成人巨大肝脏未分化胚胎性肉瘤的外科诊疗经验

Surgical diagnosis and treatment of adult huge undifferentiated embryonal sarcoma of the liver

目的探讨成人巨大肝脏未分化胚胎性肉瘤(UESL)的临床诊疗特点。方法回顾性分析2005年1月至2020年12月安徽医科大学第一附属医院和安徽医科大学第二附属医院收治的行手术切除并经病理学检查结果证实的5例成人巨大UESL患者的临床资料,均为女性,年龄49~77岁。术前CT检查结果均提示为密度不均的囊实性低密度影伴有稍高密度条索状分隔影,被误诊为肝细胞癌或肝囊腺癌。3例CA125高于正常值,5例甲胎蛋白均为阴性。结果5例患者均接受手术治疗,肿瘤平均最大径20.2 cm(范围:15.0~30.0 cm),术后病理学检查结果均提示为UESL,5例免疫组化均表达波形蛋白;3例完整切除肿瘤并达到R0切除,其中2例截至末次随访仍无瘤生存(89和55个月),另1例无瘤生存158个月后死于肾癌;其余2例虽完整切除肿瘤但术中肿瘤破裂,分别于术后2和19个月复发,总体生存时间分别为3和26个月。结论外科根治性切除是成人巨大UESL的首选治疗方式,术中应避免肿瘤破裂。肿瘤种植转移是影响UESL预后的主要因素。

Objective To investigate the clinical characteristics of adult undifferentiated embryonal sarcoma of the liver(UESL).Methods A retrospective analysis was performed on the clinical data of 5 patients with UESL who underwent surgical resection and were pathologically confirmed from January 2005 to December 2020 at the First and the Second Affiliated Hospital of Anhui Medical University.All the patients were female aged from 49 to 77 years old.Preoperative CT showed a solid cystic mass with low density and a slight density of cord like septum.Imaging findings were misdiagnosed as hepatocellular carcinoma or cystadenocarcinoma.CA125 was higher in 3 patients,and AFP in all patients was normal.Results All patients were treated by surgery.The mean diameter of tumor was 20.2 cm(range:15.0 to 30.0 cm).All five patients had vimentin expression in immunohistochemistry.Three cases underwent complete resection of the tumor and achieved R0 resection,2 of them had tumor free survival until the end of the follow-up(89 and 55 months),the other 1 case died from renal cell carcinoma 158 months later.The remaining 2 cases were radically resected,but the tumors were ruptured during operation,and relapsed after 2 months and 19 months respectively.The overall survival was 3 and 26 months respectively.Conclusions Radical hepatectomy is the first choice for treatment of UESL.Intraoperative tumor rupture should be avoided and implant metastasis is a major factor affecting the prognosis of UESL.