PDF(Pubmed)
Abstract：
Olfactory neuroblastoma (ONB) is an uncommon neuroendocrine malignancy arising from the olfactory neuroepithelium. ONB frequently presents with nonspecific sinonasal complaints, including nasal obstruction and epistaxis, and diagnosis can be obtained through a combination of physical examination, nasal endoscopy, and computed tomography and magnetic resonance imaging. Endoscopic resection with negative margins, with or without craniotomy, as necessary, is the standard of care for definitive treatment of ONB. Regional metastasis to the neck is often detected at presentation or may occur in a delayed fashion and should be addressed through elective neck dissection or radiation. Adjuvant radiotherapy should be considered, particularly in the case of high grade or tumor stage, as well as positive surgical margins. Systemic therapy is an area of active investigation in both the neoadjuvant and adjuvant setting, with many advocating in favor of induction chemotherapy for significant orbital or intracranial involvement prior to surgical resection. Various targeted immunotherapies are currently being studied for the treatment of recurrent or metastatic ONB. Prolonged locoregional and distant surveillance are indicated following definitive treatment, given the tendency for delayed recurrence and metastasis.
摘要：
嗅觉神经母细胞瘤（ONB）是一种罕见的神经内分泌恶性肿瘤，起源于嗅觉神经上皮。ONB经常表现为非特异性鼻窦不适，包括鼻塞和鼻出血,可以通过身体检查的组合来获得诊断，鼻内窥镜检查,计算机断层扫描和磁共振成像。内镜切除切缘阴性，不管有没有开颅手术，如有必要，是ONB最终治疗的护理标准。颈部的局部转移通常在出现时检测到，或者可能以延迟的方式发生，应通过选择性颈部解剖或放射来解决。应考虑辅助放疗，特别是在高级别或肿瘤阶段的情况下，以及积极的手术切缘。系统治疗是新辅助和辅助治疗的积极研究领域，许多人主张在手术切除前对严重的眼眶或颅内受累进行诱导化疗。目前正在研究各种靶向免疫疗法用于治疗复发性或转移性ONB。明确治疗后，需要长期的局部和远处监测，考虑到延迟复发和转移的趋势。
