• Written By: Howard Pomeranz, MD, PhD
    Neuro-Ophthalmology/Orbit

    This study evaluated the effectiveness of standard oncological therapy for optic pathway gliomas (OPGs). The authors reviewed the medical records of OPG patients treated with chemotherapy at one medical center between 1993 and 2009. The results were disappointing, with nearly three-fourths of patients experiencing visual decline during the study period and no correlation between radiological tumor growth and visual deterioration. These findings suggest that more effective chemotherapy treatment regimens are necessary in order to better manage OPGs in children.

    The authors retrospectively correlated ophthalmological outcomes over a mean follow-up of four years with radiological findings in 19 patients with progressive OPGs who received chemotherapy. Indications for treatment among the study's subjects were radiological tumor progression (six patients), visual decline (six) or both (seven). Fifteen patients (78 percent) switched to second-line chemotherapy (seven due to allergies and eight due to treatment failure).

    Conservative management of OPGs in children is usually effective. Due to the indolent nature of this tumor, the most important treatment challenge is vision preservation. For progressive tumors, chemotherapy is preferred over radiation therapy because of the potential side effects of radiation in children.

    During the course of chemotherapy, 11 patients (57.8 percent) displayed radiological tumor progression, four (21.5 percent) demonstrated stability and four displayed tumor regression. During the follow-up period, 14 patients (73.6 percent) had overall visual deterioration, four (21 percent) had stable vision and one (5.2 percent) improved. Visual acuity deteriorated in 17 eyes (47.2 percent), was stable in 14 (38.8 percent) and improved in five (13.8 percent). Ten eyes (27.7 percent) became legally blind.

    The authors conclude that their current treatment paradigm for progressive OPGs should be reconsidered. They say that new, more effective treatments are needed in order to preserve vision in these patients.