Fact Sheet on Pineoblastoma

This week we explore pineoblastoma (also pinealoblastoma), a malignant tumour of the pineal gland, located in the brain.

A pineoblastoma is a supratentorial midline primitive neuroectodermal tumour and may occur in patients with hereditary uni- or bilateral retinoblastoma.

When retinoblastoma patients present with pineoblastoma this is characterised as ‘trilateral retinoblastoma’. Prognosis of patients with trilateral retinoblastoma is dismal: only a few patients have survived more than five years after diagnosis. All survivors were diagnosed with small tumours in a subclinical stage. Recent advances in (high-dose) chemotherapy treatment regimens and early detection have improved survival of patients with trilateral retinoblastoma to up to 50%. (Wikipedia).

Pineal tumours originate from normal cells in the pineal gland. The pineal gland is located in the centre of the brain and is involved in the secretion of specific hormones.

Tumour types occurring in the pineal region may or may not involve the pineal gland. Tumours that may occur in this region but are not necessarily pineal tumours include: germinoma, non-germinoma (eg, teratoma, endodermal sinus tumour, embryonal cell tumour, choriocarcinoma, and mixed tumours), meningioma, astrocytoma, ganglioglioma, and dermoid cysts.

There are three types of pineal tumours: Pineocytoma: Slow-growing, grade II tumour. Pineoblastoma: More aggressive, grade IV, malignant tumour. A grade III intermediate form has also been described. Mixed Pineal Tumour: Contains a combination of cell types. (American Brain Tumor Association).

Pineoblastoma is one of several different types of tumours that arise in the area of the pineal gland, requiring different therapies. The exact diagnosis is critical for choosing the correct therapy. Pineal tumours typically present with hydrocephalus (a build-up of fluid pressure within the brain). A team of experts is needed for optimum therapy. Pineal gland tumours as a group are rare, accounting for less than 1% of all primary brain tumours. Pineoblastomas represent just under half of all pineal gland tumours. Pineoblastoma usually occurs in children and young people between the ages of 20 and 40 years. It is equally common in males and females.

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