Perineural arachnoidal gliomatosis: case report

Arquivos Brasileiros de Oftalmologia

Luciano Sousa Pereira²  Timothy James Mcculley¹ 

[1] ,University of California San Francisco Department of Ophthalmology San Francisco CA

关键词: Optic nerve glioma;    Neurofibromatosis;    Magnetic resonance imaging;    Human;    Female;    Child;    Case reports;    Glioma de nervo óptico;    Neurofibromatose;    Ressonância magnética;    Humano;    Feminino;    Criança;    Relatos de casos;   

DOI  :  10.1590/S0004-27492008000400026

来源: SciELO

【 摘 要 】

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.

【 授权许可】

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