Diagnosis:
Suggested by MRI and confirmed by pathology (brain biopsy/resection)
Clinical features:
Parinaud syndrome
Hydrocephalus
Findings on Investigations:
MRI:
- Pineal mass
- Contrast enhancement
- CSF leptomeningeal metastasis can occur
CSF analysis:
- leptomeningeal metastasis can occur
Pathology:
Histology:
- Small round blue cell tumor, high nuclear cytoplasmic ratio. Homer-Wright (around neuritic processes) & Flexner-Wintersteiner Rosettes (around lumen). Necrosis & mitosis may occur.
Immunohistochemistry: positive for= synaptophysin, NSE, neurofilament, Class III beta tubulin, chromogranin, retinal S antigen. Rarely GFAP positive
Treatment:
Options:
- Surgery
- Radiation therapy
- Chemotherapy