Abstract
Object Esthesioneuroblastoma (ENB) is a rare malignant neuroendocrine tumor originating
from the olfactory neuroepithelium in the cribriform plate. Controversy still exists
regarding the role of pathologic grading (Hyams grade) 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-institution experience, including 109 patients with
ENB treated at our institution from 1962 to 2009. Multivariate analysis was performed
utilizing Cox regression analysis models utilizing age, gender, modified Kadish stage,
and Hyams grade.
Results Mean age was 49 ± 16 (median 50) years at presentation (range 12 to 90 years). Median
follow up was 5.1 years. All-cause mortality was significantly influenced by Hyams
grading in univariate (p = 0.04) and multivariate (p = 0.02) analysis, 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 (grade 1 to 2) versus high (grade 3 to 4) Hyams grade. Median overall survival
was 7.2 ± 0.7 years.
Conclusion ENB has a variable outcome, which is primarily prognosticated by the extent of involvement
at presentation (Kadish stage and lymph node metastasis) and higher Hyams pathologic
grade.
Keywords
esthesioneuroblastoma - olfactory neuroblastoma - outcome - Hyams grade - surgical
treatment
