Esthesioneuroblastoma: a Danish demographic study of 40 patients registered between 1978 and 2000.

Objective: A retrospective review of all diagnosed cases of esthesioneuroblastoma registered in Denmark between 1978 and 2000 was carried out in order to obtain epidemiological data and optimize national treatment guidelines.

Methods: Forty cases were verified histologically and included in the analysis Epidemiological and histopathological data were evaluated in relation to the clinical outcome.

Results: The 40 cases represent an incidence rate of 0.4 cases/million inhabitants per year. Eight (20%) patients were classified as Kadish stage A, 13 (32.5%) as stage B and 19 (47.5%) as stage C. The histopathological findings were classified according to the grading system of Hyams The median follow-up time was 2.3 years (range 0.3-11.1 years). The 5-year crude survival rate was 61%, with a median survival of 3.1 years (range 0.3-19.2 years). The 5-year disease-free survival rate was 50%, with a median survival of 1.7 years (range 0-19.2 years). Only 3 (7%) patients had positive cervical lymph nodes at presentation. A nationwide consensus regarding treatment was seen in patients classified as Kadish stages A and B. The longest duration before the first recurrence of esthesioneuroblastoma was 5(1/2) years.

Conclusions: The following therapeutic guidelines are suggested: Kadish stage A patients, surgical tumour resection and radiotherapy; Kadish stage B, surgical tumour resection and radiotherapy; Kadish stage C, surgical tumour resection via a craniofacial resection and radiotherapy combined with chemotherapy. Long-term follow-up of esthesioneuroblastoma patients is mandatory.