骨原发性上皮样血管内皮瘤4例临床病理分析

目的探讨骨原发性上皮样血管内皮瘤(EHE)的临床病理特征及诊断和鉴别诊断,以提高对该病变的认识。方法观察4例骨原发性EHE的临床病理特征及免疫组化表型特征,并结合文献分析讨论。结果骨EHE好发于年轻男性,男女之比为2∶1。主要临床表现为局部疼痛,其临床病程长短不一,可复发转移。病变以多中心性居多,X线下为无特征性的溶骨性病变。镜下上皮样瘤细胞呈条索状或巢团状分布于黏液样或透明变性的间质中,形成不同发育阶段的血管,可见单细胞血管腔。免疫组化:瘤细胞CD34、CD31、F8因子、vimentin和广谱CK(+)。结论骨EHE是一种少见的血管源性恶性肿瘤,具有独特的临床病理学特征及预后,临床及影像学诊断有困难,病理学检查是最佳诊断手段,应注意与感染、转移癌及其他良性病变相鉴别。

Epithelioid hemangioendothelioma of bone:a clinicopathologic analysis of 4 cases

Objective To explore the clinicopathologic features and diagnosis and differential diagnosis of epithelioid hemangioendothelioma(EHE) of bone.Methods The clinicopathologic features and immunohistochemical phenotype were observed in 4 bony EHE cases and related literature was reviewed.Results Bony EHE predominantly occurred at the age from 20 to 30 years of male.The ratio of male to female was 2∶1.The most common clinical symptom was localized pain.Their clinical courses were discordant and recurrence and metastasis may occur.There were predominantly multicentric and nothing special in clinical and X-Ray.Histopathologically,the distinctive features were that isolated,cords and nests of epithelioid tumor cells disposed in a hyaline or myxoid matrix with formation of blood vessels in various differentiation.Cytoplasmic vacules or vessel channel were observed.The bony trabeculae were usually seen and rimmed by osteoclastic giant cells.It was classified into classic type and malignant one according to cytologic and architecture characteristic in order to differentiate malignant degree.Immunohistochemically,tumor cells positively expressed CD34,CD31,FⅧRAg,vimentin and/or cytokeratin-pan.Conclusion EHE of bone is a rare type of malignant angiomatous tumor with distinct clinicopathologic features and often difficult to make diagnosis in clinical and X-ray.Pathologic examination is the best mean to make diagnosis for it.It should be differentiated from infection,metastatic carcinoma and other benign lesions.