Long-Term Outcome of Esthesioneuroblastoma: Hyams Grade Predicts Patient Survival

Object: Esthesioneuroblastoma (ENB) is a rare malignant neuroendocrine tumor originating from the olfactory neuroepithelium in the cribriform plate. Modified Kadish stage and nodal disease at presentation have proven to predict outcome; however, controversy still exists regarding the role of pathologic grading (Hyams) in prognostication. This study was undertaken to describe our experience with ENB and assess the role of pathologic grading in patient outcome.

Methods: This was a retrospective single institutional experience. The study included 109 patients with pathologically proven ENB treated within our institution from 1960 to 2009. Hyams grade was confirmed on all cases available (n = 87) by pathology interpretation blinded to outcomes. Multivariate analysis was performed utilizing Cox regression analysis models built utilizing age, gender, modified Kadish stage, and Hyams grade.

Results: Mean age was 49 ± 16 (median 50) years at presentation. Forty-four percent in this series. Mean overall survival was 7.2 ± 0.7 years and median survival was 5.1 years. Mean progression-free survival was 4.8 ± 0.7 years. All causes of mortality were significantly influenced by Hyams grading in univariate (P = 0.045) and multivariate (p = 0.019) analysis. This was in addition to proven prognostic factors, Kadish staging, lymph node metastasis, and age. Median survival was 9.8 years compared with 6.9 years with low versus high Hyams grade. Median follow-up was 5.1 years.

Conclusion: ENB has a variable outcome that is primarily prognosticated by the extent of involvement at presentation (Kadish stage and lymph nodes metastasis). However, it appears that higher Hyams grade pathology at diagnosis influences patient survival.