Pineoblastomas are high grade (cancerous - grade 4), fast growing tumours of the pineal region of the brain. They develop from primitive cells and are commonly found near the base of the cerebral hemispheres and the area above the pituitary gland and close to the hypothalamus.
Pineoblastoma is one of a dozen different tumours that can present in and around the pineal area. It originates from embryonal cells which were left behind from the time the person was a developing fetus. Healthy embryonal cells go on to differentiate and play a part in the growth of body parts such as the pineal gland and retina. In the case of pineoblastomas these cells have divided incorrectly causing an abnormal growth or tumour.
Due to their position these tumours often cause hydrocephalus, a build-up of pressure within the skull. This happens because the tumour, usually located close to the third ventricle (see diagram below) blocking the cerebro-spinal fluid (CSF) surrounding the brain from draining away. As well as headaches and nausea due to the hydrocephalus, symptoms often also include abnormalities in eye movements.
Although pineoblastomas can occur at any age, they are predominantly diagnosed during the first two decades of life.
After a pineoblastoma is diagnosed through scans and/or a biopsy, your MDT will consider the best treatment option for you.
Surgery to reduce the size of the tumour or remove it completely is an essential part of brain tumour treatment in general. However, due to the position of pineoblastomas deep within the brain, surgery may not always be a viable option as there could be a risk of damaging critical areas of the brain. Even if doctors deem that surgery is possible for a pineoblastoma, in most occasions it does not result in complete resection. This is because pineoblastomas, grade four tumours, may have already invaded other, difficult to reach parts of the brain or spine.
Due to the swelling and pressure on the brain, you may have surgery to drain the buildup of fluid. Some neurosurgery centres offer a minimally invasive procedure called endoscopic third ventriculostomy which which will not require surgery. Steroids may also be prescribed to manage swelling associated with hydrocephalus.
Radiotherapy is the gold standard of treatment for pineoblastomas. Depending on factors such as whether cancerous cells have spread to other parts of the brain, your MDT will decide the type of radiotherapy you will have and at which dose. Pineoblastomas may also be treated with chemotherapy.
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