Optic pathway gliomas in adults

Optic pathway gliomas (OPGs) are considered relatively benign pediatric tumors. Adult patients with OPG can be divided into 2 groups: adult patients with tumors diagnosed in childhood and adult patients diagnosed during adulthood.

To characterize the clinical course of adult patients with OPG.

We retrospectively collected clinical and imaging data of all adult OPG patients monitored in our medical center between 1990 and 2012.

Twenty-two adult patients were included. Age at diagnosis varied widely (6 months-66 years), as did age at last follow-up (18-74 years). Ten patients were diagnosed at adulthood and 12 in childhood. Of the patients diagnosed at childhood, 6 had radiological progression during childhood, and 3 of those patients suffered visual impairment. From this group, 1 patient had further progression during adulthood accompanied by additional visual decline, and 2 patients had additional visual decline during adulthood despite no signs of progression. Of the 6 patients whose tumors were stable during childhood, all 6 remained stable during adulthood. Of 10 patients diagnosed at adulthood, 6 patients suffered visual deterioration; in 5 of them, a concomitant progression was noted. Two patients were diagnosed with high-grade gliomas.

OPGs may be active during childhood or adulthood. Those patients who experienced anatomic 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, visual decline, or both.

NF1, neurofibromatosis 1OPG, optic pathway gliomas.