Other names: Pineal gland tumor, Pinealoblastoma
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 30% (3 in 10) 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.
Pineoblastoma is a rare tumor that develops in the pineal gland in the brain.
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:
Fluid buildup in the brain can add pressure. This condition is known as hydrocephalus and may need treatment.
Often, doctors do not know what causes pineoblastoma. But some factors increase risk. Pineoblastoma may:
Your child’s care team may refer your family to a genetic counselor if needed.
A pinoblastoma diagnosis requires tests and procedures. The doctor will give your child a physical exam, ask about their medical history, and order tests such as:
There are different types of pineoblastoma based on the genetic changes (mutations) that caused the tumor. Your child's tumor needs molecular testing for an accurate diagnosis and to determine the best treatment.
Types of pineoblastoma in children include:
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Listen to the podcastThis cancer usually stays in the brain and spinal cord (central nervous system). In 10–20% (1–2 of 10) of cases, pineoblastoma spreads through the cerebrospinal fluid to other parts of the body.
Pineoblastoma is classified by metastasis, or spread of disease:
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 for pineoblastoma depends on:
Treatments may include:
The doctor will review the treatment options.
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:
Successful removal of the tumor and treatment with radiation therapy are linked to better outcomes for children with pineoblastoma.
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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 get radiation therapy to the pineal gland are at risk for long-term endocrine system changes.
After completing treatment, it is important to:
Childhood cancer survivors should get follow-up care throughout their lives.
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Reviewed: August 2024
The brain and spinal cord make up the central nervous system (CNS). CNS tumors may be benign or malignant.
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