肝脏血管肉瘤临床分析并文献复习

Two cases of hepatic angiosarcoma and literature review

目的 探讨肝脏血管肉瘤的病理学特征、临床特点和治疗方法.方法 对我院收治的2例肝脏血管肉瘤进行回顾性分析,并结合相关文献进行分析总结.结果 原发性肝血管肉瘤临床极为罕见,术前几乎均误诊,临床以右上腹痛、腹胀、乏力、纳差、消瘦和低热多见.右上腹明显饱满,可触及肿大的肝脏.血清AFP、CEA阴性.影像学检查：胸部X线检查均见右膈肌升高.超声检查：肝内可见单个或多个大小不等的无回声区,边界不清,彩色多普勒显示肿块内血液供应丰富.肝CT检查：肝内可见巨大或多个低密度区,增强扫描病灶呈边缘结节状强化,延迟扫描病灶明显缩小.活检或手术病理可确诊.本病治疗效果差,平均生存期约5个月.结论 肝脏血管肉瘤是一种少见的高度恶性肿瘤,其诊断主要依靠病理组织学和免疫组化,治疗以手术切除为首选,放化疗均不敏感,预后较差.

Objective To investigate the pathological and clinical features and treatment of hepatic angiosarcoma. Methods Two cases of hepatic angiosarcoma were discussed and pertinent literature were reviewed.Results Primary hepatic angiosarcoma is extremely rare. Most of the clinical cases were misdiagnosed in preoperative patients. More common clinical symptoms included right upper quadrant abdominal pain,bloating,fatigue,anorexia,weight loss and fever. Serum alpha fetoprotein （AFP） and carcino-embyronic antigen （CEA） were negative. The right diaphragm elevated in chest X-ray. The liver showed single or multiple echo-free areas of various sizes and a rich blood supply within the area. Liver showed large or multiple low-density areas and nodular edge enhancement with enhanced scan. The disease could be confirmed by biopsy or surgical pathology. The average survival period is about 5 months. Conclusion Hepatic angiosarcoma is a rare malignant tumor and the diagnosis mainly relies on the pathological and immunohistochemistry. Surgery resection,radiation and chemotherapy do not provide uptimistic outcomes.