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Ependymoma

What is Ependymoma?

Ependymoma is a tumor of the brain and spinal cord. It is the 3rd most common malignant brain tumor in children. There are about 200 new cases of pediatric ependymoma in the United States each year.

Ependymomas are most common in children under 5 years of age. However, they may develop at any age.

Ependymomas grow from cells that line the fluid-filled ventricles of the brain and central canal of the spinal cord.

These tumors can sometimes spread through the cerebrospinal fluid (CSF).

Many ependymomas form in the fourth ventricle in the posterior fossa region. Tumors in this location are most likely to affect the brain stem and cerebellum.

Many ependymomas form in the fourth ventricle in the posterior fossa region. Tumors in this location are most likely to affect the brain stem and cerebellum.

In children, about 75% of ependymomas occur in the posterior fossa region of the brain, but they can also develop in other areas of the central nervous system (CNS). Depending on the location of the tumor, different parts of the CNS may be affected including the:

  • Cerebrum
  • Cerebellum
  • Brainstem
  • Spinal cord

Many ependymomas form in the fourth ventricle in the posterior fossa. These tumors are most likely to affect the brain stem and cerebellum.

Treatment for ependymoma includes surgery to remove as much of the tumor as possible. Radiation therapy is often used along with surgery to kill any remaining cancer cells. Chemotherapy may also be used before or after surgery.

The survival rate for childhood ependymoma is 50-70%. Prognosis depends on the location of the tumor, the amount of tumor that can be removed with surgery, and features of the tumor cells. Doctors are learning more about the biology and molecular features of ependymoma. This information can help predict how a tumor might respond to treatment.

Ependymoma can come back after treatment, and children often need long-term care to watch for recurrence.

Risk Factors and Causes of Ependymoma

Most ependymomas occur in infants and young children under 5 years old.

Certain changes in genes and chromosomes within the tumor cell are associated with ependymoma. Usually, it is not known why these genetic changes occur.

Some children may be at increased risk for brain tumors including ependymoma due to neurofibromatosis type 2 (NF2), a rare inherited condition.

Symptoms of Ependymoma

Signs and symptoms of childhood ependymoma depend on several factors including the size and location of the tumor and the child’s age and stage of development.

Ependymoma symptoms may include:

  • Headache
  • Nausea and vomiting, often worse in the morning
  • Back or neck pain
  • Vision problems
  • Loss of balance or problems walking
  • Leg weakness
  • Seizures
  • Irritability or confusion
  • Problems urinating or change in bowel function
  • Increased head size in infants

As the tumor grows, it often blocks the normal flow of cerebrospinal fluid. This causes a buildup of fluid within the brain known as hydrocephalus. The fluid increases pressure in the brain (intracranial pressure). Many of the symptoms of ependymoma are due to hydrocephalus.

Diagnosis of Ependymoma

  1. A physical exam and medical history help doctors learn about symptoms, general health, past illness, and risk factors.

  2. A neurological exam measures different aspects of brain function including memory, vision, hearing, muscle strength, balance, coordination, and reflexes.

  3. Imaging tests are used to help identify the tumor. Magnetic resonance imaging (MRI) of the brain and spinal cord is the main imaging technique used to evaluate ependymoma. MRI is also done after surgery to see if any tumor remains.

  4. A lumbar puncture may be performed to look for cancer cells in the cerebrospinal fluid.

  5. A biopsy is used to diagnose ependymoma. In a biopsy, a small sample of the tumor is removed during surgery. A pathologist looks at the tissue sample under a microscope to identify the specific type and grade of ependymoma.

Grading and Staging of Ependymoma

Ependymoma tumors are identified by how they look under the microscope. They are generally classified as grade I, grade II, or grade III tumors. The more abnormal the cells look, the higher the grade. Children with ependymoma usually have tumors that are grade II (classic ependymoma) or III (anaplastic ependymoma).

Certain types of ependymoma tend to have a better outcome than other types. However, other factors also affect treatment and prognosis.

  Tumor Subtypes Based on Histology
Grade I  Subependymoma
Myxopapillary ependymoma
Grade II  Classic ependymoma (includes papillary, clear cell, and tanycytic ependymoma)
Grade III     Anaplastic ependymoma

Prognosis for Ependymoma

The overall 10-year survival for pediatric ependymoma is about 50-70%. The prognosis depends on multiple factors. Children with certain types of ependymoma tend to have a better chance for long-term survival. However, late recurrences or relapse (beyond 5 years) may occur.

Factors that influence chance of cure include:

  • Location of the tumor
  • Type and grade of the tumor
  • Whether the cancer is localized or has spread (metastasis)
  • If surgery can completely remove the tumor
  • Age at diagnosis
  • If the cancer has come back (relapse or recurrence)
  • Molecular features of the tumor

Scientists are learning more about the molecular types of ependymoma. These advances can help doctors understand why patients with tumors in specific parts of the brain or tumors that develop at a certain age respond better to treatments. Doctors can use molecular classification of the tumor to help predict risk and plan treatments.

