Abstract
BACKGROUND: Esthesioneuroblastoma (ENB) is an uncommon neoplasm arising from the olfactory mucosa. The optimal treatment regimen for ENB remains unclear. This study aims to evaluate its clinical features, long-term outcomes and explore optimal treatment patterns. METHODS: Clinical data of consecutive 44 ENB patients were reviewed retrospectively. The correlation between clinical features and treatment approaches were analyzed, with several prognostic factors explored meanwhile. RESULTS: The age of onset of ENB showed a bimodal distribution, with peaks at 10~20 and 50~60 years. The median follow-up time was 84 months (range, 27~198 months).The 5-year overall and progression free survival rates were 42.7% and 39.1%, respectively, with 10-year rates of 28.9% and 21.7% respectively. Overall, 19 patients developed recurrent disease. Patients undergoing surgery combined with adjuvant radiotherapy had significantly higher 5-year overall survival (67.5% vs. 33.3%, P=0.043) and progress-free survival (60.0%vs. 18.7%, P=0.008) than those receiving other treatment approaches. No-Skin-involved ENB was associated with markedly better 5-year overall survival (45.5%vs.0 %, P=0.038) and progress-free survival (31.3% vs. 0 %, P=0.001) compared with skin-involved tumor. CONCLUSIONS: ENB is a rarely malignant tumor with high probability of locoregional recurrence and poor survival. Surgical resection followed by radiotherapy has been shown to achieve optimal local control and overall survival.