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Optic Pathway Gliomas in Adults

Shofty, Ben MD*,‡; Constantini, Shlomi MD, MSc*,‡; Bokstein, Felix MD§; Ram, Zvi MD*; Ben-Sira, Liat MD; Freedman, Sigal MSc*; Vainer, Gilad MD, PhD; Kesler, Anat MD#

doi: 10.1227/NEU.0000000000000257
Research-Human-Clinical Studies

BACKGROUND: 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.

OBJECTIVE: To characterize the clinical course of adult patients with OPG.

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

RESULTS: 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.

CONCLUSION: 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.

ABBREVIATIONS: NF1, neurofibromatosis 1

OPG, optic pathway gliomas

*Division of Neurosurgery,

Gilbert Israeli Neurofibromatosis Center,

§Neuro-Oncology Service,

Pediatric Radiology Unit,

Pathology, and

#Neuro-Ophthalmology Unit, Tel-Aviv Medical Center, and Sackler Faculty of Medicine, Tel-Aviv University, Tel Aviv, Israel

Correspondence: Shlomi Constantini, MD, MSc, Department of Pediatric Neurosurgery, Dana Children’s Hospital, Tel-Aviv Medical Center, 6 Weizman St, Tel Aviv, 64239 Israel. E-mail: sconsts@netvision.net.il

Received June 28, 2013

Accepted November 14, 2013

Copyright © by the Congress of Neurological Surgeons