骨上皮样血管内皮瘤2例报道并文献复习

Epithelioid hemangioendothelioma of bone: a report of two cases and review of literature

目的 探讨骨上皮样血管内皮瘤的临床病理学特征和鉴别诊断。方法 观察和分析2例骨上皮样血管内皮瘤的病理形态特征和免疫组化标记,并结合文献复习,总结骨上皮样血管内皮瘤的临床病理学特点和鉴别诊断。结果 最常见的临床症状是骨疼痛;X线呈溶骨性骨破坏;肿瘤多呈分叶状,瘤细胞呈条索状、巢状或散在分布于透明变性的黏液基质中;CD34、CD31、CK和vimentin(+),EMA部分(+)。结论 原发于骨的上皮样血管内皮瘤是一种少见的低度恶性的血管源性肿瘤,需与转移癌、骨上皮样血管瘤和上皮样血管肉瘤等鉴别。

Objective To study the clinicopathologic characteristics and differential diagnosis of epithelioid hemangioendothelioma (EHE) of the bone. Methods Two cases of EHE were observed on histopathological characteristics and immunohistochemical staining with review of the literatures. Results The most common presenting symptom was localized pain. Radiograph showed a well-defined osteolytic lesion. There were characterized by isolated, cord, and nests of epithelioid endothelial cells in hyaline or myxoid matrix. Immunohistochemical study revealed positive for CD34, CD31 and CK(pan). Conclusions EHE of the bone is a rare type of angiomantous tumor with low malignant behaviour, and the differential diagnosis includes metastatic carcinoma, epithelioid haemangioma and epithelioid angiosarcoma.