Lg-001. optic pathway gliomas; from childhood to adulthood

BACKGROUND: Optic pathway gliomas (OPG) are considered relatively benign pediatric tumors. Adult OPG can be divided into two groups; adult patients with tumors diagnosed in childhood, and adult patients diagnosed during adulthood. In this study we have characterized the clinical course of patients belonging to these two groups. METHODS: We retrospectively collected clinical and imaging data for all adult OPG patients who have been monitored in our medical-center between 1990 and 2012. RESULTS: Twenty-two adult patients were included. Age distribution at diagnosis varied widely (6m–66y), as did age at last follow-up (18y-74y). Ten patients were diagnosed at adulthood, 12 in childhood. Of the patients that were diagnosed at childhood, 6 had radiological progression during childhood, and 3 of those suffered visual impairment. From this group of 6 patients, 1 had further radiological progression during adulthood accompanied by additional visual decline, 2 patients had additional visual decline during adulthood despite no signs of radiological progression. Of the 6 patients whose tumors were stable during childhood, all 6 remained stable during adulthood. Out of 10 patients diagnosed at adulthood, 6 patients suffered visual deterioration; in 5 of them a concomitant radiological progression was noted. Two patients were diagnosed with high-grade gliomas; both died of their disease. CONCLUSION: OPGs may be active during childhood or adulthood. Those patients that experienced anatomical activity during childhood are prone to continue experiencing active disease during adulthood. A significant percentage of patients diagnosed with low-grade OPG at adulthood may suffer progression, or visual decline, or both.