儿童鼻部横纹肌肉瘤的临床特点分析

Analysis of clinical features of rhinosarcoma in children

目的总结分析儿童鼻部横纹肌肉瘤的临床表现、诊断及手术治疗。方法收集昆明市儿童医院2013年6月~2019年8月16例鼻部横纹肌肉瘤患儿的临床资料,并进行分析。结果胚胎型12例,腺泡型4例,I期3例(单纯手术治疗),Ⅱ期3例(手术+化疗),Ⅲ期10例(5例活检+化疗,5例活检+化疗+放疗)。随访时间6个月~6年,其中Ⅱ期1例,化疗后复发,予以手术切除肿瘤后二次化疗;Ⅲ期1例,化疗后病灶局限,行手术治疗,术后继续放化疗,随访6个月未见复发及转移。结论肿瘤原发于鼻腔及鼻前庭,病灶较局限,可行手术切除,再行放化疗。肿瘤原发于鼻咽部及鼻窦,可行活检确诊,待放化疗使病灶局限后再行手术治疗。

OBJECTIVE To summarize and analyze the clinical manifestations,diagnosis and surgical treatment of nasal rhabdomyosarcoma in children.METHODSThe clinical data of 16 children with rhabdomyosarcoma of the nose in Kunming Children’s Hospital,from June 2013 to August 2019 were collected and analyzed.RESULTS Twelve cases of embryonic type,4 cases of acinar type,3 cases of stage I(surgery alone),3 cases of stage II(surgery+chemotherapy),10 cases of stage III(5 biopsy+chemotherapy,5 biopsy+surgery+radiotherapy).The follow-up time was 6 months to 6 years.Among them,one child in stage II relapsed after chemotherapy and received second chemotherapy after surgical removal of the tumor;one child in stage III had limited lesions after chemotherapy and received surgical treatment,and continued radiotherapy and chemotherapy.No recurrence or metastasis was found during follow-up for half a year.CONCLUSION The tumor originated in the nasal cavity and nasal vestibule,and the lesions were relatively localized.It can be surgically removed,followed by radiotherapy and chemotherapy.The tumor originated in the nasopharynx and nasal sinuses,which can be diagnosed by biopsy,and surgery should be performed after radiotherapy and chemotherapy have limited the lesion.