摘要
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3.5～29.0 cm(平均12.4 cm).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EGIST 3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 explore the clinicopathological and immunohistochemical features of extra-gastrointestinal stromal tumors (EGIST) arising from the omentum and mesentery and to investigate the cellular origin of these tumors, prognostic factors, and the relationships with gastrointestinal stromal tumors. Methods Nineteen cases of mesenchymal neoplasms arising from the omentum and mesentery (previously diagnosed as smooth-muscle tumors or schwannomas) were studied morphological with a panel of immunohistochemistry including CD117 and CD34. Results Among the 19 cases, 14 tumors were confirmed to be EGIST, of which 6 tumors arose from the omentum and 8 cases located at the mesentery. The size of tumors ranged from 3.5cm to 29.0 cm (mean 12.4cm) in diameter. Histologically, there were 9 cases of mainly spindle cell type, 2 cases of mainly epithelioid cell type and 3 cases of mixed cell type. all EGIST expressed CD117(14/14) and a percentage of them expressed also CD34 (8/14) and/or SMA (6/14), anyhow, all EGIST were negative for desmin and S-100 protein. Six patients with tumors arising from the omentum were all alive without evidence of disease (tumor-free). Among 7 cases with tumors of the mesentery, three patients died of the disease, 1 alive with the disease and 3 patients alive without evidence of the disease. Conclusions EGIST were identical by their histological and immunohistochemical features with gastrointestinal stromal tumors (GIST). This tumor may arise from the multipotential mesenchymal stem cells. EGIST have various clinical behavior, and the parameters used for predicting the prognosis of GIST may not be completely suitable for EGIST evaluation.
出处
《中华病理学杂志》
CAS
CSCD
北大核心
2005年第1期11-14,共4页
Chinese Journal of Pathology
关键词
网膜
肠系膜胃肠道外间质瘤
病理
免疫组织化学
预后
Peritoneal neoplasms
Mesenchynoma
Proto-oncogene protein c-kit
Immunohistochemistry
