Visual outcome following chemotherapy for progressive optic pathway gliomas

Background: Optic pathway gliomas (OPG) are relatively indolent tumors that may occur sporadically or in association with neurofibromatosis 1. Treatment is initiated only when a clear clinical or radiological deterioration is documented. Chemotherapy is the standard first line of treatment. Due to the indolent nature of this tumor, the most important challenge in OPG treatment is vision preservation. Methods: In this study we determined the visual outcome of 19 patients with progressive OPGs who received chemotherapy and correlated it with imaging. Results: Mean neuro-ophthalmological follow-up is 4 years and 3 months. Indications for treatment were radiological tumor progression (6 patients), visual decline (6 patients), or both (7 patients). Fifteen patients (78%) had to change to 2nd line chemotherapy (7 due to allergies and 8 due to treatment failure). During the course of chemotherapy, 11 patients (57.8%) displayed radiological tumor progression, 4 (21.5%) demonstrated stable tumor, and 4 (21.5%) displayed tumor regression. During the follow-up period, 14 (73.6%) had an overall visual deterioration, 4 (21%) had stable vision, and 1 patient (5.2%) improved. Visual acuity was examined in 38 eyes. Seventeen eyes (47.2%) deteriorated, fourteen (38.8%) were stable, and five (13.8%) improved. Ten eyes (27.7%) deteriorated to legal blindness. There was no correlation between radiological tumor growth and visual deterioration. Conclusions: The majority of our patients, who received chemotherapy for progressive OPG, experienced a decline in their visual function. New, more effective treatments are needed in order to preserve vision in this group.