Alotaibi Hammam A, Priola Stefano M, Bernat Anne-Laure, Farrash Faisal
Miscellaneous, Prince Sultan Military Medical City, Riyadh, SAU.
Neurosurgery, University of Toronto, Toronto, CAN.
Cureus. 2019 Jun 13;11(6):e4897. doi: 10.7759/cureus.4897.
Esthesioneuroblastoma (ENB) is a rare malignant tumor of the nasal cavity. The genetic basis of its development is still under study and has not been fully delineated. It has varying symptoms depending on the lesion's location within the nasal cavity. The most commonly used systems for such lesions are the Kadish staging and Haymes grading systems. The objectives are to review the most recent published literature evaluating the different treatments/ combination of treatment and assess the most appropriate treatment modality that can provide the longest progression-free survival and overall survival for ENB patients. Moreover, a look at what the latest literature suggests when it comes to adjuvant treatments and their effect on survival is also key to further the body of knowledge for neurosurgeons, Ears, Nose, and Throat (ENT) physicians and all the different subspecialties that deal and serve these population of patients. The published literature was reviewed starting from 1990. The focus was made on single-center experiences given their availability and easy access. The most recently published systematic review was used as the benchmark; research published after that was included in this study. The database search in OVID was conducted using the following keywords: "Esthesioneuroblastoma", "ENB", "Olfactory Neuroblastoma", Nose neoplasm", skull base neoplasm", "radiation", and "resection". The database search found 17 papers which included 14 single-center reports, one systematic review, and two nationwide multi-center reviews. Surgery plus adjuvant radiation therapy appears to provide the best overall survival and progression-free survival especially in patients with high Kadesh grade. On the other hand, surgery alone or biopsy followed with radiation therapy provided the lower progression-free survival and overall survival from time of diagnosis. The role of chemotherapy, however, requires further investigation to assess its potentially harmful effects. The use of surgery as a stand-alone modality of treatment should be cautiously and rarely used in patients with lower staging scores and multiple negative resection margins.
嗅神经母细胞瘤(ENB)是鼻腔罕见的恶性肿瘤。其发生发展的遗传基础仍在研究中,尚未完全阐明。根据鼻腔内病变位置的不同,其症状也有所不同。对于此类病变,最常用的分期系统是卡迪什分期系统和海姆斯分级系统。目的是回顾评估不同治疗方法/联合治疗的最新发表文献,并评估能为ENB患者提供最长无进展生存期和总生存期的最合适治疗方式。此外,了解最新文献中关于辅助治疗及其对生存影响的内容,对于神经外科医生、耳鼻喉科(ENT)医生以及所有诊治这些患者群体的不同亚专业领域进一步积累知识也至关重要。从1990年开始回顾已发表的文献。鉴于单中心经验易于获取,因此重点关注了单中心经验。以最近发表的系统评价作为基准,该研究纳入了在此之后发表的研究。在OVID数据库中使用以下关键词进行搜索:“嗅神经母细胞瘤”、“ENB”、“嗅觉神经母细胞瘤”、“鼻肿瘤”、“颅底肿瘤”、“放疗”和“切除术”。数据库搜索找到17篇论文,其中包括14篇单中心报告、1篇系统评价和2篇全国性多中心综述。手术加辅助放疗似乎能提供最佳的总生存期和无进展生存期,尤其是对于卡迪什分级高的患者。另一方面,单纯手术或活检后放疗从诊断时起提供的无进展生存期和总生存期较低。然而,化疗的作用需要进一步研究以评估其潜在的有害影响。对于分期较低且手术切缘阴性的患者,单独使用手术作为治疗方式应谨慎且很少使用。
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