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摘要: 目的:探讨影响成年人鼻腔鼻窦横纹肌肉瘤(SNRMS)临床预后的因素。方法:回顾性分析2006-01-2014-12期间收治的23例成年(年龄>18岁)SNRMS患者的临床资料。用Kaplan-Meier法进行生存分析,组间比较采用Log-rank检验,多因素分析采用Cox模型。结果:23例患者平均随访20个月,死亡14例,存活9例。全组病例1年和2年生存率分别为77.1%和35.0%。组间1年和2年生存率分析显示,TNM分期中2、3期分别为100.0%、66.7%和83.3%、10.5%(P<0.05);综合治疗组和单独治疗组分别为86.7%、50.0%和50.8%、0(P<0.05)。21例初诊非远处转移组,1年和2年远处转移率分别为38.1%和70.5%。COX多因素生存分析发现接受放疗、化疗和肿瘤直径<5 cm是SNRMS的预后良好因素;而淋巴结转移、肿瘤累及脑膜和眶内是SNRMS的预后不良因素。14例死亡患者中,1例死于局部侵犯脑膜脑组织,1例死于局部区域复发伴远处转移,12例死于远处转移。结论:成年SNRMS就诊时多晚期,预后差,远处转移是主要的死亡原因,控制远处转移率是提高生存率的关键。Abstract: Objective:To investigate the clinical prognostic impact factors of adult sinonasal rhabdomyosarcoma (SNRMS). Method:The clinical features,treatment methods,and disease outcome were reviewed retrospectively for twenty-three adult SNRMS between 2006 January and 2014 December. The survival analysis was performed by Kaplan-Meier estimate and the comparison between groups by Log-rank test. Multivariate analysis was carried out by Cox proportional hazard model.Result:Patients' ages ranged from 18 to 59 years (median, 23.2 years).With a median follow-up of 20 moths (3-47 moths), 14 cases dead and 9 cases alive, the 1-year and 2-year overall survival (OS) rates were 77.1% and 35.0%, respectively. Within the 1-year and 2-year OS rates,early stage group had a higher overall survival rates than advanced diease group (100.0%,66.7% and 83.3%,10.5%, P<0.05); combined therapy group had a higher overall survival rates than single treatment group (86.7%,50.0% and 50.8%,0, P<0.05).In the non-metastasis group (21 cases), 1-year and 2-year distant metastasis rates were 38.1% and 70.5%, respectively. Multivariate analysis showed that radiotherapy, chemotherapy and tumor diameter less than 5 cm were good prognostic factors (P<0.05), while the lymph node metastasis,meningeal involvement and orbital involvement were poor prognostic factors (P<0.05). In the 14 cases of dead patients, 92.8% (13/14) died of distant metastasis.Conclusion:Adult RMS had a high advanced rate with poor prognosis. Distant metastasis is the leading cause of death. Controlling distant metastasis is a key to improve the survival rate.
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Key words:
- nasopharyngeal nepplasms /
- rhabdomyosarcoma /
- combined therapy /
- prognostic factors
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