Pineoblastoma

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%.

Why choose St. Jude for your child’s pineoblastoma treatment?

• St. Jude has one of the largest pediatric brain tumor programs in the country.
• The St. Jude staff is dedicated to treating children who have serious illnesses. Here, you will find a hospital and staff that has only one focus—your child.
• St. Jude is committed to not only finding cures, but also helping children hold on to their quality of life. Children are not just small people. Their bodies and systems are unique, along with the disorders that affect them. For the best care, they need a specialized team. That is what you get from the St. Jude Brain Tumor Program.
• By working closely with the pediatric neurosurgeons at Le Bonheur Children’s Hospital in Memphis, the St. Jude Brain Tumor Program offers an expert team of doctors from many medical fields. The team includes: neuro-oncologists, who study and treat cancer in the brain and nervous system; neurosurgeons, who operate on the brain, skull, scalp and spinal cord; and radiation oncologists, who treat cancer with radiation. All of these doctors have devoted their careers to finding cures and providing treatment for children with brain tumors. This diverse knowledge is vital to the complete care of children with brain tumors.
• Our brain tumor doctors also work closely with support staff in nursing, rehabilitation services, neuropathology, pharmacy, neuroradiology, neuropsychology, clinical nutrition, child life and social work, along with many others. Support staff members have been specially trained to care for children with pediatric brain tumors.
• The nurse-to-patient ratio at St. Jude is unmatched— averaging 1:3 in hematology and oncology, and 1:1 in the Intensive Care Unit.
• The St. Jude Brain Tumor Program has made great advances in treatments for brain tumor patients. Some of the world’s best neuroscientists, cancer biologists and doctors from the St. Jude Neurobiology and Brain Tumor Divisions combine their efforts to seek cures. In this interactive program, discoveries about the biology of both normal and harmful cell growth are rapidly tested in clinic studies. These studies include Phase I, II and III trials of the most common and deadly forms of childhood brain tumors.
• Being able to take a drug directly from the lab to your child is one of the major advantages of centers like St. Jude. Before treating a patient with any new drug, the St. Jude staff does many tests in the lab. The tests show if the drug is safe, if it works, and the best way to use it to help patients.
• Along with the treatment studies being constantly developed at St. Jude, the staff also treats patients on studies developed by the Children’s Oncology Group, the Pediatric Brain Tumor Consortium and the Pacific Pediatric Neuro-Oncology Consortium. Many St. Jude staff members play key roles in developing these studies, which are large tests carried out at centers across the United States.
• St. Jude practices patient family-centered care. Patient family-centered care is health care that focuses on the family as a child’s primary source of strength, support and well-being. At the heart of patient family-centered care is the belief that health care staff and the family are partners, working together to best meet the needs of the child. Excellence in health care happens when we work together and honor the expertise each of us brings to every health care encounter.
• St. Jude is the only National Cancer Institute-designated Comprehensive Cancer Center devoted solely to children.

Contact the Brain Tumor Team any time, 24 hours a day, 7 days a week.

Call our Brain Tumor Coordinator, Tabatha E. Doyle, RN: 

(901) 595-2544

Or email us at braintumors@stjude.org