Clinical presentation and outcome of patients with optic pathway gliomas: A series of twenty patients

Abstract

Background: Optic pathway gliomas (OPGs) are rare neoplasms where the management strategy is still evolving. The purpose of this study is to present our experience of twenty cases of OPGs with an intent to add to the general fund of knowledge. Materials and Methods: Retrospective chart review of patients with OPGs surgically managed in a single tertiary centre over a period of 5 years. Variables analysed include Age at diagnosis, sex, clinical manifestations, neurofibromatosis (NF1) status, tumour location, treatment modality, symptomatic progression, and complications. Results: Of the 20 patients with OPG in the study with a mean age of 12.8 years, four patients had NF1 and 16 had sporadic OPG. Dimness of vision (80%) and headache (55%) were the most common presenting symptoms. Majority of the tumours were chiasmatic (40%) in location and vision was affected bilaterally. Subtotal resection could be achieved in 12 (60%) partial decompression in 4 (30%) and gross total resection in 2 (10%). Four patients required additional shunt surgery for hydrocephalus. Postoperative radiotherapy was administered to thirteen patients and only one patient received chemotherapy. At the end of a mean follow up period of 44 months tumour recurrence was noticed in 11/20 (55%) patients. Vision improved in none, remained stable in fifteen and deteriorated in five. Three patients (15%) succumbed to tumour regrowth in the follow up period. Conclusions: OPGs are uncommon tumours with unpredictable outcome. Visual deficits are often irreversible and progressive. Curative resection is difficult and adjuvant therapy has variable response. Overall survival rates are good but progression free and survival rates are suboptimal.

Keywords

• chiasma
• glioma
• neurofibromatosis
• optic nerve
• orbit