Specific changes in the molecular or genetic characteristics of the tumor cells may be considered higher risk. High risk ependymoma includes posterior fossa tumors with extra copies of the chromosome 1q (1q gain).

Doctors may also look for other molecular changes including:

  • supratentorial tumors with RELA fusion (C11orf95-RELA fusion)
  • supratentorial tumors with a YAP fusion

Certain tumor features make the disease harder to cure. Doctors may use molecular profiles of tumors to help plan treatments.

Ependymoma is hard to cure without successful surgery to remove the tumor. When surgery is incomplete or ependymoma recurs, the long-term prognosis is usually poor.

Treatment of Ependymoma

Treatment of ependymoma usually includes surgery followed by focal radiation therapy. Some patients may also receive chemotherapy. Treatment depends on the type and location of tumor, age of the child, and whether the tumor is newly diagnosed or recurrent.

  1. Surgery to remove as much of the tumor as possible is the main treatment for ependymoma. The goal is gross total resection, or complete removal of the tumor. However, it may not always be possible to remove all of the tumor due to risk of damage to brain structures.

    Risks of surgery depend on the size of the tumor and the specific region of the brain involved. Possible complications include damage to brain structures that control muscle movement, speaking, swallowing, or hearing.

    A young cancer patient with a scar on the back of his head.

    Some children who have surgery for ependymoma develop posterior fossa syndrome. Symptoms usually develop within a few days after surgery and include changes in speech, swallowing, motor function, and emotions. Symptoms can be mild or severe, and most children improve over time. Read more about posterior fossa syndrome.

    For patients with certain grade I tumors, surgery alone may be an effective treatment depending on tumor type, location, and the extent of resection. Some patients may have a second surgery after a course of chemotherapy to remove more of the tumor.

  2. Radiation therapy is usually used after surgery for ependymoma except in infants. Focal radiation therapy to the tumor and a small margin of healthy tissue is generally preferred. The dose and volume of radiation depends on the type and location of the tumor and spread of disease. Because ependymoma does not respond as well to chemotherapy, radiation therapy is used in children more than 1 year of age.

  3. Chemotherapy is often used along with focal radiation therapy and surgery. Chemotherapy may be used before surgery to make removal of the tumor safer and more complete. In very young children, especially infants who are less than 1 year of age, chemotherapy may be used to help delay radiation until the child is older. The chemotherapy treatment plan will depend on factors such as spread of disease and molecular features of the tumor. A combination of chemotherapy medicines is used to treat ependymoma. These medicines often include:

    Other types of chemotherapy may also be used.

  4. Children who have a recurrence of disease after initial surgery and radiation therapy may be offered treatment within a clinical trial. There is a lack of successful standard therapies for recurrent ependymoma. Clinical trials include the use of immunotherapy drugs targeting the immune system to help fight the cancer and targeted therapies acting on specific proteins in the tumor cells.

Some children with hydrocephalus due to ependymoma may have a shunt placed in the brain to prevent cerebrospinal fluid from building up. A shunt is a small tube that drains fluid from the brain.

A shunt is a small tube that drains cerebrospinal fluid to prevent the build-up of fluid.

A shunt is a small tube that drains cerebrospinal fluid to prevent the build-up of fluid.

Current risk-based approaches to ependymoma treatment classify patients based on specific tumor features and predicted outcomes. Doctors consider:

  • Whether surgery completely removed the tumor
  • Presence of metastatic disease
  • Tumor histology
  • Genetic features of the tumor

The goal of a risk-based approach is to achieve cure while lowering the risk of treatment side effects. For low risk patients, lower intensity therapy can reduce long-term problems from radiation and chemotherapy. For high-risk patients, more intensive therapy can improve chance of survival.

Incorporating palliative care and support services such as rehabilitation, psychology and social work can help ependymoma patients and families manage symptoms, promote quality of life, and make care decisions.

Life after Ependymoma

Ongoing follow-up care, laboratory tests, and routine imaging are needed to monitor patients for recurrence of disease and other medical problems. The care team will set a schedule based on type of tumor, response to treatment, and individual patient needs.

Later relapse of ependymoma is more common compared to some other cancers. Most patients who relapse do so within 5 years of their diagnosis. However, these tumors can also recur after the 5-year milestone of cancer survival.

General Follow-Up Schedule for Ependymoma Survivors:

  • Years 0-3: MRI of the brain every 3-4 months
  • Years 3-5: MRI of the brain every 6 months
  • After 5 years: MRI of the brain every 12 months for 2-5 more years

Survivors of CNS tumors are at risk for problems such as decreased cognitive function, hearing loss, sleep problems, hormone problems, stroke, and second cancers. These problems may be caused by the tumor itself or develop as long-term or late effects related to treatment. Regular checkups and screenings by a primary care physician are important to watch for health problems that can develop years after therapy.

Find More Information on Ependymoma

More: Life After Brain Tumors


Reviewed: July 2019