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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。
Objective To investigate the clinicopathological features of primary hepatic angiosarcoma (PHA). Methods The clinicopathological and immunohistochemical features of 6 cases of PHA were analyzed. Results 6 cases, 3 males and 3 females, mean age 72 years. Microscopic observation of tumor morphology, showing that heterotrophic tumor cells along the sinusoidal growth, the formation of irregular-sized vessels and endovascular papillae, well differentiated cavernous hemangioma-like structure, poorly differentiated naive single-cell blood vessels and solid The hyperplastic area is formed like fibrosarcoma, poorly differentiated cancerous changes. Immunohistochemistry: 6 cases of CD31 strong (+), 5 cases of CD34, FLi-1 and ERG (+). Conclusions Primary hepatic angiosarcoma is a rare malignant tumor with poor prognosis. The clinical symptoms and imaging findings are non-specific. The final diagnosis depends on histopathology and a number of vascular endothelial markers. Early detection, complete surgical resection and postoperative chemotherapy can prolong survival.