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小儿头颈部横纹肌肉瘤

Pediatric rhabdomyosarcoma of the head and neck.

作者信息

Kraus D H, Saenz N C, Gollamudi S, Heller G, Moustakis M, Gardiner S, Gerald W L, Ghavimi F, LaQuaglia M P

机构信息

Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, New York 10021, USA.

出版信息

Am J Surg. 1997 Nov;174(5):556-60. doi: 10.1016/s0002-9610(97)00171-2.

DOI:10.1016/s0002-9610(97)00171-2
PMID:9374237
Abstract

PURPOSE

Survival for pediatric rhabdomyosarcoma has improved with the use of multidrug chemotherapy and external beam radiotherapy. This study was performed to determine survival in a cohort of patients treated on one of three multidrug treatment protocols for head and neck rhabdomyosarcoma and to identify factors that place patients at risk for treatment failure.

METHODS

Pertinent prognostic variables including age, sex, subsite of origin, resectability, and TNM stage were analyzed by the Kaplan-Meier methods with comparisons between variables performed using the Prentice-Wilcoxon test statistic.

RESULTS

Overall 5-year survival was 74% (95% confidence interval 64% to 84%). Local failure accounted for the cause of death in 10 patients, and 8 died of disseminated disease. On univariate analysis, each variable contributing to the TNM staging system was significant in determining survival; invasiveness (P = 0.01), size (P = 0.02), nodal metastases (P <0.01), and distant disease (P <0.01).

CONCLUSION

Survival has improved for head and neck rhabdomyosarcoma treated with multimodality therapy. Patients with advanced-stage disease are at greatest risk for treatment failure and require the most aggressive therapy.

摘要

目的

多药化疗和外照射放疗的应用提高了小儿横纹肌肉瘤的生存率。本研究旨在确定一组接受三种头颈部横纹肌肉瘤多药治疗方案之一治疗的患者的生存率,并确定使患者面临治疗失败风险的因素。

方法

采用Kaplan-Meier方法分析包括年龄、性别、原发部位、可切除性和TNM分期等相关预后变量,并使用Prentice-Wilcoxon检验统计量对变量进行比较。

结果

总体5年生存率为74%(95%置信区间64%至84%)。局部失败是10例患者的死亡原因,8例死于播散性疾病。单因素分析显示,TNM分期系统中的每个变量在确定生存率方面均具有显著性;侵袭性(P = 0.01)、大小(P = 0.02)、淋巴结转移(P <0.01)和远处疾病(P <0.01)。

结论

多模式治疗的头颈部横纹肌肉瘤患者生存率有所提高。晚期疾病患者治疗失败风险最高,需要最积极的治疗。

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