A 6-year-old child with neurofibromatosis type 1 presents with progressive proptosis and visual loss in the right eye. MRI shows a fusiform enlargement of the optic nerve with a 'kinking' appearance. The most likely histopathology is:
- A Meningioma with psammoma bodies
- B Glioblastoma with necrosis and endothelial proliferation
- C Pilocytic astrocytoma with Rosenthal fibres ✓
- D Schwannoma with Antoni A and B areas
Correct answer: C. Pilocytic astrocytoma with Rosenthal fibres
Explanation
Optic nerve glioma in children, especially with NF1, is typically a pilocytic astrocytoma (WHO grade 1) showing Rosenthal fibres and eosinophilic granular bodies. Meningioma occurs more in middle-aged women. Glioblastoma is rare in the optic nerve. Schwannoma does not occur in the optic nerve as it lacks Schwann cells. The 'kinking' or tortuous appearance on MRI is characteristic of optic nerve glioma.
Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.