儿童胆管胚胎型横纹肌肉瘤的诊治

　　Diagnosis and treatment of embryonal rhabdomyosarcoma of biliary tract in children

　　儿童胆管胚胎型横纹肌肉瘤的诊治

　　Abstract:Objective To analyze the diagnosis and treatment of embryonal rhabdomyosarcoma of biliary tract in children.Methods The clinical symptoms and treatment of 4 children with embryonal rhabdomyosarcoma of biliary tree were retrospectively reviewed.Results Three boys and one girl,aged from 22 months to 65 months, were diagnosed with embryonic rhabdomyosarcoma of biliary tract.Jaundice presented in all patients.Of the 4 patients, 1 had fever, 1 had abdominal pain, 3 had white feces, and 1 had pruritis; 1 had the right upper abdomen mass, 1 complicated with stones.Two cases were misdiagnosed as hepatitis initially.Tumor recurred and metastasized in one case 1.5 years after operation.One case has stayed alive for 2 months so far.The other 2 cases were lost to follow up.Conclusions Embryonal rhabdomyosarcoma of the biliary tract in children is a rare and poorly prognostic tumor.Ultrasound, abdominal CT scan and MRI are helpful to make the diagnosis.Comprehensive treatments should be carried out to improve the prognosis of this disease.

　　目的：分析少儿胆管胚胎型横纹肌肉瘤的诊治。方法 ：回顾4个患胆道系统的胚胎型横纹肌肉瘤症状及治疗。结果 3例男孩和1例女孩 ，年龄从1岁10个月到5岁5个月大，确诊为胆管胚胎型横纹肌肉瘤。都有胆汁。4例，1例黄疸，1例腹痛，3例陶土色大便，及1例皮肤瘙痒；1例右上腹肿块，1例患有结石。2例最先误诊为肝炎。1例术后1年半复发并肝转移，1例手术后2个月至今存活。其他2例没有调查到。结论 少儿胆管胚胎型横纹肌肉瘤罕见，预后不良。CT及MRI 对诊断有帮助。综合治疗提高存活率。