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嗅神经母细胞瘤的综合治疗

Combined modality therapy of esthesioneuroblastoma.

作者信息

McLean J Nicolas, Nunley Sunjay R, Klass Carmen, Moore Charles, Müller Susan, Johnstone Peter A S

机构信息

Department of Otolaryngology-Head and Neck Surgery, Emory University School of Medicine, Atlanta, GA 30322, USA.

出版信息

Otolaryngol Head Neck Surg. 2007 Jun;136(6):998-1002. doi: 10.1016/j.otohns.2006.11.051.

Abstract

OBJECTIVE

Esthesioneuroblastoma (ENB) is a rare tumor of the olfactory epithelium. The objective of this study was to evaluate treatment modalities including surgery, IMRT, and chemotherapy and patient outcomes.

PATIENTS AND METHODS

A retrospective analysis was performed on a total of 21 patients. Therapy included craniofacial resection (CFR), radiotherapy, chemotherapy, or a combination of these methods.

RESULTS

The median follow-up period was 47 months. Surgery was performed in 90.4% of cases; radiotherapy was performed adjuvantly in 15 (72.7%) patients. Surgery, radiotherapy, and chemotherapy were administered to 7 (33.3%) patients. Eight (38.3%) patients had local recurrence. The 5-year crude overall survival was 71.4% and actuarial 5-year overall survival was 58% with confidence interval (CI, 25 and 81, respectively). The 5-year crude disease-free survival rate was 59% and the 5-year actuarial disease-free survival rate was 62% (CI, 28 and 83, respectively).

CONCLUSION

Multidisciplinary therapy of ENB should be considered, especially for Kadish C and high-grade lesions. Craniofacial resection (CFR), Intensity modulated radiation therapy (IMRT), and chemotherapy should be investigated in a multi-institution trial of ENB.

摘要

目的

嗅神经母细胞瘤(ENB)是一种罕见的嗅上皮肿瘤。本研究的目的是评估包括手术、调强适形放疗(IMRT)和化疗在内的治疗方式以及患者的预后情况。

患者与方法

对总共21例患者进行回顾性分析。治疗方法包括颅面切除术(CFR)、放疗、化疗或这些方法的联合应用。

结果

中位随访期为47个月。90.4%的病例接受了手术治疗;15例(72.7%)患者接受了辅助放疗。7例(33.3%)患者接受了手术、放疗和化疗。8例(38.3%)患者出现局部复发。5年粗总生存率为71.4%,5年精算总生存率为58%,置信区间分别为25和81。5年粗无病生存率为59%,5年精算无病生存率为62%(置信区间分别为28和83)。

结论

应考虑对ENB进行多学科治疗,尤其是对于卡迪什C期和高级别病变。应在ENB的多机构试验中研究颅面切除术(CFR)、调强适形放疗(IMRT)和化疗。

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