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.
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 that are larger than normal or are differing sizes
Changes in eye movements, especially problems looking up
Headache
Dehydration
Thirsty more than normal
Bed-wetting that starts suddenly
Frequent urination (peeing) 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
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 may:
Be more common in some ages than others, depending on the type
Your child’s care team may refer your family to a genetic counselor if needed.
Diagnosis of pineoblastoma
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:
Blood studies to look for tumor markers (substances released by certain tumors)
Genetic tests to look for changes to the DNA of cells
Types of pineoblastoma
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:
Pineoblastoma-miRNA1
Pineoblastoma-miRNA2
Pineoblastoma, FOXR2 activated
Pineoblastoma, RB1 activated
Stages of pineoblastoma
This 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:
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
The goal of surgery is to remove as much of the tumor as possible. Surgery for pineoblastoma can be difficult because of the tumor location and the risk to nearby blood vessels. In some cases, it is not possible to remove all of the tumor in 1 surgery. In such cases, surgeons may decide to:
Do both a biopsy and chemotherapy so that surgeons can later fully remove the tumor
Some patients with pineoblastoma may have surgery to help reduce the side effects from hydrocephalus. A surgeon will make a small opening in the third ventricle in the brain. This opening helps cerebrospinal fluid flow more freely to keep it from building up and causing harm. In other cases, doctors may place a shunt, a small tube that drains cerebrospinal fluid from the brain. The shunt may be temporary or permanent.
Radiation Therapy
Radiation therapy is often given after pineoblastoma surgery. Energy from the radiation can damage or destroy rapidly growing cancer cells. Doctors can also use radiation to shrink the tumor. The dose and amount of radiation therapy depend on:
Your child’s age
The type of tumor
If the disease has spread
Radiation is not used in young children because it increases the risk of long-term side effects. In patients older than 3, radiation is usually given to the whole nervous system (craniospinal irradiation). A higher dose (boost) is given at the primary tumor site.
Radiation therapy uses beams of radiation to shrink tumors and kill cancer cells. It works by damaging the DNA inside cancer cells.
Chemotherapy
Chemotherapy is often used along with radiation therapy and surgery:
Before surgery, doctors sometimes use chemotherapy to shrink the tumor so it is easier to remove.
After surgery, chemotherapy can destroy cancer cells left behind.
In very young children, chemotherapy may be used to slow the growth of cancer until the child is old enough to get radiation safely.
Several chemotherapy medicines may be used at the same time to treat pineoblastoma. Common treatments are vincristine, cyclophosphamide and cisplatin. Your child's care team may use other types of chemotherapy.
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 radiation therapy
If the tumor was newly diagnosed or has returned (recurred)
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 you and your family. You may want to talk with a social worker, psychologist, or another mental health specialist.
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:
Have regular checkups and screenings by a primary care provider
Maintain healthy habits, including physical activity and healthy eating
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?
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.
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.
The treatments that cure cancer can also have certain long-term and late side effects. Learn about which treatments are linked to certain late effects.
Medulloblastoma is the most common malignant brain tumor in children. It is a fast-growing tumor that begins in the cerebellum in the back of the brain.
