Abstract：
Olfactory neuroblastoma is a rare sinonasal malignancy arising from the olfactory epithelium that is characterized by skull base involvement and a modest natural history. Because of its rarity and long course, identification of independent prognostic factors is dependent on multivariate analysis of large, long-term data. In this review, we outline evidence for the evaluation and treatment of olfactory neuroblastoma obtained from recent large-scale population-based studies, meta-analyses and multicenter studies. Hyams grade is currently the only pathological grade system for olfactory neuroblastoma. The modified Kadish staging and Dulguerov classification are available for clinical staging. The results of large-scale studies have confirmed Hyams, the modified Kadish and Dulguerov as independent prognostic factors. Surgery followed by radiotherapy provides the best overall survival and recurrence-free survival for resectable disease. The question of whether postoperative radiotherapy should be administered for all cases or only for those at risk of recurrence remains unanswered. Exclusively endoscopic resection is indicated for modified Kadish A/B cases without any increase in the risk of death or recurrence, and is also indicated for modified Kadish C cases if a negative surgical margin is ensured. For more advanced cases, such as those with extensive brain infiltration, the open approach is indicated. Elective nodal irradiation prevents late nodal recurrence of N0 patients. Chemotherapy has failed to show a benefit in survival or disease control. Current needs for olfactory neuroblastoma include the development and validation of refined staging systems suitable for current practice; expansion of indications for endoscopic surgery; less invasive surgery; definitive radiotherapy and novel systemic therapy.
摘要：
嗅觉神经母细胞瘤是一种罕见的鼻窦恶性肿瘤，起源于嗅觉上皮，其特征是颅底受累和适度的自然史。由于它的稀有和漫长的过程，独立预后因素的识别依赖于大的多变量分析，长期数据。在这次审查中,我们概述了从最近的大规模人群研究中获得的嗅觉神经母细胞瘤评估和治疗的证据，荟萃分析和多中心研究。Hyams分级是目前嗅觉神经母细胞瘤的唯一病理分级系统。改良的Kadish分期和Dulguerov分类可用于临床分期。大规模研究的结果证实了Hyams,改良Kadish和Dulguerov作为独立的预后因素。手术后放疗为可切除的疾病提供了最佳的总生存率和无复发生存率。是否应对所有病例或仅对有复发风险的患者进行术后放疗的问题仍未解决。对于改良的KadishA/B病例，不增加死亡或复发的风险，仅需内镜切除术。如果确保手术切缘阴性，也适用于改良的KadishC病例。对于更高级的案例，比如那些有广泛大脑浸润的人，表明了开放的方法。选择性淋巴结照射可预防N0患者的晚期淋巴结复发。化疗未能显示出在生存或疾病控制方面的益处。嗅觉神经母细胞瘤的当前需求包括开发和验证适合当前实践的精细分期系统；扩大内窥镜手术的适应症；侵入性较小的手术；明确的放射疗法和新颖的全身疗法。
