The pineal gland is a small organ in the center of the brain that is responsible for controlling melatonin secretion.

[6] Due to the pineal gland's location at the center of the brain and the rapidly growing nature of this disease, obstruction of CNS fluid is a common symptom.

MicroRNA dysregulation has been found to be associated with many cases of pineoblastoma, specifically, mutations in DICER1 and DROSHA genes.

[11] Recent advances in (high-dose) chemotherapy treatment regimens and early detection have improved survival of patients with trilateral retinoblastoma to up to 50%.

[3] Due to the aggressive nature of the disease, tumor spread at the time of diagnosis is common.

[13] CT will show large, multilobulated masses with heterogenous contrast enhancement and peripheral calcification of the pineal gland.

PET-CT has also been used in diagnosis, and shows increased uptake of fludeoxyglucose with pineoblastomas compared to other pineal masses.

Diagnosis also requires CSF sampling via lumbar puncture to assess for cytology and tumor markers.

This includes synaptophysin, neurofilament protein, and CRX, a specific pineal or retinal marker, positive staining.

[13][7] Initial treatment for pineoblastoma often includes a shunting procedure to redirect accumulated cerebrospinal fluid secondary to obstructive hydrocephalus.

Surgery to remove the tumor is associated with better outcomes, however, this is not always possible due to the proximity of the pineal gland to neurovascular structures.

When pineoblastomas occur with retinoblastomas, the prognosis is typically worse, and these patients require more aggressive treatment.