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目的探讨上皮样血管内皮瘤的临床及病理特点,提高对该病的诊治水平。方法利用中国医院知识数据库检索关键词为“上皮样血管内皮瘤”的1994—2013年文献,去除重复病例,收集整理并分析经病理确诊为上皮样血管内皮瘤的病例。结果检索到文献149篇,收集上皮样血管内皮瘤病例239例,男女比例为1∶1.91,年龄4~78岁,平均39.6岁。病变发生于肝、肺、骨、脑、鼻、甲状腺、乳腺、纵隔、胃肠道、脾、肾、卵巢、子宫、阴茎、皮肤、淋巴结等。腔镜或穿刺活检确诊91例,手术病理确诊148例。病理特点为肿瘤细胞似上皮样,呈多角形或类圆形,瘤细胞无明显核分裂像,瘤细胞形成小的细胞内管腔,见胞质内空泡,可见红细胞,免疫组化血管内皮标记物CD31、CD34、FⅧ-Rag中至少一项阳性,Vimentin阳性。结论上皮样血管内皮瘤女性较男性多见,可发生于各年龄阶段及多种部位,临床表现多样,确诊需病理。
Objective To investigate the clinical and pathological features of epithelioid hemangioendothelioma and to improve the diagnosis and treatment of the disease. Methods The Chinese hospital database of knowledge was searched from 1994 to 2013 with the key words of “Epithelioid hemangioendothelioma”. Duplicate cases were removed, and cases of pathologically diagnosed as epithelioid hemangioendothelioma were collected and analyzed. Results A total of 149 articles were retrieved and 239 cases of epithelioid hemangioendothelioma were collected. The ratio of male to female was 1: 1.91, ranging in age from 4 to 78 years with an average of 39.6 years. Lesions occur in the liver, lung, bone, brain, nose, thyroid, breast, mediastinum, gastrointestinal tract, spleen, kidney, ovary, uterus, penis, skin, lymph nodes and so on. Endoscopic or biopsy confirmed 91 cases, surgical pathology confirmed 148 cases. Pathological features of tumor cells like epithelial, polygonal or round, no obvious mitotic tumor cells, tumor cells to form a small intracellular lumen, see the cytoplasmic vacuoles, visible red blood cells, immunohistochemical vascular endothelial markers At least one of CD31, CD34 and FⅧ-Rag is positive and Vimentin is positive. Conclusions The epithelioid hemangioendothelioma is more common in men than in men and can occur in all age stages and various sites. The clinical manifestations are diverse and the pathology is confirmed.