小儿胆道胚胎型横纹肌肉瘤的诊治

目的 回顾4例小儿胆道胚胎型横纹肌肉瘤的诊治经验,以提高对该病的认识,改善预后.方法 对4例小儿胆道胚胎型横纹肌肉瘤的临床表现、诊治等进行回顾分析.结果 男3例,女1例,年龄1岁10个月～5岁5个月,黄疸4例,发热1例,腹痛1例,陶土色大便3例,尿黄4例,皮肤瘙痒1例,右上腹肿块1例,合并结石者1例,误诊为肝炎者2例.本组1例术后1年半复发并肝转移,1例已术后2个月,尚存活,2例失访.结论 小儿胆道胚胎型横纹肌肉瘤罕见,预后差,易误诊为肝炎等而延误治疗,B超、CT、MRI等有助于本病的诊断,积极综合治疗可提高存活率.

Diagnosis and treatment of embryonal rhabdomyosarcoma of biliary tract in children

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.