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目的 研究网膜和肠系膜胃肠道外间质瘤 (EGIST)临床病理和免疫组织化学标记特点,并探讨其组织来源、预后评价及与胃肠道间质瘤 (GIST)的关系。方法 运用形态学和免疫组织化学(CD117、CD34等)研究 19例网膜和肠系膜原诊断为平滑肌肿瘤、许旺瘤的间叶性肿瘤。结果 共诊断 14例EGIST,其中网膜 6例,肠系膜 8例。肿瘤大小 3 5~29 0cm(平均 12 4cm)。梭形细胞为主型 9例,上皮样细胞为主型 2例,混合型 3例。免疫组织化学阳性表达结果:CD117 ( 14 /14 )、CD34(8 /14)、α 平滑肌肌动蛋白 (6 /14)。结蛋白、S 100蛋白均阴性。随访结果: 6例网膜EGIST均无瘤生存; 7例肠系膜EGIST3例死于肿瘤, 1例带瘤生存, 3例无瘤生存。结论 EGIST与GIST为同一性质肿瘤,可能共同起源于多分化潜能的间叶干细胞或肿瘤向卡哈尔间质细胞分化。EGIST有独特的行为谱,预后评价不能完全套用GIST的评价指标。
Objective To investigate the clinicopathological and immunohistochemical features of omentum and mesenteric gastrointestinal stromal tumors (EGIST), and to explore the relationship between the tissue source, prognosis and gastrointestinal stromal tumors (GIST). Methods The morphological and immunohistochemical (CD117, CD34, etc.) study of 19 cases of omentum and mesentery were diagnosed as smooth muscle tumors, Schwannoma mesenchymal tumor. Results A total of 14 cases of EGIST were diagnosed, including 6 cases of omentum and 8 cases of mesentery. Tumor size 35 ~ 29 0cm (average 12 4cm). Spindle cells in the main type in 9 cases, epithelial-like cells in 2 cases, mixed type in 3 cases. Results of immunohistochemistry showed positive expression of CD117 (14/14), CD34 (8/14) and α-smooth muscle actin (6/14). Desmin, S 100 protein were negative. Follow-up results: 6 cases of omentum EGIST were tumor-free survival; 7 cases of mesenteric EGIST3 cases died of tumor, 1 case of tumor-free survival, 3 cases of tumor-free survival. Conclusion EGIST and GIST are tumors of the same nature, possibly originating from differentiated mesenchymal stem cells or tumors differentiating into cardar stromal cells. EGIST has a unique behavioral spectrum, prognostic evaluation can not fully apply the GIST evaluation.