Optic Pathway Glioma

John P. Christodouleas and Timothy A. Chan

Background

Are optic pathway gliomas (OPGs) more commonly seen in children or adults?

75% of all OPGs occur in children <10 yo.

What is the median age at Dx?

OPGs have a bimodal distribution with peaks at 5 yrs and 52 yrs of age.

OPG represents what % of all CNS tumors in children?

OPG represents 5% of all CNS tumors in children.

Are OPGs typically low grade or high grade?

OPGs are typically low grade (grade 1).

Is there a gender predilection in OPG?

Yes. In children, females are more commonly affected than males. In adults, males are more commonly affected than females.

What genetic syndrome is associated with OPG?

OPG is strongly associated with NF-1. 10%−38% of OPG pts have NF-1, and 15%−40% of NF-1 pts have OPG. (NF-2 is associated with acoustic neuromas.)

What are the subtypes of OPGs?

OPGs are organized by location:

Which OPG subtype is more common in children vs. adults?

Ant OPG is more common in younger children (and in pts with NF-1). Chiasmatic and hypothalamic gliomas are more common in adults.

Which subtype has the worst prognosis?

Hypothalamic OPG has the worst prognosis (OS 50%–80%/LC 40%–60% vs. other types (OS 90%–100%/LC 60%–90%).

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Feb 12, 2017 | Posted by admin in ONCOLOGY | Comments Off