Pineoblastoma

Pineoblastoma is a type of cancer that starts in the brain's pineal gland. The pineal gland is located in the center of the brain. The gland makes a hormone called melatonin. Melatonin plays a role in the body's natural sleep-wake cycle.

Pineoblastoma begins as a growth of cells in the pineal gland. The cells grow quickly and can invade and destroy healthy body tissue.

Pineoblastoma can occur at any age. But it happens most often in young children. Pineoblastoma may cause headaches, sleepiness and changes in the way the eyes move.

Pineoblastoma can be very difficult to treat. It can spread within the brain and into the fluid around the brain. This fluid is called cerebrospinal fluid. Pineoblastoma almost never spreads beyond the central nervous system. Treatment usually involves surgery to remove as much of the cancer as possible. Additional treatments also may be recommended.

Pineoblastoma signs and symptoms include:

• Behavior changes.
• Headaches.
• Lack of energy.
• Nausea.
• Problems with memory and thinking.

Pineoblastoma sometimes causes eye symptoms. This can happen when the cancer causes pressure on parts of the brain that help with vision. The eye symptoms that pineoblastoma causes are sometimes called Parinaud syndrome. Eye symptoms caused by pineoblastoma include:

• Blurred vision.
• Double vision.
• Problems focusing on objects that are up close.
• Problems looking upward.

When to see a doctor

Make an appointment with a doctor or other healthcare professional if you or your child have symptoms that worry you.

The cause of pineoblastoma often isn't known. This cancer starts as a growth of cells in the pineal gland. The pineal gland is located in the center of the brain.

Pineoblastoma happens when cells in the pineal gland develop changes in their DNA. A cell's DNA holds the instructions that tell the cell what to do. In healthy cells, the DNA gives instructions to grow and multiply at a set rate. The instructions tell the cells to die at a set time. In cancer cells, the DNA changes give different instructions. The changes tell the cancer cells to grow and multiply quickly. Cancer cells can keep living when healthy cells would die. This causes too many cells.

The cancer cells form a growth in the pineal gland. The growth, called a tumor, may press on nearby parts of the brain as it gets bigger. The tumor also may block the flow of fluid in the brain. This causes pressure that builds up inside the brain.

Risk factors for pineoblastoma include:

• Young age. Pineoblastoma is most common in children ages 1 to 12 years old.
• Inherited eye cancer. A genetic disorder called hereditary retinoblastoma raises the risk of pineoblastoma. Hereditary retinoblastoma runs in families. It increases the risk of a type of eye cancer called retinoblastoma and other kinds of cancer.

There is no way to prevent pineoblastoma.

Pineoblastoma diagnosis often involves imaging tests to make pictures of the brain. Other tests might involve removing tissue and fluid for testing.

Imaging tests

Imaging tests can find the location and size of the pineoblastoma. Magnetic resonance imaging, also called MRI, is often used to diagnose brain tumors. Advanced techniques also may be used. These could include perfusion MRI and magnetic resonance spectroscopy.

Additional tests might include computerized tomography scans, also called CT scans, and positron emission tomography scans, also called PET scans.

Removing a sample of tissue for testing

A biopsy is a procedure to remove a sample of tissue for testing in a lab. It can be done with a needle before surgery. Or the sample can be removed during surgery. The tissue sample goes to a lab for tests. The results might show the types of cells and how quickly they are growing.

Removing cerebrospinal fluid for testing

A lumbar puncture is a procedure to remove a sample of the fluid around the brain and spinal cord. This procedure also is called a spinal tap. To get the fluid, a healthcare professional inserts a needle between two bones in the lower spine. The health professional uses the needle to remove some cerebrospinal fluid from around the spinal cord. The fluid is tested to look for pineoblastoma cells. Cerebrospinal fluid also may be collected during a biopsy to remove tissue from the brain.

Pineoblastoma treatment usually involves surgery to remove as much of the cancer as possible. Additional treatments also may be recommended.

Surgery to relieve fluid buildup in the brain

A pineoblastoma may grow big enough to block the flow of cerebrospinal fluid. This puts pressure on the brain. An operation can redirect the fluid through a drain or tube. This helps relieve the pressure. This procedure may be done at the same time as a biopsy or surgery to remove the pineoblastoma.

Surgery to remove the pineoblastoma

A brain surgeon, also called a neurosurgeon, works to remove as much of the pineoblastoma as possible. Sometimes all of the cancer can't be removed. This is because pineoblastoma forms near important structures deep inside the brain. More treatments after surgery are usually needed. These treatments target the remaining cells.

Radiation therapy for pineoblastoma

Radiation therapy treats cancer with powerful energy beams. The energy can come from X-rays, protons or other sources. During radiation therapy, a machine directs beams to the brain and spinal cord. Extra radiation is directed at the cancer cells.

Radiation is often given to the entire brain and spinal cord. This is because the cancer cells can spread from the brain to other parts of the central nervous system. This treatment is often recommended for adults and children older than age 3.

Chemotherapy for pineoblastoma

Chemotherapy treats cancer with strong medicines. For pineoblastoma, chemotherapy is usually used after surgery or radiation therapy. Sometimes it's used at the same time as radiation therapy. For larger pineoblastomas, chemotherapy may be used before surgery. This might shrink the cancer and make it easier to remove.

Radiosurgery for pineoblastoma

Stereotactic radiosurgery focuses multiple beams of radiation on exact points to kill cancer cells. Radiosurgery is sometimes used to treat pineoblastoma that comes back after treatment.

Clinical trials

Clinical trials are studies of new treatments. These studies offer a chance to try the latest treatment options. Side effects from these treatments may not be known. Ask your child's healthcare professional whether your child might be able to participate in a clinical trial.

Make an appointment with your usual doctor or other healthcare professional if you or your child have any symptoms that worry you. If your health professional thinks that you or your child might have pineoblastoma, you may be referred to a specialist. This might be a cancer doctor, called an oncologist. You also may see a surgeon who specializes in operating on the brain, called a neurosurgeon.

Appointments can be short and being prepared can help. Here's some information that may help you get ready.

What you can do

When you make the appointment, ask if there's anything you need to do in advance, such as fasting before having a specific test. Make a list of:

• Your symptoms, including any that seem unrelated to the reason for your appointment.
• Key personal information, including major stresses, recent life changes and family medical history.
• All medications, vitamins or other supplements you take, including the doses.
• Questions to ask your doctor.

Take a family member or friend along, if possible, to help you remember the information you're given.

For pineoblastoma, some basic questions to ask your doctor include:

• Do I have cancer?
• Do I need to have the cancer removed?
• Has the cancer spread?
• Do I need more tests?
• What are my treatment options?
• What are the potential risks for these treatment options?
• Do any of the treatments cure my cancer?
• Can I have a copy of my pathology report?
• How much time can I take to consider my treatment options?
• Are there brochures or other printed materials that I can take with me? What websites do you recommend?
• What would happen if I choose not to have treatment?

Don't hesitate to ask other questions.

What to expect from your doctor

Your doctor is likely to ask you several questions, such as:

• When did your symptoms begin?
• Have your symptoms been continuous or occasional?
• How severe are your symptoms?
• What, if anything, seems to improve your symptoms?
• What, if anything, appears to worsen your symptoms?