Costa Susana P F, Rocha Eduardo, Rodrigues Jéssica, Soares André, Moreira Diana, Vieira Cláudia
Department of Radiotherapy, IPO Porto, Porto, Portugal.
Cancer Epidemiology Group, IPO Porto Research Center, IPO Porto, Porto, Portugal.
Rep Pract Oncol Radiother. 2024 Jun 6;29(2):197-203. doi: 10.5603/rpor.99705. eCollection 2024.
Esthesioneuroblastoma (ENB) is an uncommon malignant sinonasal tumor. There are few data regarding ENB management, namely its treatment. We review our institute's experience in the treatment of ENB and evaluate survival outcomes.
Retrospective study of patients with ENB treated between 1984-2022. A total of 20 patients were identified, 13 men and 7 women, aged between 20 and 76 years.
Eleven patients were stage C of the modified Kadish staging system at initial presentation, 7 stage B, 1 stage A and 1 stage D. Seventeen patients underwent surgery alone or combined with adjuvant treatment (radiotherapy or chemoradiotherapy). The majority of the patients (71.4%) treated with surgery alone were stage B, whereas most of the patients (63.6%) that underwent surgery combined with adjuvant treatment were stage C. Five of the 7 patients treated with surgery alone had a locoregional recurrence. Two of the 10 patients treated with surgery followed by adjuvant treatment had relapsed, locoregionally and at a distance, respectively. One patient was treated with chemotherapy and 2 patients were treated with chemoradiotherapy and neoadjuvant chemotherapy followed by chemoradiotherapy, respectively. The recurrence and persistence rates were 35% and 15%, respectively. The median time from the end of the first treatment to recurrence was 20.9 months. Two- and 5-year overall survival rates were 83.9% and 77.9%; while progression-free survival rates were 76.7% and 61.0%, respectively.
Sixty percent of patients were treated with a multimodal approach, which appeared to be a favorable strategy for the majority of patients.
嗅神经母细胞瘤（ENB）是一种罕见的鼻窦恶性肿瘤。关于ENB的治疗，即其管理的数据很少。我们回顾了我院治疗ENB的经验并评估生存结果。
对1984年至2022年间治疗的ENB患者进行回顾性研究。共确定了20例患者，13例男性和7例女性，年龄在20至76岁之间。
11例患者在初次就诊时为改良Kadish分期系统的C期，7例为B期，1例为A期，1例为D期。17例患者接受了单独手术或联合辅助治疗（放疗或放化疗）。单独接受手术治疗的患者大多数（71.4%）为B期，而接受手术联合辅助治疗的大多数患者（63.6%）为C期。单独接受手术治疗的7例患者中有5例出现局部区域复发。接受手术加辅助治疗的10例患者中有2例分别出现了局部区域复发和远处复发。1例患者接受了化疗，2例患者分别接受了放化疗以及新辅助化疗后再进行放化疗。复发率和持续率分别为35%和15%。从首次治疗结束到复发的中位时间为20.9个月。2年和5年总生存率分别为83.9%和77.9%；无进展生存率分别为76.7%和61.0%。
60%的患者接受了多模式治疗方法，这似乎对大多数患者是一种有利的策略。
