【摘要】 目的探讨原发性肝血管肉瘤(PHA)的临床病理特点。方法分析6例PHA的临床病理及免疫组化特点。结果 6例中男性3例,女性3例,平均年龄72岁。镜下观察肿瘤形态多样,可见异型瘤细胞沿肝窦生长,形成大小不等不规则血管腔及腔内乳头,分化好的海绵状血管瘤样结构、分化差的幼稚单细胞血管腔及实性增生区形成似纤维肉瘤、低分化癌样改变。免疫组化:6例CD31强(+),5例CD34、FLi-1和ERG(+)。结论原发性肝血管肉瘤是一种少见的预后很差的恶性肿瘤,临床症状和影像学检查无特异性,最终确诊需依赖病理组织学并多项血管内皮标记物。早期发现、完全手术切除及术后化疗可延长生存期。更多还原

【Abstract】 Purpose To investigate the clinicopathological characteristics of primary hepatic angiosarcoma. Methods Six cases of primary hepatic angiosarcoma were analyzed by gross examination, light microscopy and immunohistochemical staining. Results Three cases were male,and three were female. The average age was 72 years.Microscopically,there were various patterns: sinusoidal infiltration by atypical tumor cells,malignant pleomorphic tumor cells forming papilla and irregular vessel-like structures, relatively well-differentiated cavernous vascular space and epithelioid hemangioendothelioma-like appearances, solid sheets resemble with fibrosarcoma or poorly differentiated carcinoma. Immunohistochemical staining showed that 6 cases were strongly positive expression of CD31,5 cases expressed CD34,FLI-1 and ERG. Conclusions Primary hepatic angiosarcoma is a rare malignant tumor. All cases show no specific clinical symptoms and imaging examinations. The final pathological diagnosis depends on histology( anastomosing vessels lined by atypical endothelial cells) and combined application of many vascular endothelial immunohistochemical markers( CD31,ERG,CD34,FLi-1). Early diagnosis and radically surgical resection with adjuvant chemotherapy can be helpful to prolong the survival time of the patient.更多还原