目的探讨原发性肝脏癌肉瘤的临床病理学特征、诊断和治疗。方法报道1例原发性肝脏癌肉瘤的临床资料,并结合文献进行复习。结果肝脏癌肉癌均为肿瘤中同时包含恶性上皮成分和间叶成分,两者之间无移行过渡,免疫组化显示肿瘤分别表达上皮源性和间叶源性的标志物。该例术后生存8个月,后失访。结论原发性肝脏癌肉瘤临床罕见,预后差,首选治疗方式为早期根治性手术切除。 Objective To investigate the clinicopathological features, diagnosis and treatment of primary liver carcinosarcoma. Methods One case of primary hepatic carcinosarcoma was reported, and its clinical data were reviewed. Results All of the cancerous carcinomas of the liver were composed of both malignant and mesenchyme components in the tumor. There was no transitional transition between them. Immunohistochemistry showed that the tumors expressed epithelial and mesenchymal markers respectively. The case survived 8 months after the lost. Conclusions Primary liver carcinosarcoma is rare in clinical practice and its prognosis is poor. The preferred treatment is radical radical resection.