant atypia that formed interlacing bundles or whorl patterns. These neoplastic cells did not invade adjacent organs, including the gut. Immunohistochemical findings revealed that the neoplastic cells were positive for c-kit, CD34, and vimentin. Molecular analysis showed a c-kitmutation at exon 9 with duplication of Ala and Tyr. Our diagnosis was E-GIST, which belongs to the intermediate group of GIST. Following the operation, we administered imatinib mesylate for 6 months. After stopping for 5 months, it was administered again for local recurrence. We are planning our future strategy for this case including surgical resection as necessary.