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.