McLean J Nicolas, Nunley Sunjay R, Klass Carmen, Moore Charles, Müller Susan, Johnstone Peter A S
Department of Otolaryngology-Head and Neck Surgery, Emory University School of Medicine, Atlanta, GA 30322, USA.
Otolaryngol Head Neck Surg. 2007 Jun;136(6):998-1002. doi: 10.1016/j.otohns.2006.11.051.
Esthesioneuroblastoma (ENB) is a rare tumor of the olfactory epithelium. The objective of this study was to evaluate treatment modalities including surgery, IMRT, and chemotherapy and patient outcomes.
A retrospective analysis was performed on a total of 21 patients. Therapy included craniofacial resection (CFR), radiotherapy, chemotherapy, or a combination of these methods.
The median follow-up period was 47 months. Surgery was performed in 90.4% of cases; radiotherapy was performed adjuvantly in 15 (72.7%) patients. Surgery, radiotherapy, and chemotherapy were administered to 7 (33.3%) patients. Eight (38.3%) patients had local recurrence. The 5-year crude overall survival was 71.4% and actuarial 5-year overall survival was 58% with confidence interval (CI, 25 and 81, respectively). The 5-year crude disease-free survival rate was 59% and the 5-year actuarial disease-free survival rate was 62% (CI, 28 and 83, respectively).
Multidisciplinary therapy of ENB should be considered, especially for Kadish C and high-grade lesions. Craniofacial resection (CFR), Intensity modulated radiation therapy (IMRT), and chemotherapy should be investigated in a multi-institution trial of ENB.
嗅神经母细胞瘤（ENB）是一种罕见的嗅上皮肿瘤。本研究的目的是评估包括手术、调强适形放疗（IMRT）和化疗在内的治疗方式以及患者的预后情况。
对总共21例患者进行回顾性分析。治疗方法包括颅面切除术（CFR）、放疗、化疗或这些方法的联合应用。
中位随访期为47个月。90.4%的病例接受了手术治疗；15例（72.7%）患者接受了辅助放疗。7例（33.3%）患者接受了手术、放疗和化疗。8例（38.3%）患者出现局部复发。5年粗总生存率为71.4%，5年精算总生存率为58%，置信区间分别为25和81。5年粗无病生存率为59%，5年精算无病生存率为62%（置信区间分别为28和83）。
应考虑对ENB进行多学科治疗，尤其是对于卡迪什C期和高级别病变。应在ENB的多机构试验中研究颅面切除术（CFR）、调强适形放疗（IMRT）和化疗。
