Optico-hypothalamic glioma: an analysis of 16 cases

We reviewed our experience of 16 patients with histologically proven optico-hypothalamic gliomas. They ranged in age from 0.3 to 15 years at the time of diagnosis. Fifteen tumors were located in the optic chiasm, optic nerve, optic tract and/or hypothalamus, while one tumor was confined to one optic nerve. All tumors were classified as low-grade astrocytomas, which were mainly composed of pilocytic astrocytes. No patient had associated neurofibromatosis. The initial treatment for tumors included surgery in 12, radiotherapy in 7, and chemotherapy in 4 patients. After treatment, visual function improved in 3 out of 14, and endocrine function improved in 1 out of 4 evaluable patients. The 5-year 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 disease may occur late in the course of the illness; (3) gliomas that arise in patients under 2 years of age and involve the optic chiasm may act aggressively despite their histological benignity; (4) the beneficial effects of radiotherapy occur in about half of the patients; (5) although 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.