Extensive central nervous system involvement in optic pathway gliomas in neurofibromatosis type 1.

Abstract:

:Optic pathway gliomas (OPG) in neurofibromatosis type 1 (NF1) usually remain localized to the anterior visual pathway. However, a small number can demonstrate widespread dissemination. We describe three children with NF1 OPGs and extensive central nervous system involvement. In one case, a postmortem examination revealed tumor cells extending continuously from the optic nerves to the conus medullaris. This is the most widespread NF1 OPG reported in the literature. We suggest that rapid visual deterioration in the absence of radiographic changes or increased intracranial pressure can be caused by increasing tumor infiltration within the central nervous system.

journal_name

Pediatr Blood Cancer

journal_title

Pediatric blood & cancer

authors

Shaikh F,Johnston D,Michaud J,Hurteau J,Vassilyadi M,Keene D

doi

10.1002/pbc.23143

subject

Has Abstract

pub_date

2011-10-01 00:00:00

pages

688-6690

issue

4

eissn

1545-5009

issn

1545-5017

journal_volume

57

pub_type

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