成人原发性肝肉瘤四例诊治分析

Adult primary hepatic sarcomas

目的探讨成人原发性肝肉瘤的临床特点、诊断及治疗。方法回顾性分析我院自1998年至2008年收治的4例原发性肝肉瘤患者的临床资料，总结其发病特点、检查结果、诊断、治疗及随访。结果4例患者中男3例，女1例，年龄25～65岁，分别因腹痛、乏力、消瘦及体检发现肿块而就诊，无明显家族史，肝脏酶学检测正常或稍增高，CT表现大多见肝内较大肿块，囊实性为多，密度不均匀，边界欠清，增强后无明显强化或部分强化；分别误诊为肝囊肿、肝癌、肝腺瘤，均行肝叶切除术。术后病理学诊断分别为：恶性间叶瘤（未分化肉瘤）、癌肉瘤、平滑肌肉瘤、恶性纤维组织细胞瘤部分血管肉瘤。现存活3例（已分别为术后4、56、84个月），死亡1例（术中死亡），其中1例术后5年肝内复发。结论原发性肝肉瘤的诊断容易误诊，确诊需依赖术后病理及免疫组织化学检查。采用以手术治疗为主的综合治疗是延长患者生存期的主要手段。患者的预后与肿瘤的病理类型、分化程度、是否浸润转移及是否手术切除等密切相关。

Objective To discuss the clinical features, diagnostic methods and treatment of the primary hepatic sarcomas. Methods The clinical data of 4 patients with primary hepatic sarcoma admitted to our hospital from January 1998 to December 2008 were viewed. Results There were 3 males and 1 female with age from 25 to 65 years. Abdominal pain, fatigue, weight loss and mass were among the complains. All 4 cases were misdiagnosed as liver cyst, liver cancer and liver adenoma respectively, and final diagnosis was established only by laparotomy and pathology. They were malignant mesenchymoma, ,leiomyosarcoma and malignant fibrous histiocytoma respectively. Till now, 3 cases were still alive （respectively 4, 56 and 84 months after surgery）, 1 case died intraoperatively. One case recurred 5 years after the operation. Conclusions Primary liver sarcoma is difficult to diagnose. Postoperative pathology and immunohistochemical examination remain the mainstay for final diagnosis. The prognosis is dependent on pathological type, differentiation, tumor invasion and metastasis.