[Classification and prognosis of esthesioneuroblastoma based on 7 treated cases].

BACKGROUND: The esthesioneuroblastoma is a rare tumour of neuroectodermal origin, which arises usually in the area of the olfactory epithelium and invades the paranasal sinuses, the orbit and the brain. The low incidence of this disease makes a development of standardised clinical and histological classification difficult. Up to now this tumour is considered to be slow progressive but strained by a high rate of local recurrences. Metastasis are usually seen late at an advanced stage.

PATIENTS: In the last 18 years 7 patients with an esthesioneuroblastoma were treated in our department. This relatively large number of patients allows a retrospective evaluation of the different already existing classifications concerning treatment and prognosis.

RESULTS AND CONCLUSIONS: In all cases of a disease limited on the paranasal sinuses the patients were successfully treated either by a combination of resection and radiation (4 cases) or resection alone (1 case). No patient underwent a chemotherapy. Two cases with lethal outcome showed an extremely aggressive tumour progression. In such cases of extensive disease an additional chemotherapy has always to be taken into account. Our experiences and the analysis of the literature gives some indications that middle-aged patients have a worse prognosis than young or old patients.