肝原发性恶性血管肿瘤临床病理分析

Hepatic primary malignant vascular tumor: clinical and pathological analysis

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EnVision法对7例血管肉瘤和4例上皮样血管内皮瘤（epthelioid hemangioendothelioma，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—6．5cm。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。

Purpose To investigate the clinicopathological characteristics, differential diagnosis, and prognosis of hepatic primary malignant vascular tumors. Methods 7 cases of angiosarcoma and 4 cases of epthelioid hemangioendothelioma （EHE） resected during operation were analyzed by hematoxylin-eosin and .EnVision immunohistochemical staining. Results The age of the patients ranged from 23 to 80 years. 8 cases were males, and 3 cases were females. All cases had no specific clinical manifestations and laboratory findings. 6 cases showed simple nodule in the right lobe of the liver, whereas 5 cases were multiple nodules, among which 4 cases involved the left lobe. The maximal diameters of the tumor ranged from 2.0 to 6. 5 cm. Varieties of morphology were seen in 7 cases of angiosarcoma, such as spindle and epithelioid tumor cells. The histological features of the 4 cases of EHE were fibrous sclerosis center surrounded with cell-rich area and epithelioid cells that formed intra-cellular vascular lumina. All of the cases expressed at least one of the three immunohistochemical makers： CD31, CD34 and FⅧRAg, and 4 cases expressed them all at the same time. 1 case of EHE and 5 cases of angiosarcoma were nestin positive. Conclusions Primary malignant vascular tumors are very rare in the liver. Morphological observation and varied vascular endothelial immunohistochemical markers can make the accurate diagnosis. Early diagnosis and surgical resection with adjuvant chemotherapy can be helpful to prolong the survival time of the patient.