肝脏未分化胚胎性肉瘤4例

Diagnosis and treatment of undifferentiated embryonal sarcoma of the liver:A retrospective study of four cases

目的:探讨肝脏未分化胚胎性肉瘤(undifferen-tiated embryonal sarcoma of the liver,UESL)的诊断与治疗.方法:回顾性总结分析本院2005-2011年住院手术治疗并经术后病理组织学检查确认的4例UESL患者的临床资料.结果:男1例,女3例,年龄分别为6岁、14岁、14岁和46岁.既往无肝病背景,其临床表现主要为腹部包块、腹痛和发热等.术前常规化验检查显示肝功能基本正常,肝炎病毒标志物和甲胎蛋白等肿瘤标志物均为阴性.术前影像学检查确诊较困难,术后病理组织学检查均确诊为UESL.所有患者均行根治性手术切除,其中2例因出现发热和腹膜炎体征而疑有肿瘤破裂出血,转行急诊手术治疗,并于术后联合行化学药物辅助治疗.结论:UESL是一种少见的高度恶性肿瘤,无特异性临床表现,术前难以确诊;治疗上仍以手术完整切除肿瘤为首选,联合化疗等综合辅助治疗可进一步提高疗效.

AIM： To explore the diagnosis and treatment of undifferentiated embryonal sarcoma of the liver （UESL）. METHODS： Clinical data for four patients with pathologically confirmed UESL who were treat- ed at our hospital between 2005 and 2011 were retrospectively analyzed. RESULTS： Three children （2 female and I male） and one female adult were included. Their age was 14, 14, 6 and 46 years old, respectively. Clini- cal presentation was typically an abdominal mass that may be accompanied by pain and/or fever. Diagnosis relies on histopathological examina-tion and it is difficult for radiological examination to establish a definite diagnosis. Tumors were removed surgically in all four patients, and two patients received adjuvant chemotherapy. CONCLUSION： UESL is an unusual malignancy of the liver. Whenever feasible, radical resection should be attempted as a part of combination modalities. Multiagent adjuvant chemotherapy and supportive therapy might improve sur- vival.