Pineoblastoma
Also called: pineal gland tumor, pinealoblastoma
What is pineoblastoma?
The pineal gland is a tiny organ located deep inside the brain that secretes (gives off) a hormone called melatonin, which controls sleep. One type of tumor that starts in this gland is called a pineoblastoma.
Pineoblastoma is more aggressive than other types of pineal gland tumors. Its fast growth usually causes cerebrospinal fluid (CSF) to build up in the brain. This condition is called hydrocephalus. While pineoblastoma may spread through the CSF in 10% to 20% of cases, most of the time the tumors do not spread to other parts of the body.
The cause of pineoblastoma is not known, although genetic abnormalities are suspected.
How common is pineoblastoma?
Pineal gland tumors as a group are rare, accounting for less than 1% of all primary brain tumors. Pineoblastomas represent just under half of all pineal gland tumors.
Pineoblastoma usually occurs in children and young people between the ages of 20 and 40 years. It is equally common in males and females.
What are the signs and symptoms of pineoblastoma?
Because this type of tumor blocks the flow of CSF, many of its symptoms are related to CSF buildup. These symptoms include the following:
- Nausea and vomiting
- Headache
- Double vision
- Eye movement problems, such as trouble looking up
How is pineoblastoma treated?
Treatment for pineoblastoma focuses on removing the tumor or reducing its size and managing symptoms. The treatment team may consist of a primary care doctor, a cancer specialist (oncologist), a surgeon, a radiation therapist, and others who work together to decide the best treatment plan.
A treatment plan for pineoblastoma will likely consist of one or more of these options:
- Radiation—uses high-energy light sources (such as X-rays or electron beams) to destroy cancer cells or to slow their growth. Most pineoblastoma tumors are treated with radiation. It may be used on the entire brain and spinal cord.
- Surgery—may be used to remove part or all of the tumor. This not only removes the cancerous tissue from the brain, but also helps to determine what type of tumor it is. This is a complex surgery that requires expertise.
Surgery is also sometimes used to place a tube (shunt). The shunt helps drain the CSF buildup and relieve pressure in the brain.
- Chemotherapy (“chemo”)—uses cancer-killing drugs that are often combined with radiation or surgery to destroy any remaining tumor cells. Unlike radiation, which targets the area of the body containing the tumor, chemo medicines work throughout the body, killing cancer cells all over the body.
At the same time, chemo also kills normal healthy cells, which can lead to side effects. These include nausea, loss of appetite, hair loss, infection, fatigue, and more. Because of this, treatments will often be spaced apart to give the body time to recover between doses.
What are the survival rates for pineoblastoma?
The overall 5-year survival rate for children with pineoblastoma is about 60% to 65%.
