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与嗅神经母细胞瘤总生存相关的患者、疾病和治疗因素。

Patient, disease, and treatment factors associated with overall survival in esthesioneuroblastoma.

机构信息

Department of Otorhinolaryngology-Head and Neck Surgery, University of Pennsylvania, Perelman School of Medicine, Philadelphia, PA.

Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA.

出版信息

Int Forum Allergy Rhinol. 2017 Dec;7(12):1186-1194. doi: 10.1002/alr.22027. Epub 2017 Oct 17.

DOI:10.1002/alr.22027
PMID:29045018
Abstract

BACKGROUND

Esthesioneuroblastomas (ENB) are uncommon and data regarding outcomes are often limited to single-institution series. The National Cancer Database (NCDB), which contains outcomes information from treatment centers across the United States, represents an opportunity to evaluate outcomes for rare diseases such as ENB across multiple institutions.

METHODS

The NCDB was queried for location codes corresponding to the nasal cavity and paranasal sinuses and the histology code for ENB. Multivariate analyses were performed to evaluate for contributing factors to overall survival.

RESULTS

A total of 1225 patients with ENB met the inclusion criteria. The 5-year overall survival was 76.2% (95% confidence interval [CI], 73.4-79.0%). Overall survival was associated with Kadish stage, grade, treatment sequence, margin status, Charlson/Deyo score, age, and gender (p < 0.05). Multivariate analysis demonstrated that, compared with surgery alone, surgery followed by radiation without chemotherapy had improved all-cause mortality (odds ratio [OR], 0.61; 95% CI, 0.40-0.95). Surgery with chemotherapy alone was associated with increased odds of all-cause mortality (OR, 4.86; 95% CI, 2.31-10.25). Multivariate subanalysis for Kadish stages A and B demonstrated no difference in survival between surgery and surgery followed by radiation, but surgery followed by chemoradiation had worse overall survival (OR, 3.03; 95% CI, 1.07-8.56). For Kadish stage C, surgery followed by radiation had improved overall survival compared with surgery alone (OR, 0.44; 95% CI, 0.24-0.81).

CONCLUSION

The most common treatment for ENB is surgery followed by radiation, which is associated with the highest overall survival. The role of adjunctive chemotherapy needs to be re-evaluated in further studies.

摘要

背景

嗅神经母细胞瘤(ENB)较为罕见,其疗效数据通常仅限于单机构系列研究。国家癌症数据库(NCDB)包含了美国各地治疗中心的疗效信息,为评估罕见疾病(如 ENB)的疗效提供了机会。

方法

通过 NCDB 检索鼻腔和副鼻窦的部位代码和嗅神经母细胞瘤的组织学代码。采用多变量分析评估与总生存率相关的因素。

结果

共有 1225 例符合纳入标准的 ENB 患者。5 年总生存率为 76.2%(95%置信区间[CI],73.4-79.0%)。总生存率与 Kadish 分期、分级、治疗顺序、切缘状态、Charlson/Deyo 评分、年龄和性别相关(p<0.05)。多变量分析显示,与单纯手术相比,手术联合放疗不化疗降低了全因死亡率(比值比[OR],0.61;95%CI,0.40-0.95)。单纯手术化疗与全因死亡率增加相关(OR,4.86;95%CI,2.31-10.25)。Kadish 分期 A 和 B 的多变量亚组分析显示,手术和手术联合放疗的生存率无差异,但手术联合放化疗的总生存率较差(OR,3.03;95%CI,1.07-8.56)。对于 Kadish 分期 C,手术联合放疗的总生存率高于单纯手术(OR,0.44;95%CI,0.24-0.81)。

结论

ENB 的最常见治疗方法是手术联合放疗,其总生存率最高。辅助化疗的作用需要在进一步的研究中重新评估。

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