37例青少年横纹肌肉瘤的临床观察

Clinical Observation of Rhabdomyosarcoma in 37 Adolescents

目的探讨青少年横纹肌肉瘤的临床疗效及预后。方法对我院2010年1月至2017年12月收治的37例青少年横纹肌肉瘤患者的临床资料进行分析,包括性别、年龄、肿瘤原发部位、病理类型、分期、治疗方法、疗效及生存期。有2例单纯手术,2例行单纯放疗,13例仅行全身化疗,20例行手术联合放化疗。化疗方案均含有长春新碱、放线菌素D、表阿霉素、环磷酰胺等药物。单纯全身化疗的患者,行12周期VAD方案化疗。手术联合放化疗的患者,术后行6周期的VAC方案化疗,配合局部放疗,放疗结束后再行4周期的VDC方案化疗。按照实体瘤疗效评价标准(RECIST)评价近期客观疗效,随访。结果男22例,女15例,年龄13至30岁,中位年龄22岁。根据美国横纹肌肉瘤研究组(IRS)的分期标准,I期7例、Ⅱ期4例、Ⅲ期6例、Ⅳ期20例。所有患者均经病理组织活检及免疫组化确诊,胚胎型20例,腺泡型13例,多形型4例。有2例单纯手术,2例行单纯放疗,13例仅行全身化疗,20例行手术联合放化疗。随访至2018年3月30日,存活9例,死亡28例。结论青少年横纹肌肉瘤常见病理类型为胚胎型,治疗以手术、放疗、化疗为主,但其恶性度高,死亡率高,需要多学科综合协作有望提高生存率,其预后与肿瘤分期及病理类型可能有关。

Objective To investigate the clinical efficacy and prognosis of juvenile rhabdomyosarcoma.Methods The clinical data of 37 patients with rhabdomyosarcoma admitted to our hospital from January 2010 to December 2017 were analyzed.Results There were 22 males and 15 females,ranging in age from 13 to 30 years,with a median age of 22 years.According to the staging criteria of the American rhabdomyosarcoma research group(IRS),there were 7 cases in stage I,4 cases in stage II,6 cases in stage III,and 20 cases in stage IV.All patients were confirmed by histopathological biopsy and immunohistochemistry.There were 20 cases of embryonal type,13 cases of acinar type and 4 cases of pleomorphic type.There were 2 cases of simple operation,2 cases of radiotherapy alone,13 cases of systemic chemotherapy only,and 20 cases of surgery combined with radiotherapy and chemotherapy.Follow up to March 30,2018,9 cases survived and 28 died.Conclusion The common pathological type of juvenile rhabdomyosarcoma is embryonal type.It is mainly treated with surgery,radiotherapy and chemotherapy,but its malignant degree is high and the mortality is high.It is expected to improve the survival rate by multidisciplinary comprehensive cooperation.The prognosis is likely to be related to the tumor staging and pathological types.