Chang Xiaofeng,Cheng Haiyan,Qin Hong,et al.Diagnosis and treatment of rhabdomysarcoma of biliary tree in children[J].Journal of Clinical Pediatric Surgery,2020,19(07):608-613.[doi:10.3969/j.issn.1671-6353.2020.07.009]
儿童胆道横纹肌肉瘤的诊断与治疗
- Title:
- Diagnosis and treatment of rhabdomysarcoma of biliary tree in children
- Keywords:
- Biliary Tree; Rhabdomyosarcoma/DI; Rhabdomyosarcoma/TH; Child
- 分类号:
- R735.8;R729
- 摘要:
- 目的 分析儿童胆道横纹肌肉瘤(rhabdomyosarcoma of the biliary tree,RMS/BT)的临床特点、治疗策略及预后情况。方法 回顾性研究2013年1月至2020年3月首都医科大学附属北京儿童医院诊治的6例RMS/BT患儿临床资料,并对患儿预后情况及相关并发症进行随访。结果 6例RMS/BT患儿中男5例、女1例,中位发病年龄28.5个月(7~50个月),中位随访时间21个月(3~43个月)。原发于肝内胆道2例,原发于肝外胆道3例,原发于肝门部胆总管近端和左右肝管1例。肝内型主要以腹痛或局部包块为原发症状就诊,肝外型和肝门部肿瘤主要以胆道梗阻表现为首发症状就诊。根据横纹肌肉瘤治疗前TNM (tumor-node-metastasis)临床分期标准,T2bN0M0期2例,T2bNxM0期3例,T1bN0M0期1例;根据美国横纹肌肉瘤研究组术后-病理分期系统,5例为Ⅲ期,1例为Ⅰ期。RMS/BT患儿采取以手术、化疗及放疗为主的综合性治疗模式。2例行一期肿瘤切除术,4例行肿瘤活检化疗后再手术。术后6例均行化疗,3例进行了原发部位的放疗,未结束化疗的1例拟接受放射治疗。在治疗过程中1例发生肿瘤破裂、胆瘘、胆汁性腹膜炎。本组6例RMS/BT患儿中,5例存活,2例复发,1例死亡。存活的5例中,2例结束治疗无瘤生存,2例仍在治疗中,1例肿瘤复发继续治疗中。结论 RMS/BT对化疗敏感,遵循肿瘤活检、化疗、延迟手术及放疗的诊疗流程,大多能达到肿瘤根治性切除、有效减少局部复发的目的 。
- 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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备注/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