骨原发性恶性上皮样血管肿瘤临床病理分析

目的探讨骨原发性恶性上皮样血管肿瘤的临床病理特征、诊断及鉴别诊断。方法对4例骨原发性上皮样血管内皮细胞瘤(EHE)和2例上皮样血管肉瘤(EA)进行临床、光镜及免疫组化分析。结果 4例EHE中3例行刮除术,1例行化学治疗,均存活。2例EA分别行单纯化疗和手术治疗,1例失访,1例2个月后死亡。镜检:EHE的肿瘤细胞呈巢状、片状或条索状排列,间质常黏液样变性或透明变性,瘤细胞轻度异型,常见胞质内空泡。EA主要由呈实性片状、巢状排列的上皮样肿瘤细胞组成,瘤细胞异型性大,核呈空泡状,核仁显著,核分裂易见。免疫组化:瘤细胞CD31和vimentin(+)。结论 EHE低度恶性,EA高度恶性,两者鉴别主要依靠病理学特征,治疗和预后均差异较大,准确诊断是正确治疗的前提。

Clinicopathologial features of malignant epithelioid vascular tumors of bone

Objective To explore the clinicopathologic features,diagnosis and differential diagnosis of malignant epithelioid vascular tumors of the bone.Methods The clinical,histological and immunohistochemical features were analyzed in four cases of epithelioid hemangioendotheliom(EHE) and two cases of epithelioid angiosarcoma(EA) occurred in the bone.Results Four cases of EHE were treated with curettagen(n=3)or chemotherapy(n=1),and two cases of EA were treated with chemotherapy and surgery,respectively.Follow-up information was available in 5 patients,showing that four patients of EHE survived,one case of EA was lost to follow-up,and one died 2 months after diagnosis.Histologically,EHE consisted of nests,solid sheets and cords of epithelioid endothelial cells with mild atypia in hyaline or myxoid matrix stroma,and intracytoplasmic lumens(vacuoles) were frequently seen.EA consisted predominantly of solid sheets and nests of epithelioid cells,which had large vesicular nuclei with marked atypia and prominent nucleoli,and mitoses were frequently seen.Immunohistochemically,the tumor cells stained positive for CD31 and vimentin.Conclusions EHE of bone is a lower-grade malignant tumor,but EA is high-grade one.Their differential diagnosis relies mainly on pathological characteristics,the treatment and prognosis are significantly different,and accurate diagnosis is the premise of a correct treatment.