OPTICO-HYPOTHALAMIC GLIOMA - AN ANALYSIS OF 16 CASES

We reviewed our experience of 16 patients with histologically proven o ptico-hypothalamic gliomas. They ranged in age from 0.3 to 15 years at the time of diagnosis. Fifteen tumors were located in the optic chias m, optic nerve, optic tract and/or hypothalamus, while one tumor was c onfined to one optic nerve. All tumors were classified as low-grade as trocytomas, which were mainly composed of pilocytic astrocytes. No pat ient had associated neurofibromatosis. The initial treatment for tumor s included surgery in 12, radiotherapy in 7, and chemotherapy in 4 pat ients. After treatment, visual function improved in 3 out of 14, and e ndocrine function improved in 1 out of 4 evaluable patients. The 5-yea r actuarial survival rate was 84.0%, and that at 10 years 71.1 %. Our experience and the literature indicate that: (1) patients with disease limited to the optic nerve are adequately managed by resection alone; (2) chiasmal-hypothalamic gliomas behave variably, and progressive di sease may occur late in the course of the illness; (3) gliomas that ar ise in patients under 2 years of age and involve the optic chiasm may act aggressively despite their histological benignity; (4) the benefic ial effects of radiotherapy occur in about half of the patients; (5) a lthough chemotherapy may be an effective adjuvant treatment modality, it is not an alternative to radiation therapy at present. Both surgery and irradiation therefore offer the best treatment now available for patients with progressive disease.