Esthesioneuroblastoma: The Princess Margaret Hospital experience

Background. Esthesioneuroblastoma is rare. The aim of the study was to review our experience and to evaluate the staging system and treatment that best correlates with the patient outcome. Methods. Thirty-nine patients were identified between 1972 and 2006. Results. At presentation 10% had cervical metastases. None had distant metastasis. Five were treated with surgery, 2 with chemotherapy, 1 with radiotherapy, and 30 with surgery and radiation. Local disease control was 82.6% at 5 years. Recurrence was seen in 33% with local and regional disease recurrence at 15% and 18%, respectively. The 5- and 10-year overall survival rates were 87.9% and 69.2%, respectively. Dulguerov classification correlated most closely to survival and recurrence. Conclusions. Dulguerov classification best correlates with the patient's outcome. A combined approach is the preferred treatment. It makes no difference whether radiotherapy is given pre or postsurgical resection. Recurrence can occur even 15 years after treatment. Therefore, long-term follow-up is essential.