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Revista Romana de PEDIATRIE | Volumul LXIII, Nr. 1, An 2014
ISSN 1454-0398  |  e-ISSN 2069-6175
ISSN-L 1454-0398
DOI: 10.37897/RJP

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THE OPTIC PATHWAYS GLIOMAS IN CHILDREN WITH NEUROFIBROMATOSIS 1

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ABSTRACT

Optic pathways gliomas have the maximal clinical expression in childhood around the age of 5, being the second tumor in neurofibromatosis type 1 in frequency. Considering their location,it describes three types of optic pathways gliomas: type I - retrobulbar gliomas, type II - optic tracts gliomas and type III - chiasmatic gliomas. Neuroimaging exams are essential in diagnosis and selection surgical patients. Management of these tumors is often difficult even they exhibit histological benign features. Patients harboring optic nerve gliomas with symptomatic and documented neuroimaging progression have indication of tumor resection. Optic nerve gliomas associated with neurofibromatosis type 1 have generally a good prognosis. Unfavorable prognostic factors are represented by the early clinical onset under 6 years of age and chiasmatic and retrochiasmatic location.

Keywords: optic pathways gliomas, neurofibromatosis type 1, children

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