Terada Tadashi
Department of Pathology, Shizuoka Municipal Shimizu Hospital, Miyakami 1231, Shimizu-Ku, 424-8636, Shizuoka, Japan.
World J Gastroenterol. 2008 Dec 21;14(47):7256-9. doi: 10.3748/wjg.14.7256.
The author reports a very rare case of sporadic primary multiple extragastrointestinal stromal tumors (EGISTs) of the omentum associated with different mutations of the exon 11 of the c-kit gene in a 75-year-old man with gastric cancer. During an operation for the cancer, two solid tumors (10 mm and 8 mm) were found in the omentum. Both tumors consisted of cellular spindle cells. Mitotic figures were two and three per 50 high power fields. The tumor cells were positive for KIT, CD34 and vimentin, but negative for desmin, S100 protein, alpha-smooth muscle actin and p53 protein. Ki67 labeling was 2% and 3%. The larger EGIST showed a deletion of codons 552-558 of exon 11 of the c-kit gene, while the smaller EGIST had a point mutation at codon 559 (GTT-->GAT) in exon 11 of the c-kit gene. Exons 9, 13, and 17 of the c-kit gene, and exons 12 and 18 of the platelet derived growth factor receptor alpha genes showed no mutations. The case shows that sporadic multiple EGISTs can occur in the omentum.
作者报告了一例非常罕见的病例，一名75岁的胃癌男性患者，其大网膜出现散发性原发性多发胃肠道外间质瘤（EGIST），且伴有c-kit基因第11外显子的不同突变。在该癌症手术过程中，于大网膜发现了两个实性肿瘤（10毫米和8毫米）。两个肿瘤均由细胞性梭形细胞组成。每50个高倍视野下的有丝分裂象分别为2个和3个。肿瘤细胞KIT、CD34和波形蛋白呈阳性，但结蛋白、S100蛋白、α平滑肌肌动蛋白和p53蛋白呈阴性。Ki67标记率分别为2%和3%。较大的EGIST显示c-kit基因第11外显子的552 - 558密码子缺失，而较小的EGIST在c-kit基因第11外显子的559密码子处有一个点突变（GTT→GAT）。c-kit基因的第9、13和17外显子，以及血小板衍生生长因子受体α基因的第12和18外显子均未显示突变。该病例表明散发性多发EGIST可发生于大网膜。
