骨上皮样血管内皮细胞瘤3例临床病理分析

Epithelioid haemangioendothelioma of bone:a clinicopathologic analysis of three cases

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 explore the clinicopathologic features of epithelioid haemangioendothelioma of bone. Methods The clinical materials, radiology, and histomorphology were analyzed with review of the related literature. Results Among the 3 patients studied, 2 were females and 1 was male. Patients ranged in age from 26 to 63 years （ mean 40. 3 years）. The most clinical presentation was localized pain. Radiographic features were not specific, appeared as a well-demarcated, lyric lesion with variable peripheral sclerosis. Section showed a neoplasm composed of cords and nests of epithelioid cells in chondroid-like matrix. Some cells contained intracytoplasmie vacuoles representing primitive vascular lumina. Immunohistochemically, tumor cells were positive for CD34, CD31, and Fli-1. Available follow-up showed no recurrence in 2 cases and 1 with liver metastases. Conclusions Epithelioid haemangioendothelioma of bone is a rare malignant tumor, sometimes could not be separated from other vascular tumors of bone. Recognition is based on the radiographic findings, careful histologic examination and immunohistochemical staining.