肝原发性恶性血管肿瘤临床病理分析

Clinicopathological study on primary malignant vascular tumors of liver

目的 :探讨肝原发性恶性血管肿瘤的临床病理学与鉴别诊断及预后的关系。方法 :采用 H- E和免疫组织化学 SP法染色对本院 6例肝血管肉瘤和 5例上皮样血管内皮瘤 (EHE)进行研究 ,观察肝原发性恶性血管肿瘤的临床病理学、免疫组化特点。患者年龄 6个月至 6 8岁 ,男∶女为 4∶ 7。 结果 :临床症状和实验室检查无特异性 ,肿瘤呈单个或多结节、直径为 3～13.7cm、常有出血和坏死。 6例血管肉瘤的病理学特征表现多样化 ,呈血管瘤、梭形细胞和上皮样肉瘤形态 ;5例 EHE的病理学特征为具有血管分化的树突状细胞和 (或 )胞质内血管腔的上皮样细胞、间质黏液样变到致密纤维化。免疫组化因子 和(或 ) CD34 阳性。 结论 :肝原发性恶性血管肿瘤极少见 ,生物学行为差异较大 ,血管肉瘤为高度恶性 ,预后差 ;EHE预后好于血管肉瘤 ,合理手术预后较好。根据其组织学和免疫组化特点 ,可作出明确的病理诊断。

Objective:To study the clinicopathological and immunohistochemical features of hepatic malignant vascular tumors. Methods:H E and immunohistochemistry were used to study 6 angiosarcoma and 5 epithelioid hemangioendothelioma (EHE) of the liver. Patients were aged 0.5 68 years, 7 were females and 4 were males. Results:Patients presented with nonspecific symptoms. Macroscopically,the tumors were usually multiple (3 13.7 cm in diameter) and were frequently hemorrhagic and multinodular. Histologically,there was a wide morphologic spectrum among cases. Six angiosarcoma included areas similar to cavernous and capillary hemangioma,spindle cell and epithelioid sarcoma. In the hepatic EHE,the tumor were comprised of dendritic and epithelioid cells that often contained vacuoles representing intracellular lumina. The stroma was fibrous with myxohyaline areas. Immunohistochemically,all tumors were positive for at least one endothelial marker(factor Ⅷ related antigen and /or CD 34 ). Conclusion:Hepatic malignant vascular tumor is very rare, the angiosarcoma of the liver is highly malignant with short survival. The prognosis of EHE is usually much better than that of angiosarcoma, surgical resection can obtain prolonged survival. The diagnosis can be made based on histological and immunohistochemical features of the tumor.