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BACKGROUND Optic pathway gliomas (OPG) are typically astrocytic neoplasms that represent 3-5% of all pediatric brain tumors. The prognosis of these tumors has often been poor. Total surgical resection of OPG is not amenable, thus the role of surgery remains limited. Chemotherapy can be considered as the main modality of treatment; it is also able to postpone the use of radiotherapy which may harm the brain growth in children. METHODS We conducted the PRISMA-guided systematic literature search from 6 electronic databases to identify studies reporting outcomes of chemotherapy in OPG patients. The inclusion criteria are literature reporting the survival outcomes of chemotherapy in OPG patients with minimum subjects of 10 patients and aged below 22 years old. RESULTS A total of 48 out of 557 studies were assessed for its full paper eligibility. Thus, we found 10 studies comprising of 451 patients that met the inclusion criteria for analysis. From 8 different regimens of chemotherapy that were reported, the most commonly used regimens are Carboplastin ± Vincristine. The 3-year, 5-year, and 10-year progression-free survival (PFS) rates were ranged from 23-70%, 34-73%, and 47.1% respectively. The 3-year, 5-year, and 10-year overall survival (OS) rate has a range of 91-95%, 70.1-97%, and 61.3%-62.3% respectively. In detail, we found that 1 study with Cisplatin with Etoposide regimens reported 0% of the 5-year PFS rate, therefore it was excluded from the analysis. There are 2 studies that reported a high rate of 5-year radiotherapy free survival with chemotherapy, ranging from 61-82%. CONCLUSION This systematic review showed that chemotherapy in patients with OPG leads to an unsatisfactory 5-year PFS rate (34-73%), however it is able to provide a high 5-year OS (91-95%). In the absence of good quality control for these studies, future prospective clinical trials with adjusted confounding factors are urgently needed.

NCOG-39. PROGRESSION-FREE AND SURVIVAL RATE OF CHEMOTHERAPY IN PEDIATRICS WITH OPTIC PATHWAY GLIOMAS: A SYSTEMATIC REVIEW