Perineural arachnoidal gliomatosis: case report / Gliomatose aracnoidal perineural: relato de caso
Arq. bras. oftalmol
;
71(4): 595-598, jul.-ago. 2008. ilus
Article
in English
| LILACS
| ID: lil-491898
ABSTRACT
Gliomas are the most common infiltrative neoplasms of the optic nerve and can present as two distinct growth patterns intraneural glial proliferation and perineural arachnoidal gliomatosis (PAG). It has been suggested that perineural arachnoidal gliomatosis is seen almost exclusively in the setting of neurofibromatosis type 1 (NF1). We describe a child with perineural arachnoidal gliomatosis occurring without neurofibromatosis type 1, supported by both radiographic and histological findings. A 4-year-old female without neurofibromatosis type 1 presented with rapidly progressive right-sided proptosis. Magnetic resonance imaging (MRI) revealed an enhancing fusiform intraconal lesion, which was hypointense on T1 and hyperintense on T2-weighted images characteristic of perineural arachnoidal gliomatosis, the optic nerve was visualized coursing the tumor. Histopathologic study was consistent with perineural arachnoidal gliomatosis. Perineural arachnoidal gliomatosis can develop independent of neurofibromatosis type 1, as demonstrated by this case.
RESUMO
Gliomas são as neoplasias infiltrativas mais freqüentes do nervo óptico e podem se apresentar através de dois padrões distintos de crescimento proliferação glial intraneural e gliomatose aracnoidal perineural. Existem evidências de que a gliomatose aracnoidal perineural é vista quase exclusivamente em pacientes com neurofibromatose tipo 1. Descrevemos um caso de gliomatose aracnoidal perineural ocorrendo em criança sem neurofibromatose tipo 1, comprovado tanto por achados radiológicos quanto histológicos. Uma criança de quatro anos de idade, do sexo feminino, sem evidências de neurofibromatose tipo 1, apresentou quadro de proptose à direita rapidamente progressiva. Ressonância magnética revelou lesão intraconal fusiforme hipointensa em T1 e hiperintensa em T2 - característico de gliomatose aracnoidal perineural, o nervo óptico pôde ser observado atravessando o tumor. O estudo histológico foi consistente com gliomatose aracnoidal perineural. Gliomatose aracnoidal perineural pode se desenvolver independente da presença de neurofibromatose tipo 1, como demonstrado por esse caso.
Full text:
Available
Index:
LILACS (Americas)
Main subject:
Arachnoid
/
Neurofibromatosis 1
/
Optic Nerve Glioma
Limits:
Child, preschool
/
Female
/
Humans
Language:
English
Journal:
Arq. bras. oftalmol
Journal subject:
Ophthalmology
Year:
2008
Type:
Article
Affiliation country:
Brazil
/
United States
Institution/Affiliation country:
Santa Casa de Misericórdia de São Paulo/BR
/
University of California San Francisco/US
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