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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.

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