Premium
Extensive central nervous system involvement in optic pathway gliomas in neurofibromatosis type 1
Author(s) -
Shaikh Furqan,
Johnston Donna,
Michaud Jean,
Hurteau Julie,
Vassilyadi Michael,
Keene Daniel
Publication year - 2011
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.23143
Subject(s) - medicine , neurofibromatosis , central nervous system , optic nerve , neurofibromatoses , pathology , neurofibromatosis type 2 , conus medullaris , neurofibromatosis type i , anatomy , magnetic resonance imaging , radiology
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. Pediatr Blood Cancer 2011; 57: 688–690. © 2011 Wiley‐Liss, Inc.