Optic Pathway Gliomas: Clinical Manifestation, Treatment, and Follow-Up

Objective: Optic pathway gliomas (OPG) are infrequent pediatric brain tumors that affect the optic nerve and the visual pathway in the brain. A wide spectrum of signs and symptoms, such as visual impairment, nystagmus, proptosis, and visual loss, may occur at different stages. Other manifestations, such as hydrocephalus, diancephalic syndrome, neurologic deficits, and growth and developmental delays, may be present as well. Surgical resection, chemotherapy, and radiotherapy are used as treatment strategies. The purpose of this study is to evaluate the clinical presentation of OPG patients and their final outcomes in response to treatment. Methods: Thirty-seven patients with an initial diagnosis of OPG were studied as part of a single-center retrospective cohort for their clinical presentation, treatment, and response to treatment over a 10-year period. Results: The mean age of the patients was 37.1 months, and there was a nearly equal sex distribution. The most prevalent manifestation was visual impairment (94.4%), and other symptoms included nystagmus (50%), growth and developmental delays (27.8%), neurological deficits (19.4%), diencephalic syndrome (13.5%), proptosis (11.1%), and hydrocephalus (29.7%). Tumor staging revealed that 10.8% of the cases were stage A, 54.1% were stage B, and 35.1% were stage C. In 30 evaluable patients, the response to treatment included complete response (CR; 23.3%), partial response (PR; 43.3%), stable disease (23.3%), progressive disease (3.3%), and death due to disease (6.7%). A significant association was detected between tumor staging and the response to treatment, with higher rates of CR and PR in stage B and a higher mortality rate in stage C (p = 0.005). Conclusion: OPG occur more commonly in the first decade of life, with visual disturbance as the most prevalent symptom. Tumor staging and the treatment strategy are proposed to affect the response to treatment, although the specific tumor behavior in each patient should be considered as well. (C) 2016 S. Karger AG, Basel