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Primary Orbital Cystic Pilocytic Astrocytoma
Hardeep Singh Mudhar1, Murtuza Nuruddin2, Soma Roy2
1National Specialist Ophthalmic Pathology Service (NSOPS), Department of Histopathology, Royal Hallamshire Hospital, Sheffield, UK.
Purpose:
To describe the clinical, radiological and histopathological features of a highly unusual primary orbital glioma.
Procedures:
A 6-year-old female presented with right-sided proptosis that revealed a well-defined intraconal mass on CT imaging with intact orbital bony walls. During the removal of the lesion, it dissected away freely from the muscles and was separated from the optic nerve and globe. There was no communication with the brain.
Results:
Histology showed histological features highly favouring a pilocytic astrocytoma enveloped by meninges. No non-neoplastic brain tissue was identified.
Conclusion:
This is the first documentation of a primary orbital pilocytic astrocytoma not anatomically connected with the brain. We speculate that the tumour had arisen from developmental brain heterotopia.
