Optic nerve glioma with perineural arachnoid gliomatosis

Case contributed by Magdi Mahsoub
Diagnosis probable

Presentation

The patient presented with left sided exophthalmos.

Patient Data

Age: 20 years
Gender: Male
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CT show intra-conal focal lesion of fluid density encasing the left optic nerve which appears prominent and tortuous.

Two partially calcified mass lesions in the subcutaneous tissues of the scalp suggesting trichilemmal cysts.

CT brain otherwise normal.

This study is a stack
Axial
T1
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Axial T2
fat sat
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Axial
FLAIR
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Coronal T2
fat sat
Sagittal
T2
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Sagittal
T1
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Axial
DWI
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Axial
ADC
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Axial
T1 C+
This study is a stack
Axial T1
C+ fat sat
This study is a stack
Oblique T1
C+ fat sat
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Case Discussion

Gliomas of the optic nerve can present as two distinct growth patterns: intraneural glial proliferation and perineural arachnoidal gliomatosis (PAG) 1. In perineural gliomatosis, there's an extraneural growth of glioma into the surrounding subarachnoid space characteristic of NF1-associated optic nerve gliomas 2. In perineural gliomatosis, you can identify the optic nerve within the lesion in contrast to the classic intraneural glioma where the tumor replaces the optic nerve 3.

The enlarged left optic nerve and perineural subarachnoid cystic dilatation, in combination with an abnormally high FLAIR signal at the left cerebellar hemisphere (FASI), highly suggest neurofibromatosis type 1 (NF1) with optic nerve glioma and perineural arachnoidal gliomatosis (PAG).

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