骨多发性上皮样血管肉瘤的临床病理分析

Clinical and Pathological Analysis of Primary Bone Epithelioid Angiosarcoma

目的探讨骨上皮样血管肉瘤的临床病理特征、诊断、鉴别诊断、治疗及预后。方法对1例骨上皮样血管肉瘤进行组织形态学观察及免疫组织化学染色分析,结合相关文献资料进行分析与总结。结果骨上皮样血管肉瘤镜下组织学表现为:肿瘤细胞为上皮样,呈巢状、条索状及丛状分布。易与低分化癌和其他上皮样肉瘤相混淆。免疫组织化学显示瘤细胞特异地表达CD31、CD34和FⅧ因子等血管源性标志物。结论骨上皮样血管肉瘤是一种罕见的肿瘤,具有高度侵袭性且往往迅速转移,预后很差。病理医师需仔细观察光镜下组织形态,并结合一组免疫组化标记,才能做出正确诊断。

Objeetive To investigate the clinic pathological feature of bone epithelioid angiosarcoma, understand its differential diagnosis, treatment and prognosis. Method One case of epithelioid angiosarcoma occurred in the bone was anaJyzed by histological and immunohistochemical staining, and relevant documentations were reviewed. Result Histologically, the tumor consisted predominantly of epithelioid cells with nest, cord and fasciculate patterns. Multicentric bone epithelioid angiosarcoma is easily misdiag-nosed as carcinoma and other epithelioid sarcoma. The immunohistochemical analysis revealed that the tumor mass was strongly positive for vascular endothelial markers, such as CD31, CD34 and factor VIII. Conclusion Bone epithelioid angiosarcoma is rare and high-grade, often transfers to lung or other positions in a short time. It should be correctly diagnosed by carefully histological observation and a battery of immunohistochemisty markers.