小儿胆道葡萄状肉瘤(附1例及文献复习)

目的：提高对小儿胆道胚胎型横纹肌肉瘤(葡萄状肉瘤)的认识，减少误诊。方法：对本病例及国内文献报道的胆道胚胎型横纹肌肉瘤(葡萄状肉瘤)病例共16例进行分析。结果：男11例，女5例。年龄1～6岁。黄疸16例，发热8例，右上腹痛6例，陶土色大便9例，尿黄6例。肝肿大13例，脾肿大2例，右上腹肿块2例。临床误诊为肝炎5例。B超考虑葡萄状肉瘤仅2例，CT考虑胆总管横纹肌肉瘤仅1例。病检诊断：胚胎型横纹肌肉瘤3例，葡萄状肉瘤13例。7例切除并化疗、放疗，随访最长的8个月未复发。1例部分切除并化疗，3个月后随访残留灶缩小，病情稳定。2例行姑息手术后2个月左右死亡。1例切除后2个月检查已复发。结论：小儿胆道横纹肌肉瘤为胚胎型横纹肌肉瘤(葡萄状肉瘤)，发病年龄1～6岁，男性比女性多见。其主要症状为阻塞性黄疸，常伴有发热、肝肿大等，临床易误诊为肝炎。B超、CT等辅助检查有助于诊断，确诊需经病理检查。本病恶性程度高，预后差，应积极外科治疗并配合化疗、放疗，可延长生存时间。

Embryonal rhabdomyosarcoma (sarcoma botryoides) of biliary tree in childhood: report of 1 case and literature review

Objective:To improve the understanding and diagnosis of embryonal rhabdomysarcoma of biliary tree in childhood.Methods:The management of 1 child with sarcoma botryoides of biliary tree and other 15 cases reported were reviewed.Results:11 cases of boys and 5 of girls,varying from 1 to 6 years old.Clinical symptoms at presentation included jaundice in 16/16,fever 8/16,abdominal pain 6/16,white feces 9/16 and hepatomegaly 13/16, etc.5 cases of which were misdiagnosed as having hepatitis.There were only 2 cases considered as rhabdomyosarcoma by ultrasound and 1 by abdominal CT scan.7 cases had not recurred in 8 months after combined treatment of complete surgical excision,chemotherapy and radiotherapy.Treated with combination of partial excision and chemotherapy,1 case's tumor turned smaller 3 months later.But 2 cases treated solely with partial excision died 2 months later and 1 case recurred 2 months later.Conclusion:All rhabdomyosarcoma of biliary tree in childhood are botryoid,embryonal rhabdomyosarcoma.The patients are aged from 1 to 6 years old,and boys are more common than girls.The main clinical symptoms are obstructive jaundice,fever and hepatomegaly,so it is easily misdiagnosed as hepatitis in clinic.Ultrasound and abdominal CT sacn are helpful in diagnosing embryonal rhabdomysarcoma (sarcoma botryoides) of biliary tree,but pathological examination is essential for confirming the disease.A combined approach of aggressive surgery,chemotherapy and radiotherapy may result in long-term survival.