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Pineoblastoma

Other names: Pineal gland tumor, Pinealoblastoma

What is pineoblastoma?

Pineoblastoma is a rare brain tumor that may occur in children and young adults. This tumor begins in the pineal gland, a tiny structure found deep in the brain.

About half of all tumors found in the pineal gland are pineoblastomas. Because of their location, these tumors can be hard to treat. Pineoblastoma tumors make up less than 1% of childhood brain tumors. The 5-year survival rate for childhood pineoblastoma ranges from 67–85% in the United States, depending on the type.

The pineal gland releases the hormone melatonin, which controls sleep. Melatonin also helps control the release of reproductive hormones from the pituitary gland, such as luteinizing hormone (LH) and follicle-stimulating hormone (FSH). These hormones are important for fertility and reproductive health. Some treatments for pineoblastoma may affect fertility.

Medical illustration of brain anatomy

Pineoblastoma is a rare tumor that develops in the pineal gland in the brain.

Symptoms of pineoblastoma

Signs and symptoms of pineoblastoma depend on the tumor size and if it has spread to other parts of the brain. Often, the growing tumor causes cerebrospinal fluid to build up in the brain, adding pressure. This is known as hydrocephalus. The increased pressure may cause some symptoms that occur with pineal tumors.

Pineoblastoma symptoms may include:

  • Eye pupils are larger than normal or are differing sizes
  • Change in eye movements, especially problems looking up
  • Headache
  • Dehydration
  • Thirsty more than normal
  • Bed-wetting that starts suddenly
  • Frequent urination at night
  • Nausea and vomiting
  • Changes in energy level
  • Tiredness
  • Problems with coordination, balance, or movement
  • Weakness on 1 side of the body
  • Seizures
  • Changes in behavior
  • Slow rate of growth
Medical illustration of hydrocephalus

Fluid buildup in the brain can add pressure. This condition is known as hydrocephalus and may need treatment.

Risk factors of pineoblastoma

Often, doctors do not know what causes pineoblastoma. But some factors increase risk. Pineoblastoma:

Your child’s care team may refer your family to a genetic counselor if needed.

Diagnosis of pineoblastoma

A cancer diagnosis requires tests and procedures. The doctor will give your child a physical exam, ask about their medical history, and order tests such as:

  • Biopsy to check for cancer cells
  • Imaging tests
  • Neurological exam with tests that check how the brain and spinal cord function
  • Lumbar puncture to look for cancer cells in the cerebrospinal fluid (CSF)
  • Blood tests such as complete blood count
  • Blood studies to look for tumor markers (substances released by certain tumors)
  • Genetic tests to look for changes to the DNA of cells

Stages of pineoblastoma

This cancer usually stays in the brain and spinal cord (central nervous system). In 10–20% of cases, pineoblastoma spreads through the cerebrospinal fluid to other parts of the body.

Pineoblastoma is classified by metastasis, or spread of disease:

  • M0: The tumor is in 1 place with no sign of spread to other parts of the body.
  • M1: Tumor cells have spread to the cerebrospinal fluid (CSF).
  • M2: The tumor has spread to other parts of the brain.
  • M3: The tumor has spread to the spine.
  • M4: The tumor has spread outside the central nervous system (CNS).

Pineal gland tumors are also grouped based on how they look under a microscope, and how fast they may grow and spread in the body. The more abnormal the tumor cells look, the higher the grade of tumor. Pineoblastomas are high-grade (grade 4) tumors.

Treatment of pineoblastoma

Treatment for pineoblastoma depends on:

  • The type of pineoblastoma
  • Patient age and health when diagnosed
  • If the cancer has spread
  • If the tumor can be surgically removed
  • If the patient is old enough to receive radiotherapy
  • Available treatments

Treatments may include:

The doctor will review the treatment options.

Prognosis for pineoblastoma

The prognosis for pineoblastoma depends on many factors. The 5-year survival rate for childhood pineoblastoma ranges from 67–85% in the United States, depending on the type, your child’s age, and the treatments received.

Your child’s care team is the best source of information about your child’s case.

Factors that may affect the chance of cure include:

  • Type of pineoblastoma
  • Age at diagnosis
  • If the cancer has spread.
  • If the tumor can be surgically removed
  • If the patient is old enough to receive radiotherapy

Successful removal of the tumor and treatment with radiation therapy are linked to better outcomes for children with pineoblastoma. 

Support for patients with pineoblastoma

Coping with a cancer diagnosis and treatment can be stressful for the patient and family. You may want to talk with a social worker, psychologist, or another mental health specialist.

Learn how to talk to your child about cancer.

After treatment, your child's doctor may use imaging tests and exams to watch for recurrence. Your child may also need treatment for neurological, cognitive, and endocrine problems.

Some treatments can cause late effects. These are health problems that happen months or years after the end of treatment. Patients who have radiation therapy to the pineal gland are at risk for long-term endocrine problems, such as low pituitary function. The endocrine glands make hormones that control body functions. This can lead to problems such as: 

Ongoing monitoring of hormone levels is important. Patients may need medicines including hormone replacement to replace missing natural hormones when the body does not make what it needs.

Patients who receive radiation therapy to the pineal gland are at risk for long-term endocrine system changes that may lead lead to problems such as delayed growth, fatigue, and fertility problems.

Patients who get radiation therapy to the pineal gland are at risk for long-term endocrine system changes.

After completing treatment, it is important to:

  • Have regular checkups and screenings by a primary care provider
  • Maintain healthy habits, including physical activity and healthy eating
  • Have a survivorship care plan to share with health providers. This plan should include:
    • Guidance on health screenings
    • Disease risk factors
    • How to improve health

Childhood cancer survivors should get follow-up care throughout their lives.

Questions to ask your care team

  • What does the biopsy show?
  • Has my child’s cancer spread?
  • What are my child's treatment options?
  • What are the possible side effects of each treatment?
  • How should my child manage side effects?
  • Will my child need to be in the hospital for treatment?
  • Where is the treatment available? Is it close to home or will we have to travel?
  • What resources can we use to help cope with this illness?
  • What monitoring does my child need after treatment?
  • How can I help my child stay healthy?
  • What are the possible late effects of treatment?

Key points about pineoblastoma

  • Pineoblastoma is a rare brain tumor of the pineal gland, which is located deep within the brain.
  • These tumors may be hard to treat due to their location.
  • Signs and symptoms of pineoblastoma depend on the tumor’s size and if it has spread to other parts of the brain.
  • Diagnosis may require a physical exam, biopsy, imaging tests, neurological exam, lumbar puncture, blood tests, and genetic tests.
  • Treatment depends on the tumor, but may include surgery, radiation, and chemotherapy.
  • The survival rate for newly diagnosed pineoblastoma is about 67–85% in the U.S. This depends on the disease type, your child’s age and health, and the type of treatment they have. Discuss your child’s prognosis with their care provider.


Reviewed: June 2023