Neuroblastoma of the olfactory nerve. Radiotherapy experiences in 6 patients

: Six patients with neuroblastomas of the olfactory nerve (esthesioneuroblastoma) are presented who were irradiated between 1983 and 1986 at the Medical Radiologic Institute of Tubingen. Clinical manifestations, diagnostics, histology, therapy, and courses are compared and discussed with regard to a survey of literature. An attempt is made to find out the value of radiotherapy in the treatment of this rare disease. In stage A (tumor restricted to the nasal cavity, 1 patient), a local tumor control of up to now 28 months could be achieved by a treatment combination of surgery and radiotherapy. A treatment consisting of surgery or radiotherapy alone should even in this stage only be performed in connection with a close follow-up because of the increased local recurrence risk. Tumors of stage B (manifestation in the nasal cavity and the paranasal sinuses) did not occur in this group of patients. Five patients suffered from tumors of stage C (tumor extent beyond the paranasal sinuses). A good palliative effect was obtained temporarily by radiotherapy alone in three out of these patients showing large inoperable tumors and rapidly progressing clinical symptoms. A complete remission now lasting 16 months was achieved only in one patient by radical surgery with unilateral evisceration of orbit and homogeneous postirradiation. In case of stage C tumors it is recommended to perform, if possible, a radical tumor excision with evisceration of orbit in case of unilateral manifestation in the orbit and a postirradiation applying a radical, large volume technique. In order to reduce the risk of radiogenic cerebral necroses, it should be attempted to avoid dose maxima as they can occur when applying a combined ventro-dorsal and lateral irradiation technique.