Treatment of esthesioneuroblastomas

Abstract

Objective: To analyze the clinical features, treatment outcomes, pattern of failures, and course of the disease of a cohort of patients treated for esthesioneuroblastoma (ENB) with craniofacial resection (CFR) at a single institution during a 12-year period.

Material and methods: Retrospective analysis of 11 patients with ENB treated with CFR in a tertiary care academic medical center from 1998 to 2009.

Results: Median age at diagnosis was 51 years (range 41-67 years). The most common presenting symptom was nasal obstruction (91%). Four patients (36%) presented with Kadish stage B, six patients (55%) with Kadish stage C, and one patient (9%) with Kadish stage D. The initial treatment was craniofacial resection (CFR) alone for three patients (23%), CFR followed by postoperative radiation therapy (RT) in seven patients (64%), while one patient (9%) received both neoadjuvant and adjuvant RT in addition to surgery. The mean and median follow-up times were 66 and 58 months, respectively (range 23-158 months). Seven patients are currently alive with no evidence of disease (64%), while two patients are alive with disease (18%). Overall survival was 100% at one year postoperatively and 80% five years after the primary treatment. The progression free survival was calculated to 73% at one year and 64% at five years.

Conclusions: ENB is an uncommon diagnosis with an incidence of 0.037/100,000 persons/year in the catchment area of our institution. Treatment can be challenging, especially with advanced disease. CFR with RT offers good oncologic disease control with minimal morbidity.

Keywords: Complications; Craniotomy; Crâniotomie; Esthesioneuroblastoma; Esthésioneuroblastome; Morbidité chirurgicale; Outcome; Résultat; Surgical morbidity.