Difficulties in the classification and prognosis of the esthesioneuroblastoma shown in seven treated cases.

The esthesioneuroblastoma is a rare tumour of neuroectodermal origin, which arises usually in the area of the olfactory epithelium and invades the par anasal sinuses, the orbit and the brain. The low incidence of this disease makes a development of standardised clinical and histological classificatio n 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 a n esthesioneuroblastoma were treated in our department. This relatively lar ge number of patients allows a retrospective evaluation of the different al ready 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 resectio n and radiation (4 cases) or resection alone (1 case). No patient underwent a chemotherapy. Two cases with lethal outcome showed an extremely aggressi ve tumourprogression. In such cases of extensive disease an additional chem otherapy has always to be taken into account. Our experiences and the analy sis of the literature gives some indications that middle-aged patients have a worse prognosis than young or old patients.