肝脏未分化胚胎性肉瘤九例的临床病理特点及其治疗

目的 总结肝脏未分化胚胎性肉瘤(UESL)的临床病理学特点.方法 对11年来第二军医大学东方肝胆外科医院手术切除的9例UESL病人的临床资料进行回顾性分析.结果 该组9例年龄5～63岁,其中6例年龄5～10岁;肿瘤长径平均15.1 cm.术前6例误诊为肝囊腺瘤(癌),2例误诊为原发性肝癌,1例误诊为肝良性囊性占位,病理学检查均证实为未分化胚胎性肉瘤.结论 UESL为少见高度恶性肿瘤;CT与B超表现的不一致性为本病的重要特点;治疗上目前仍以手术切除肿瘤为首选,手术前后联合化疗及放疗等综合治疗可提高疗效.

Clinicopathological features and therapy of undifferentiated embryonal sarcoma of liver

Objective To investigate the clinicopathological features of undifferentiated embryo-nal sarcoma of the liver (UESL). Methods The clinical data of 9 UESL cases treated in our hospital between 1996 and 2007 were retrospectively analyzed. Results The patients were children and young adults. Clinical presentation was typically an abdominal mass that may be accompanied by pain and fe-ver. Tumors were all removed surgically in the 9 patients. Conclusion UESL is an unusual malignan-cy of the liver. Discrepancy of internal architecture between US and CT is one of the important charac-teristic of UESL. Whenever feasible, surgical resection should be attempted as a part of combination modalities. Modern rnultimodal treatment and supportive therapy might promote the survival rate.