骨原发性上皮样血管肿瘤的临床病理特点

The Clinical and Pathological Features of Primary Bone Epithelioid Vascular Tumors

骨原发性上皮样血管肿瘤包括上皮样血管瘤、上皮样血管内皮瘤和上皮样血管肉瘤,三者在组织形态学和生物学行为方面构成了从良性、低度恶性到高度恶性的谱系,且它们的生物学行为、治疗及预后均不相同。因其发病率低,临床表现也缺乏特异性,所以术前很难确诊。加之三者的病理组织学改变也存在一定的交叉,故需结合组织形态、免疫表型及分子遗传学等进行综合判断。

Primary bone epithelioid vascular tumors encompass a histologic and biological behavior spectrum,which includes epithelioid hemangioma,epithelioid hemangioendothelioma and epithelioid angiosarcoma. There are major differences in their clinical behavior and consequently in treatment and prognosis. Because of their rarity and non-specific clinical manifestation,it＇s difficult to be diagnosed before sugery. As there is a certain cross in the histopathological changes among these three tumors, the diagnosis should be made comprehensively with the combination of morphology,immune phenotype and molecular genetics.