DIAGNOSIS AND THERAPY OF OLFACTORY NEUROB LASTOMAS

Esthesioneuroblastomas (EN) exhibit problems in early diagnosis and th erapy due to their localization at the frontal skull base. Analysis of six cases with EN (four male, two female; average age, 34.8 years) sh owed atypical initial symptoms, beginning as nasal bleeding, hyposmia and frontal headache. CT scans demonstrated hypo- to isodense tumors a t the anterior skull base with extension to the sinuses and orbits. Fi ve patients were operated on by an extranasal approach; one patient re quired orbital exenteration with later reconstructive surgery of the o rbit by a microvascularly adapted forearm flap. One patient underwent a neurosurgical procedure first that was followed by chemotherapy and stereotactically guided radiation. One patient died 1 year after onset of therapy due to intracranial tumor. One patient developed lung meta stasis 5 years after treatment. Four patients remain in clinical remis sion and receive regular follow-ups. Our analysis shows the guarded pr ognosis of EN despite multi-modality therapy. This includes the proble ms of advanced disease with complications of surgery and radiation. Al l therapeutic procedures should be planned in collaboration with otola ryngologists, neurosurgeons and radiotherapists. New computer-aided an d stereotactically guided radiation procedures can be helpful, especia lly in patients with extensive disease.