Neoadjuvant transcatheter arterial chemoembolization and systemic chemotherapy for the treatment of undifferentiated embryonal sarcoma of the liver in children  

作　　者：Min He Jia-Bin Cai Can Lai Jun-Qing Mao Jie-Ni Xiong Zhong-Hai Guan Lin-Jie Li Qiang Shu Mei-Dan Ying Jin-Hu Wang 

机构地区：[1]Department of Surgical Oncology,The Children’s Hospital,Zhejiang University School of Medicine,National Clinical Research Center for Child Health,Hangzhou 310052,Zhejiang Province,China [2]Department of Radiology,The Children’s Hospital,Zhejiang University School of Medicine,National Clinical Research Center for Child Health,Hangzhou 310052,Zhejiang Province,China [3]Cancer Center,Zhejiang University,Hangzhou 310058,Zhejiang Province,China

出　　处：《World Journal of Clinical Cases》2022年第19期6437-6445,共9页世界临床病例杂志

基　　金：Youth Program of Natural Science Foundation of Zhejiang Province,No.LQ20H160027;National Natural Science Foundation of China,No.U20A20137。

摘　　要：BACKGROUND Undifferentiated embryonal sarcoma of the liver(UESL)is a rare and aggressive mesenchymal tumor in children.Herein,we describe our experience in neoadjuvant therapy(NAT)and subsequent surgery for the treatment of UESL in children.AIM To evaluate the efficacy of NAT and explore a new choice for successful operation of UESL in children.METHODS We retrospectively analyzed six patients newly diagnosed with unresectable UESL who received NAT and then surgery at our center between January 2004 and December 2019.The tumor was considered unresectable if it involved a large part of both lobes of the liver or had invaded the main hepatic vessels or inferior vena cava.The NAT included preoperative transcatheter arterial chemoembol ization(TACE)and systemic chemotherapy.The patients were 4 boys and 2 girls with a mean age of 7 years.The longest tumor at presentation ranged from 8.6 to 14.8 cm(mean,12 cm).Extrahepatic metastases were present in 2 cases.Preoperative systemic chemotherapy was administered 3 wk after TACE.Tumor resection was performed 3 wk after one or two cycles of NAT.The patients received systemic chemotherapy after surgery.RESULTS All patients successfully underwent NAT and complete resection.The tumor volumes decreased by 18.2%–68.7%,with a mean decrease of 36%after 1 cycle of NAT(t=3.524,P=0.017).According to the Response Evaluation Criteria In Solid Tumors criteria,4 patients had a partial response and underwent surgery,while 2 had stable disease and received another cycle of NAT before surgery.Massive tumor necrosis was seen on pathological examination of the surgical specimen:>90%necrosis in two,>50%necrosis in three,and 25%necrosis in 1,with an average of 71.8%.Post-NAT complications included fever,nausea and vomiting,and mild bone marrow suppression.Elevated alanine transaminase levels occurred in all patients,which returned to normal within 7–10 d after treatment.No cardiac or renal toxicity,severe hepatic dysfunction,bleeding and nontarget embolization were observed in the patients.Th

关 键 词：Undifferentiated embryonal sarcoma of the liver UNRESECTABLE Neoadjuvant therapy Transcatheter arterial chemoembolization Systemic chemotherapy 

分 类 号：R735.7[医药卫生—肿瘤]