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Optic pathway gliomas in adults.

Ben Shofty¹, Shlomi Constantini, Felix Bokstein

¹*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.

|December 17, 2013

Summary

Optic pathway gliomas (OPGs) can remain active into adulthood, with childhood diagnoses often predicting continued activity. Adults diagnosed with low-grade OPGs may experience progression and visual decline.

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Area of Science:

Neuro-oncology
Ophthalmology
Pediatric Oncology

Background:

Optic pathway gliomas (OPGs) are typically considered benign pediatric tumors.
Adult OPG patients fall into two categories: those diagnosed in childhood and those diagnosed in adulthood.
Neurofibromatosis type 1 (NF1) is associated with OPGs.

Purpose of the Study:

To characterize the clinical course of adult patients with optic pathway gliomas.
To identify patterns of tumor activity and visual outcomes in adult OPG patients.
To differentiate OPG behavior based on age at diagnosis.

Main Methods:

Retrospective collection of clinical and imaging data from adult OPG patients.
Study period: 1990-2012.
Inclusion criteria: adult patients monitored for OPG.

Main Results:

Twenty-two adult patients were analyzed, with ages at diagnosis ranging from 6 months to 66 years.
Twelve patients were diagnosed in childhood, with some experiencing progression and visual impairment. Six of these remained stable into adulthood.
Ten patients diagnosed in adulthood showed progression and visual deterioration in a significant subset; two had high-grade gliomas.

Conclusions:

Optic pathway gliomas can exhibit activity in both childhood and adulthood.
Patients with active OPGs in childhood are likely to experience continued disease activity in adulthood.
A notable proportion of adult-onset low-grade OPGs may progress, leading to visual decline.