Abstract：
Esthesioneuroblastoma (ENB) is a rare sinonasal malignant neoplasm with 40% 5-year survival. Because of the rarity of the tumor, the optimal treatment and subsequent prediction of prognosis are unclear. We studied a modern series of patients with ENB to evaluate the association of immunohistochemical (IHC) markers and clinical stages/grades with outcomes.
A single-center retrospective review of patients with ENB treated during a 25-year period was performed. A systematic literature review evaluating the prognostic benefits of current staging systems in evaluating survival outcomes in ENB was undertaken.
Among 29 included patients, 25 (85%) were treated surgically at our institution, with 76% of those endoscopically resected; 7 (24.1%) received chemotherapy, and 18 (62.1%) received radiation therapy. The 5-year overall survival (OS) was 91.3%, and 10-year OS was 78.3%. Progression-free survival at 5 and 10 years was 85.6% and 68.2%, respectively. A total of 36 distinct IHC markers were used to diagnose ENB but were inconsistent in predicting survival. A systematic literature review revealed predictive accuracy for OS using the Kadish, TNM, and Hyams staging/grading systems was 68%, 42%, and 50%, respectively.
This study reports the 5- and 10-year OS and progression-free survival in a modern series of patients with ENB. No traditional IHC marker consistently predicted outcome. Some novel reviewed markers show promise but have yet to enter clinical mainstream use. Our systematic review of accepted staging/grading systems also demonstrated a need for further investigation due to limited prognostic accuracy.
A single-center retrospective review of patients with ENB treated during a 25-year period was performed. A systematic literature review evaluating the prognostic benefits of current staging systems in evaluating survival outcomes in ENB was undertaken.
Among 29 included patients, 25 (85%) were treated surgically at our institution, with 76% of those endoscopically resected; 7 (24.1%) received chemotherapy, and 18 (62.1%) received radiation therapy. The 5-year overall survival (OS) was 91.3%, and 10-year OS was 78.3%. Progression-free survival at 5 and 10 years was 85.6% and 68.2%, respectively. A total of 36 distinct IHC markers were used to diagnose ENB but were inconsistent in predicting survival. A systematic literature review revealed predictive accuracy for OS using the Kadish, TNM, and Hyams staging/grading systems was 68%, 42%, and 50%, respectively.
This study reports the 5- and 10-year OS and progression-free survival in a modern series of patients with ENB. No traditional IHC marker consistently predicted outcome. Some novel reviewed markers show promise but have yet to enter clinical mainstream use. Our systematic review of accepted staging/grading systems also demonstrated a need for further investigation due to limited prognostic accuracy.
摘要：
目的：鼻腔神经母细胞瘤（ENB）是一种罕见的鼻腔鼻窦恶性肿瘤，5年生存率为40％。因为肿瘤的稀有性,最佳治疗和随后的预后预测尚不清楚.我们研究了一系列现代ENB患者，以评估免疫组织化学（IHC）标志物和临床分期/分级与结果的关联。
方法：对25年期间接受ENB治疗的患者进行单中心回顾性分析。进行了系统的文献综述，评估了当前分期系统在评估ENB生存结果中的预后益处。
结果：在29名患者中，25（85％）在我们的机构接受了手术治疗，其中76％的内窥镜切除;7（24.1％）接受化疗，18人(62.1%)接受放射治疗。5年总生存率（OS）为91.3%，10年OS为78.3%。5年和10年的无进展生存率分别为85.6％和68.2％，分别。总共使用36种不同的IHC标记来诊断ENB,但在预测存活方面不一致。系统的文献综述揭示了使用Kadish的操作系统的预测准确性，TNM,Hyams分期/分级系统占68%，42%,50%,分别。
结论：本研究报告了现代系列ENB患者的5年和10年OS和无进展生存期。没有传统的IHC标记一致地预测结果。一些新的审查标记显示出希望，但尚未进入临床主流使用。我们对公认的分期/分级系统的系统评价也表明，由于预后准确性有限，需要进一步研究。
方法：对25年期间接受ENB治疗的患者进行单中心回顾性分析。进行了系统的文献综述，评估了当前分期系统在评估ENB生存结果中的预后益处。
结果：在29名患者中，25（85％）在我们的机构接受了手术治疗，其中76％的内窥镜切除;7（24.1％）接受化疗，18人(62.1%)接受放射治疗。5年总生存率（OS）为91.3%，10年OS为78.3%。5年和10年的无进展生存率分别为85.6％和68.2％，分别。总共使用36种不同的IHC标记来诊断ENB,但在预测存活方面不一致。系统的文献综述揭示了使用Kadish的操作系统的预测准确性，TNM,Hyams分期/分级系统占68%，42%,50%,分别。
结论：本研究报告了现代系列ENB患者的5年和10年OS和无进展生存期。没有传统的IHC标记一致地预测结果。一些新的审查标记显示出希望，但尚未进入临床主流使用。我们对公认的分期/分级系统的系统评价也表明，由于预后准确性有限，需要进一步研究。
