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目的探讨骨上皮样血管内皮瘤(EHE)的临床病理学特征。方法对3例骨EHE的临床资料、病理形态学、免疫组化染色进行观察,并进行随访。结果3例患者中男性1例,女性2例,年龄26～63岁,平均年龄40.3岁。临床症状主要是局部疼痛;X线表现无特异性,多为溶骨性缺损病变。组织学特点为肿瘤细胞排列成短条索状或巢状,瘤细胞呈上皮样,具有内皮细胞分化特征,分布于浅蓝色或深红色间质中。免疫组化:肿瘤细胞CD34、CD31和Fli-1(+)。2例术后随访未见复发,1例发生肝转移。结论原发于骨的EHE是一种少见的恶性血管源性肿瘤,部分病例组织形态与其他血管肿瘤有延续性,正确诊断需结合临床影像学资料、详细的组织学观察以及免疫组化染色。
Objective To investigate the clinicopathological features of bone epithelial hemangioendothelioma (EHE). Methods The clinical data, pathomorphology and immunohistochemistry of 3 cases of bone EHE were observed and followed up. Results There were 1 males and 2 females in 3 patients, aged from 26 to 63 years, with an average age of 40.3 years. Clinical symptoms are mainly local pain; X-ray showed no specificity, mostly osteolytic defect lesions. Histological features of tumor cells arranged in a short cord or nests, tumor cells were epithelial-like, with endothelial cell differentiation, distributed in light blue or dark red stroma. Immunohistochemistry: tumor cells CD34, CD31 and Fli-1 (+). No recurrence was found in 2 cases and liver metastasis in 1 case. Conclusions The primary EHE of bone is a rare malignant tumor of vasculature. Some of the cases have continuity with other vascular tumors. Correct diagnosis should be combined with clinical imaging data, detailed histological observation and immunohistochemical staining.