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Perineural arachnoidal gliomatosis: case report
Luciano Sousa Pereira1, Timothy James McCulley
1Department of Ophthalmology, University of California San Francisco, San Francisco, CA, USA. lucianospereira@gmail.com
Arquivos Brasileiros De Oftalmologia
|September 18, 2008
Summary
Perineural arachnoidal gliomatosis (PAG) is typically associated with neurofibromatosis type 1 (NF1). This case demonstrates PAG occurring independently of NF1 in a pediatric patient, highlighting a rare presentation.
Area of Science:
- Ophthalmology
- Neurology
- Oncology
Background:
- Gliomas are common infiltrative optic nerve neoplasms.
- Perineural arachnoidal gliomatosis (PAG) is a rare optic nerve glioma subtype.
- PAG is strongly associated with neurofibromatosis type 1 (NF1).
Observation:
- A 4-year-old female presented with proptosis.
- MRI revealed a characteristic enhancing intraconal lesion.
- The optic nerve was involved by the tumor.
Findings:
- Histopathology confirmed PAG.
- The patient had no clinical or genetic evidence of NF1.
- This case represents PAG without NF1.
Implications:
- PAG can occur independently of NF1.
- This finding expands the differential diagnosis for optic nerve gliomas.
- Further research is needed to understand non-NF1 associated PAG.
