Chang Xiaofeng,Cheng Haiyan,Qin Hong,et al.Diagnosis and treatment of rhabdomysarcoma of biliary tree in children[J].Journal of Clinical Pediatric Surgery,,19():608-613.[doi:10.3969/j.issn.1671-6353.2020.07.009]
Diagnosis and treatment of rhabdomysarcoma of biliary tree in children
- Keywords:
- Biliary Tree; Rhabdomyosarcoma/DI; Rhabdomyosarcoma/TH; Child
- CLC:
- R735.8;R729
- Abstract:
- Objective To explore the clinical characteristics,treatment strategies and prognosis of rhabdomysarcoma of biliary tree (RMS/BT) in childhood.Methods A retrospective analysis was performed for 6 RMS/BT children from January 2013 to March 2020.Results There were 5 boys and 1 girl with a median age of 28.5(7-50) months and a median follow-up period of 21(3-43) months.The originating places were intrahepatic biliary tract (n=2),extrahepatic biliary tract (n=3) and proximal common bile duct of hepatic hilum involving left and right hepatic ducts (n=1).Most extrahepatic or hilar cases presented initially with biliary obstruction while intrahepatic cases showed abdominal pain or local mass.According to the pre-treatment staging criteria for TNM,the types were T2bN0M0 (n=2),T2bNxM0 (n=3) and T1bN0M0 (n=1).And based upon the postoperative pathological staging system of Rhabdomyosarcoma Research Group (IRS),the stages were Ⅲ(n=5) and Ⅰ (n=1).Comprehensive treatments of surgery,chemotherapy and radiotherapy were offered.Two patients underwent primary resection and resection was delayed for the remainder after induction chemotherapy.All cases received postoperative chemotherapy.Three children received radiotherapy at primary sites.One case of chemotherapy received radiotherapy.And tumor rupture occurred in 1 case during the first diagnosis and treatment,followed by biliary fistula and biliary peritonitis.Except for one death,five children survived.Two tumor-free cases completed treatments,another two received ongoing treatments while another recurrent child was still treated.Conclusion RMS/BT is sensitive to chemotherapy.If diagnosis and treatment follow the order of tumor biopsy,chemotherapy,delayed surgery and radiotherapy,most cases may achieve radical resection so as to effectively reduce local recurrence.
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Memo
收稿日期:2020-04-02。
基金项目:中国工程院咨询研究项目（编号：2019-XY-34）
通讯作者:王焕民,Email:wanghuanmin@bch.com.cn