骨原发性上皮样血管肉瘤2例报道并文献复习

目的提高对骨原发性上皮样血管肉瘤(epithelioid angiosarcoma,EA)的认识,避免误诊。方法对2例原发于骨的EA进行临床病理、组织学及免疫组化分析,并进行文献复习。结果 2例均为男性,为骨组织内的多灶性、溶骨性病变,组织学上肿瘤主要由实性片状排列的上皮样细胞组成,瘤细胞核大,空泡状,含有明显核仁,可见细胞内空泡和血管腔样结构形成。免疫组化标记显示瘤细胞表达CD31、FⅧRAg、CK、vimentin。分别进行单纯化疗和手术治疗。1例失访,1例2个月后死亡。结论骨原发性EA是一种具有上皮样特征的高度恶性血管源性肿瘤,必须与转移癌等鉴别,CD31、CK等血管标记物对鉴别诊断具有重要的意义。

Epithelioid angiosarcoma of bone:a report of two cases and review of literatures

Purpose To increase the understanding of epithelioid angiosarcoma occurred in the bone tissue and avoid misdiagnosis.Methods Two cases of epithelioid angiosarcoma occurred in the bone were analyzed by clinicopathological,histological and immunohistochemical staining,and relevant literatures were reviewed.Results All two cases were male.Radiographic imagings showed multifocal osteolytic lesions.Histologically,the tumor consisted predominantly of solid sheets of large epithelioid cells with large vesicular nuclei and prominent nucleoli.Introcytoplasmic vacuoles contained red blood cells and well-formed vascular channels lined by tumor cells were also noted in some areas.Immunohistochemically,the tumor cells stained positive for CD31,factor Ⅷ-related antigen,CK and vimentin.The patients were treated with chemotherapy or surgery respectively.One case was lost to follow-up,and one died 2 months after diagnosis.Conclusions Primary epithelioid angiosarcoma of bone is a high-grade malignant tumor with epithelioid features morphologically,and it should different from other epitheliod tumors such as metastatic carcinoma and so on.Vascular markers(especially CD31) are very important for differential diagnosis.