儿童肝脏未分化胚胎性肉瘤诊治分析

目的探讨儿童肝脏未分化胚胎性肉瘤（UESL）的临床表现、诊断、治疗及预后特点。方法回顾2例儿童UESL并复习相关文献，从临床表现、影像学、实验室检查、治疗及预后方面对UESL进行综合分析。结果UESL主要见于5—10岁儿童。UESL的临床表现多样且无特异性，常见的临床表现为腹部肿块和腹痛。实验室检查也无特异性，肝功能、AFP多正常。影像学检查示肝脏巨大占位，瘤体多位于肝右叶，B超与CT表现的不一致性是本病的重要特点。确诊有赖于组织病理学和免疫组化检查。UESL预后较差，目前还无公认的UESL的标准治疗方案，手术、术前或术后的化疗是现行治疗方案。结论UESL是一种高度恶性肿瘤，做组织病理学检查之前，诊断困难，误诊率高，预后较差。肝脏巨大占位，AFP正常，B超与CT表现不一致，要考虑UESL的诊断。手术完整切除，同时辅以多药化疗联合治疗可明显提高患儿生存率。UESL需早诊断、早治疗，因为UESL是一种潜在可治疗肿瘤。

Diagnosis and Treatment of Undifferentiated Embryonal Srcoma of the Liver in Children

Objective To explore the clinical manifestations, diagnosis, treatment and prognosis of undifferenti- ated embryonal sarcoma of the liver （UESL） in children. Methods Two cases of UESL in children were retrospec- tively analyzed, and the literature was reviewed, the comprehensive analysis of UESL from the clinical manifesta- tions, imaging findings, laboratory data, treatment and prognosis were received. Results UESL was primarily seen between the ages of 5 and 10 years old. The clinical features of UESL were varied and often nonspeeifie. Common presenting features included large abdominal mass and abdominal pain. Laboratory studies were usually nonspeeific as well. They usually showed no abnormalities in liver function or AFP level. Imaging examination usually showed the lesion occupying a large space in the liver, the right lobe of the liver was more commonly involved. Discrepancy of imaging between ultrasound and CT was one of the important characteristics of UESL. Diagnosis depended on his- topathologieal and immunnhistoehemical examination of the mass. The prognosis of UESL was poor, now there was no standard treatment mentioned in the literature, surgery combined with chemotherapy remained the option of choice. Conclusion UESL is a highly malignant tumor, the diagnosis of UESL before histopathologic evaluation is very difficult, has a high misdiagnosis rate and poor prognosis. UESL should be included in the differential diagno- sis of mass in the liver, especially with huge liver placeholder, normal AFP and discrepancy of imaging between ul- trasound and CT. Complete tumor resection and multiagent adjuvant chemotherapy can improve the survival rate sig- nificantly. Diagnosis and management should be carried out early as UESL is a potentially treatable tumor.