Olfactory esthesioneuroma: a report of 40 cases.

Olfactory esthesioneuroma is a rare malignant tumor arising in the olfactory epithelium. Forty cases observed at the Institut Gustave-Roussy from 1956 to 1987 are reported. This tumor usually grows slowly and is usually local, but it is important to be aware of the possibility of lymph node involvement (17%) and, particularly, of rapid development of distant metastases (25%), usually within 6 months. CT scan, and more recently, NMR have proved to be of value in choosing the surgical approach. In view of the usual point of departure, a combined neurosurgical and transfacial approach seems to be a satisfactory approach for obtaining oncological control of the lesion. The role of chemotherapy is discussed. The main prognostic factors seem to be the size of the lesion, the intracranial extension, and the lymph node involvement.