Long-term oncologic outcomes in esthesioneuroblastoma: An institutional experience of 143 patients.

Abstract

Esthesioneuroblastoma (ENB) is a rare malignant neoplasm arising from the olfactory epithelium of the cribriform plate. The goal of this study was to update our oncologic outcomes for this disease and explore prognostic factors associated with survival. We performed a retrospective analysis of patients with ENB treated at a single tertiary care institution from January 1, 1960, to January 1, 2020. Univariate and multivariate analysis was performed. Overall survival (OS), progression-free survival (PFS), and distant metastasis-free survival (DMFS) were reported. Among 143 included patients, the 5-year OS was 82.3% and the 5-year PFS was 51.6%; 5-year OS and PFS have improved in the modern era (2005-present). Delayed regional nodal metastasis was the most common site of recurrence in 22% of patients (median, 57 months). On univariate analysis, modified Kadish staging (mKadish) had a negative effect on OS, PFS, and DMFS (p<0.05). Higher Hyams grade had a negative effect on PFS and DMFS (p<0.05). Positive margin status had a negative effect on PFS (p<0.05). Orbital invasion demonstrated worsening OS (hazard ratio, 3.1; p<0.05). On multivariable analysis, high Hyams grade (3 or 4), high mKadish stage (C+D), and increasing age were independent negative prognostic factors for OS (p<0.05). High Hyams grade (3+4), high mKadish stage (C+D), age, and positive margin status were independent negative prognostic factors for PFS (p<0.05). High Hyams grade (3+4) was an independent negative prognostic factor for DMFS (p<0.05). Patients with low Hyams grade and mKadish stage have favorable 5-year OS, PFS, and DMFS.