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目的探讨肝原发性恶性血管肿瘤的临床病理学特点、鉴别诊断及预后。方法采用HE和免疫组化EnV ision法对7例血管肉瘤和4例上皮样血管内皮瘤(epthelioid hem angioendotheliom a,EHE)进行免疫组化染色和形态学观察。结果患者年龄23~80岁,男性8例,女性3例。临床症状及实验室检查无特殊表现。6例肿瘤呈单结节,位于肝右叶;5例多结节者,其中4例累及肝左叶,肿瘤最大径为2~6.5 cm。7例血管肉瘤组织学表现多样,肿瘤细胞可呈梭形或上皮样细胞形态。4例EHE组织学特征:以纤维硬化区为中心,周边富于细胞,肿瘤细胞呈上皮样分化及特征性的胞质内血管腔形成。免疫组化染色至少表达CD31、CD34、FⅧRAg中的1项,其中4例3项指标均表达。1例EHE和5例血管肉瘤表达nestin。结论肝原发性恶性血管肿瘤极少见,结合组织学形态并联合应用多项血管内皮标记物可作出明确诊断。早期发现、局部手术切除和结合术后化疗有助于延长患者生存期。
Objective To investigate the clinicopathological features, differential diagnosis and prognosis of primary malignant liver tumors. Methods Seven cases of angio-sarcoma and four cases of epithelial hemangioendothelioma (EHE) were examined by immunohistochemistry and immunohistochemical EnVision. Immunohistochemical staining and morphological observation were performed. Results Patients aged 23 to 80 years, 8 males and 3 females. Clinical symptoms and laboratory tests no special performance. The tumor was single nodular in 6 cases, located in the right lobe of the liver. In 5 cases with multiple nodules, 4 cases involved the left lobe of liver and the largest diameter of the tumor was 2 ~ 6.5 cm. Seven cases of angiosarcoma histological diversity, tumor cells can spindle or epithelial cell morphology. 4 cases of EHE histological features: fibrosis zone as the center, rich in cells around the tumor cells were epithelial-like differentiation and characteristic cytoplasmic cavity formation. Immunohistochemical staining at least one of CD31, CD34, F Ⅷ RAg, of which 4 cases were 3 indicators were expressed. One case of EHE and five cases of angiosarcoma expressed nestin. Conclusions Primary malignant hepatic tumors in liver are extremely rare. Combined with histological morphology and combined application of multiple vascular endothelial markers, definite diagnosis can be made. Early detection, partial surgical resection combined with postoperative chemotherapy can help prolong the survival of patients.