Diagnosis:
Suggested by MRI and confirmed by pathology (brain biopsy/resection)
Clinical features:
Bitemporal hemianopia (chiasm lesion), homonymous hemianopia (optic radiation, pregeniculate)
Findings on Investigations:
MRI:
- Clivus or sacrum
- Optic chiasm compression
Pathology:
Histology:
- Lobulated, fibrovascular septa. Epitheloid cells in chords/rows in mucoid stroma. Physaliphorus Cells (bubble cells)= central nuclei, bubble like vacuolated cytoplasm. Focal cartilage may occur.
Immunohistochemistry: positive for= Vimentin, EMA, cytokeratins, S100