Pineal tumors

Epidemiology
3-5% of CNS tumors in US
More common in Asia
Age 10-12 years
More common in males
Pineal region associated with non-germinomatous germ cell tumors
Suprasellar region associated with germinoma

Pathology
May be established by CSF or serum tumor markers
Non-germinomas: AFP => Yolk sac or mixed tumors; B-hCG => choriocarcinoma
Germinomas: better behaved; AFP elevation in CSF or serum rules out germinoma but patients can have slightly elevated B-hCG

Clinical
Pineal tumors associated with hydrocephalus, Parinaud's syndrome (upward gaze palsy, loss of accomodation, eyelid retraction; implies dorsal midbrain compression)
Suprasellar tumors associated with bitemporal hemianopsia, endocrine abnormalities (growth failure, DI, precocious puberty); hydrocephalus associated with very large tumors

Treatment, NG-GCT
Worse outcomes (except for mature teratoma which can be treated with resection and observation)
Chemo
CSI 36Gy + tumor boost to 54Gy

Treatment, G-GCT
Neither chemo nor RT alone have satisfactory outcomes
If chemo is used, CSI can be eliminated
In patients receiving chemo, whole ventricles treated to 25Gy with tumor boost to 45Gy
If no chemo given, CSI recommended (21Gy) with boost to gross tumor of 45Gy

Pineoblastoma
Subcategory of supratentorial PNET
Treated like high risk medulloblastoma