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Updated: Aug 11, 2026
Surgical Treatment of an Endolymphatic Sac Tumor
Published on: May 26, 2023
Esthesioneuroblastoma: continued follow-up of a single institution's experience
Andrew H Loy1, James F Reibel, Paul W Read
1Department of Otolaryngology-Head and Neck Surgery, University of Virginia Health System, Charlottesville 22908-0713, USA.
Objectives:
To analyze outcomes and to provide follow-up for our increasing patient cohort with esthesioneuroblastoma.
Design:
Retrospective cohort analysis.
Setting:
Patients were examined from September 1, 1976, to May 30, 2004, in a tertiary care academic hospital.
Patients:
Fifty consecutive patients diagnosed as having esthesioneuroblastoma were treated with a standardized protocol during a 28-year period. Patients with tumors staged Kadish A or B received preoperative radiotherapy followed by craniofacial resection, while patients with Kadish stage C disease were treated with preoperative sequential chemotherapy and radiotherapy followed by a craniofacial resection. The mean follow-up is 93 months (range, 1-330 months).
Results:
The disease-free survival was 86.5% and 82.6% at 5 and 15 years, respectively. There were 17 patients (34%) who developed recurrent disease, most of which was locoregional (12 patients [71%]). There was a long interval to relapse (mean, 6 years), with the longest time to regional recurrence being 10 years. Distant relapses occurred sooner, with poorer outcomes. Of these 17 patients, 7 (41%) underwent successful salvage surgery, while 3 remain alive with disease.
Conclusions:
Excellent outcomes for esthesioneuroblastoma are achievable. Long-term follow-up is necessary because of the extended interval for recurrent disease; unlike most sinonasal malignancies, surgical salvage is possible.