Optimal treatment and prognostic factors for esthesioneuroblastoma: retrospective analysis of 187 Chinese patients

BACKGROUND: The standard treatment for esthesioneuroblastoma, a rare malignant nasal vault neoplasm, is not established. METHODS: We retrospectively assessed the clinicopathological features, prognostic factors and treatment methods for 187 patients with esthesioneuroblastoma treated in China between 1981 and 2015. Overall survival (OS) and disease-free survival (DFS) were evaluated using the Kaplan-Meier method and log-rank tests. RESULTS: Twenty-three (12.3%), 48 (25.7%) and 113 (60.4%) patients had Kadish stage A, B and C esthesioneuroblastoma; 3 (1.6%) had unknown stage. Overall, 117 (62.6%) patients received surgery and combined radiotherapy with or without chemotherapy; 35 (18.7%) received radiotherapy with or without chemotherapy; 32 (17.1%) received surgery alone; and 3 (1.6%) received palliative treatment. Three-year OS and DFS for the entire cohort were 66.7% and 57.5%, respectively. Three-year OS for stage A, B and C were 91.3%, 91.2% and 49.5% (P < 0.0001). Three-year OS was 16.7% and 66.7% for patients with and without distant metastasis (P < 0.0001). Surgery and combined radiotherapy with or without chemotherapy led to better OS and DFS than other treatment modes (both P < 0.0001). Univariate and multivariate analysis showed distant metastasis (hazard ratio [HR] = 2.162, 95% confidence interval [CI] = 1.145, 4.082, P = 0.017) and not receiving a combined modality treatment (HR = 2.391, 95% CI = 1.356, 4.218, P = 0.003) were independent prognostic factors for poor OS and DFS. CONCLUSIONS: This study indicates surgery and combined radiotherapy may currently be the optimal treatment for esthesioneuroblastoma. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (doi:10.1186/s12885-017-3247-z) contains supplementary material, which is available to authorized users.