Internal Medicine, Engaru-Kosei General Hospital, Engaru, Japan; Email:
J Gastrointestin Liver Dis. 2013 Sep;22(3):329-32.
Extragastrointestinal stromal tumors (EGISTs) are rare tumors located outside the gastrointestinal tract. While curable resection is accepted as a noninvasive EGIST treatment, the therapeutic strategy for invasive EGISTs has not yet been established. The present report is the first to show a case of invasive EGIST completely resected after downsizing the tumor with imatinib treatment. A 69-year-old female had multiple masses adjacent to the stomach and ileocecum. The primary lesion measured 18 cm in size and had invaded the stomach, pancreas and liver. The histological findings of fine-needle aspiration samples revealed a proliferation of dysplastic spindle cells that exhibited immunoreactivity for anti-c-kit antibodies. The masses were therefore diagnosed as multiple GISTs with invasion to other organs, with origin difficult to determine at the time. Nineteen months after the imatinib treatment, the tumors were downsized and distinct from the stomach, pancreas and liver. Accordingly, the tumors were regarded to be EGISTs derived from the mesentery. Because they slightly regressed 26 months after treatment, surgery was applied to remove the EGISTs. The intraoperative findings showed no invasive signs, and the tumors were completely removed. The histological findings revealed the presence of dysplastic and c-kit-positive spindle cells in the tumor with an MIB-I index of more than 5%, resulting in a final diagnosis of high-risk EGIST derived from the mesentery. No recurrence was detected for 16 months after resection. In conclusion, preoperative treatment with imatinib followed by curable resection is a feasible option to cure invasive EGISTs.
胃肠道外间质瘤(EGIST)是一种罕见的位于胃肠道外的肿瘤。虽然可切除的手术切除被认为是一种非侵入性的 EGIST 治疗方法,但侵袭性 EGIST 的治疗策略尚未确定。本报告首次报道了一例 EGIST 病例,该病例在伊马替尼治疗使肿瘤缩小后完全切除。一名 69 岁女性胃和回肠附近有多个肿块。原发性病变大小为 18cm,并侵犯了胃、胰腺和肝脏。细针穿刺样本的组织学检查结果显示,增生的发育不良梭形细胞对抗 c-kit 抗体呈免疫反应性。因此,这些肿块被诊断为多发性 GIST 合并其他器官浸润,起源难以确定。伊马替尼治疗 19 个月后,肿瘤缩小并与胃、胰腺和肝脏分离。因此,这些肿瘤被认为是源自肠系膜的 EGIST。由于治疗后 26 个月肿瘤略有退缩,因此采用手术切除 EGIST。术中发现无侵袭迹象,肿瘤完全切除。组织学检查显示肿瘤内存在发育不良和 c-kit 阳性梭形细胞,MIB-I 指数超过 5%,最终诊断为源自肠系膜的高风险 EGIST。切除后 16 个月无复发。总之,术前用伊马替尼治疗,然后进行可治愈的手术切除,是治疗侵袭性 EGIST 的一种可行方法。