骨上皮样血管内皮瘤临床病理学观察

Epithelioid hemangioendothelioma of bone: a clinicopathological analysis

目的 探讨骨上皮样血管内皮瘤的临床病理特征和诊断。方法 对 3例骨上皮样血管内皮瘤进行临床资料分析、光镜观察和免疫组织化学检测 ,并结合文献进行讨论。结果 3例病变部位均为下肢。最常见的临床症状是局部疼痛。X线表现为溶骨性骨破坏 ,1例伴有病理性骨折。组织形态学特征是上皮样瘤细胞形成较原始的小血管腔 ,呈巢状、索状、不规则形分布于有黏液样变或透明变性的间质中 ,肿瘤组织内或边缘散布成熟的骨小梁组织。 3例肿瘤均表达vimentin、FⅧRAg和CD34。随访结果 2例未见肿瘤复发 ,1例失访。结论 原发于骨的上皮样血管内皮瘤是较少见中间型血管源性肿瘤 ,其组织形态学要与骨上皮样血管瘤。

Purpose To explore the clinicopathologic features and diagnosis of epithelioid hemangioendothelioma (EHE) of bone. Methods Three cases of EHE of bone were analyzed by means of clinicopathologic data, histopathologic features, immunohistochemical technique and review of the literature. Results The lesions were located in the lower limbs. The most common presenting symptom was localized pain. Radiographs showed a well defined osteolytic lesion in 3 cases,in which one case with pathologic fracture. Microscopically, the distinctive features were that isolated, cords and nests of epithelioid endothelial cells disposed in a hyaline or myxoid matrix; cytoplasmic vacules were observed. The bony trabeculae were usually well formed and rimmed by osteoblasts. Immunohistochemically, all the tumors reacted positively with vimentin, FⅧRAg and CD34. The follow up data showed that there was no recurrence in 2 cases and 1 case was lost follow up. Conclusions EHE of bone is a rare type of borderline angiomatous tumor, and the differential diagnosis of EHE of bone includes epithelioid haemangioma, epithelioid angiosarcoma and metastatic carcinoma.