Pineoblastoma

Pineoblastoma is a rare, cancerous brain tumor that forms in your pineal gland. This tiny, pinecone-shaped gland is in the center of your brain. Pineoblastoma can spread quickly through the fluid surrounding your brain (cerebrospinal fluid).

Overview

What is pineoblastoma?

Pineoblastoma is a cancerous (malignant) brain tumor in your pineal gland. This type of cancer grows quickly and can spread to tissue and fluid around your brain.

What is the pineal gland?

The pineal gland is a tiny, pinecone-shaped gland in the middle of your brain. This gland secrets the hormone melatonin, which helps regulate your body’s circadian rhythm. Circadian rhythms are changes that your body makes to follow a 24-hour cycle. This helps you stay awake during the day and fall asleep at night.

What type of cancer is pineoblastoma?

Pineoblastoma is a fast-growing tumor in your brain. Healthcare providers might also refer to this as a grade 4 intracranial brain tumor. You might also hear it called a malignant tumor, which means it’s cancerous.

How common is pineoblastoma?

Pineoblastoma is very rare. Pineal gland brain tumors account for less than 0.2% of all brain tumors diagnosed in the United States. This type of cancer is most common among children or people younger than 20 years old but it can affect anyone at any age.

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Symptoms and Causes

What are the symptoms of pineoblastoma?

Symptoms of pineoblastoma include:

The symptoms of pineoblastoma occur due to hydrocephalus, or a buildup of cerebrospinal fluid around your brain. The cancer spreads through cerebrospinal fluid to other parts of your brain and central nervous system (CNS). Pineoblastoma rarely spreads beyond your CNS.

What causes pineoblastoma?

An overgrowth of pinealocyte cells, which are the cells within your brain’s pineal gland, causes pineoblastoma. Research suggests that a change to your genetic code (mutation) may influence these cells to function abnormally. A child can inherit genetic changes from their biological parents when the egg and sperm meet during conception (germline mutation). In contrast, these changes can happen randomly after conception (sporadically), without any history of the genetic change in a child’s biological family.

What are the risk factors for pineoblastoma?

You may be at a higher risk of developing pineoblastoma if you have retinoblastoma. Retinoblastoma is a type of cancer that affects the retina of your eyes.

In addition, if you have a genetic change to the RB1 and DICER1 genes, you’re more likely to develop pineoblastoma. A child can either inherit this genetic change or the change happens randomly in their DNA after conception. If you plan to expand your family, talk to your healthcare provider about genetic testing to see if you have risk factors for pineoblastoma.

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Diagnosis and Tests

How is pineoblastoma diagnosed?

A healthcare provider will diagnose pineoblastoma after a physical exam and testing. During the physical exam, your healthcare provider will ask you questions about your symptoms and your medical history, and they’ll check your eye movement. Tests may include:

Management and Treatment

Is there a cure for pineoblastoma?

While there isn’t a cure available for pineoblastoma, there are several treatment options. Your healthcare provider can surgically remove the tumor if it hasn’t spread. Pineoblastoma can be challenging to treat, which increases the risk of complications from this type of cancer. It may lead to symptoms that get worse over time and cause a shortened life expectancy. There’s a chance that it may come back after treatment.

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How is pineoblastoma treated?

Treatment for pineoblastoma may include:

  • Surgery to remove some or all of the tumor. Your surgeon will let you know what’s possible with surgery and the potential risks.
  • Surgery to treat hydrocephalus. A surgeon may insert a shunt, or a small tube, to drain excess cerebrospinal fluid from building up around your brain.
  • Radiation therapy. A common treatment after surgery, radiation therapy can shrink tumors or destroy cancer cells.
  • Chemotherapy. Chemotherapy is a treatment option in addition to radiation therapy and surgery. Chemotherapy can shrink the tumor before surgery or destroy the remaining cancer cells after surgery.
  • High-dose chemotherapy followed by autologous stem cell transplant. A healthcare provider will collect your own stem cells before receiving certain high-dose chemotherapy. Then you’ll receive an injection of the stem cells back into you after the chemotherapy.

As new medications and treatments become available, you may qualify to participate in clinical trials. This may include trying new targeted therapy or immunotherapy medications.

Who treats pineoblastoma?

Your care team for pineoblastoma may include:

Are there side effects of the treatment?

Radiation therapy can affect your endocrine function, including your:

  • Growth.
  • Energy levels.
  • Fertility.

Before you begin treatment, discuss the potential side effects with your healthcare provider. They’ll also schedule regular checkups to monitor and treat side effects if they occur.

Prevention

Can pineoblastoma be prevented?

There’s no known way to prevent pineoblastoma.

Outlook / Prognosis

Can you survive pineoblastoma?

Yes, some people may survive pineoblastoma. The five-year survival rate for pineoblastoma is 60% to 69.5%. This means that between 60% to nearly 70% of people with the condition are still alive five years later. While pineoblastoma can lead to a shortened life expectancy, treatment is available to help you feel better and help you live longer.

What’s the prognosis for pineoblastoma?

Your healthcare provider will tell you your prognosis (outlook) after a pineoblastoma diagnosis. While there isn’t a cure for pineoblastoma, treatment is available. A person’s life expectancy varies based on many factors, like your overall health, how you respond to treatment and the speed of tumor growth.

After a pineoblastoma diagnosis, you’ll need regular appointments with a healthcare provider. They’ll address any side effects if they arise.

Living With

When should I see a healthcare provider?

Visit a healthcare provider if you or your child experiences symptoms of pineoblastoma, like:

  • A headache.
  • Behavioral changes.
  • Eye movement problems.

If you received a pineoblastoma diagnosis and you’re undergoing treatment, let your healthcare provider know if you experience any side effects.

Treatment may affect your child’s growth if they’re young. If you notice that your child needs more time to develop for their age, talk to their provider.

What questions should I ask my doctor?

Questions to ask your doctor include:

  • What type of treatment do you recommend?
  • Has the cancer spread?
  • Do I need surgery?
  • What are the side effects of treatment?
  • What’s my life expectancy or prognosis?

A note from Cleveland Clinic

It can be difficult to hear the words, “you have cancer.” You may feel a rush of emotions and have questions about what to expect and what to do next. Your healthcare provider and care team will help you navigate your diagnosis and find a treatment option that works best for you. During this time, surround yourself with family and friends who can support you as you take the first step into treatment. Research is ongoing to learn more about new treatments for pineoblastoma to help you feel better.

Medically Reviewed

Last reviewed by a Cleveland Clinic medical professional on 05/07/2023.

Learn more about our editorial process.

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