原发性肝血管肉瘤七例诊治分析

Diagnosis and treatment of primary hepatic angiosarcoma： a report of 7 cases with a literature review

目的探讨原发性肝血管肉瘤的诊断、治疗及预后。方法回顾性分析了自2004年12月至2010年12月间北京协和医院收治的7例原发性肝血管肉瘤的临床资料和随访结果，并复习相关文献。结果7例原发性肝血管肉瘤占同期收治的肝脏恶性肿瘤0．68％（7／1027），男5例，女2例，平均年龄43．3岁（33～74岁），3例有氯乙烯接触史，肿瘤单发4例、多发3例。临床表现和实验室检查均无特异性。CT或MRI检查常误诊为肝脏良性病变及肝转移性病变。造影超声显示病变显示较为特征性的表现，PET—CT检查可明确为恶性肿瘤。2例未治疗病例分别存活了3个月和5个月，2例肝移植病例移植后生存期为3个月和8个月，2例手术切除后辅助靶向药物病例的生存期分别为14个月和19个月，1例肝切除术后6个月复发并失访。结论原发性肝血管肉瘤临床极为少见，其恶性度高，预后差。临床表现缺乏特异性。超声造影和PET—CT对诊断有一定的帮助。手术切除可明显延长生存期，术后辅助靶向药物治疗可能会改善预后。

Objective To explore the clinical features, diagnosis, treatment and prognosis of primary hepatic angiosarcoma （ PHA）. Methods The clinical data of 7 PHA patients admitted to our hospital from December 2004 to December 2010 were retrospectively analyzed and the relevant literatures reviewed. Results Seven cases （5 males and 2 females） were diagnosed as PHA among 1027 （0. 68% ） patients with primary hepatic malignant tumors. Their mean age was 43. 3 years old （ range： 33 - 74）. Four cases were of solitary lesion and three of multiple lesions. No specific clinical features were observed. The PHA lesions were easily misdiagnosed as benign or hepatic metastatic tumors. Contrast enhanced ultrasound （CEUS） showed the lesions with characteristic manifestations. And the positron emission tomography （ PET- CT） could confirm the hepatic lesions as malignant tumors. The survival time for two untreated cases was 3 ＆ 5 months, for two cases with liver transplantation （LTx） 3 ＆ 8 months and for two cases treated with surgical resection ＆ targeted therapy was 14 ＆ 19 months respectively. One case was lost to follow-up at 6 months after hepatic resection. Conclusion PHA is a clinically rare and highly malignant tumor with a rapid progression and a poor prognosis. Both CEUS and PET-CT are helpful for its differential and confirmative diagnosis. LTx should be considered as a contraindication for PHA. Hepatic resection has proven to be beneficial for PHA patients with solitary lesion. Surgical resection plus targeted medicines may improve their survival.