Six patients with
neuroblastomas of the olfactory nerve (
esthesioneuroblastoma) are presented who were irradiated between 1983 and 1986 at the Medical Radiologic Institute of Tübingen. 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.