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Optic pathway gliomas in neurofibromatosis-1: Controversies and recommendations

Optic pathway gliomas in neurofibromatosis-1: Controversies and recommendations

Authors :
Grant T. Liu
Robert Listernick
Rosalie E. Ferner
David H. Gutmann
Source :
Annals of Neurology. 61:189-198
Publication Year :
2007
Publisher :
Wiley, 2007.

Abstract

Optic pathway glioma (OPG), seen in 15% to 20% of individuals with neurofibromatosis type 1 (NF1), account for significant morbidity in young children with NF1. Overwhelmingly a tumor of children younger than 7 years, OPG may present in individuals with NF1 at any age. Although many OPG may remain indolent and never cause signs or symptoms, others lead to vision loss, proptosis, or precocious puberty. Because the natural history and treatment of NF1-associated OPG is different from that of sporadic OPG in individuals without NF1, a task force composed of basic scientists and clinical researchers was assembled in 1997 to propose a set of guidelines for the diagnosis and management of NF1-associated OPG. This new review highlights advances in our understanding of the pathophysiology and clinical behavior of these tumors made over the last 10 years. Controversies in both the diagnosis and management of these tumors are examined. Finally, specific evidence-based recommendations are proposed for clinicians caring for children with NF1.

Details

ISSN :
15318249 and 03645134
Volume :
61
Database :
OpenAIRE
Journal :
Annals of Neurology
Accession number :
edsair.doi...........821da71085291be58c963cbbdbad8dc0
Full Text :
https://doi.org/10.1002/ana.21107