Ependymoma

What is ependymoma?

An ependymoma is a rare type of primary brain or spinal cord tumors. Ependymomas are found throughout the central nervous system in the supratentorial (top of the head), posterior fossa (back of the head) and spinal compartments.

These tumors affect both children and adults. They are thought to develop from certain cells that normally mature into ependymal cells. Ependymal cells line the ventricles (fluid-filled spaces in the brain) and the central canal of the spinal cord. Sometimes, ependymoma tumor cells can spread in the fluid that surrounds the brain and spinal cord.

How common is ependymoma?

  • In the United States, 200 new cases of ependymoma are found in children and adults each year.
  • Ependymoma occurs more often in children than in adults.
  • Ependymoma is the third most common type of brain tumors in children.
  • Most ependymomas are found in young infants and children.

What are the symptoms of ependymoma?

Ependymoma causes a variety of symptoms. Some symptoms depend on the location of the cancer. If your child has an ependymoma, the following general symptoms may be present:

  • Nausea
  • Vomiting
  • Headache
  • Unsteady gait (walking)

How is ependymoma treated?

  • Surgery—is used to remove as much of the cancer as possible.
    • Complete removal of the tumor is often not possible because of the tumor location and concerns about damaging the surrounding normal brain tissue.
    • For patients with a type of ependymoma called anaplastic ependymoma, complete removal of the tumor may be harder. Anaplastic tumors grow more quickly and tend to spread into the surrounding normal brain tissue.

Following surgery, radiation therapy is commonly used to kill the remaining cancer cells:

  • Radiation therapy—uses high-energy X-rays or other types of radiation to kill cancer cells or stop them from growing. Radiation therapy is commonly used in these cases:
    • When complete removal of the tumor is not possible
    • With more aggressive anaplastic tumors
  • Chemotherapy (“chemo”)—uses powerful medicines to kill cancer cells or stop them from growing (dividing) and making more cancer cells. Chemo may be given either before or after radiation therapy to get rid of remaining tumor cells.

What are the survival rates for ependymoma?

  • The overall five-year survival rate for those with ependymoma is about 82 percent.
  • For children up to 19 years of age, the five-year survival rate is about 72 percent.

Why choose St. Jude for your child’s ependymoma treatment?

  • St. Jude is the only National Cancer Institute-designated Comprehensive Cancer Center devoted solely to children.
  • St. Jude has created more clinical trials for cancer than any other children’s hospital in the United States.
  • The nurse-to-patient ratio at St. Jude is unmatched—averaging 1:3 in hematology and oncology, and 1:1 in the Intensive Care Unit.
  • The program offers a closely knit group of doctors, scientists, nurses and support staff providing the many types of treatment and support services crucial to the complete care of children with brain tumors.
  • Working with the Collaborative Ependymoma Research Network (CERN) and using a pioneering approach to developing drugs, St. Jude researchers have found possible new and repurposed drugs for treating ependymoma. These include 5-fluorouracil (5-FU), which is currently used to treat a variety of adult cancers. Based on this work, St. Jude is conducting a clinical trial of 5-FU in children and young adults who have already had ependymoma treatment but the tumors have returned.

Contact the Brain Tumor Team any time, 24 hours a day, 7 days a week.

Call our Brain Tumor Coordinator, Tabatha E. Doyle, RN:
(901) 595-2544 or
(901) 595-4599

Or email us at braintumors@stjude.org

Associated Clinical Trials

CNS831: Maintenance Chemotherapy or Observation Following Induction Chemotherapy and Radiation Therapy in Treating Younger Patients With Newly Diagnosed Ependymoma

Phase III Randomized Trial of Post-Radiation Chemotherapy in Patients with Newly Diagnosed Ependymoma Ages 1 to 21 Years (ACNS0831)

Diseases Treated:

Intracranial ependymoma

Eligibility:

  • Patient must be greater than 12 months of age and less than 21 years of age at enrollment
  • Patients with newly diagnosed intracranial ependymoma without evidence of metastatic disease (confirmed by MRI brain and spine along with lumbar puncture to check CSF)
  • Patients with diagnosis of spinal cord ependymoma, myxopapillary ependymoma, subependymoma, ependymoblastoma, or mixed glioma are not eligible
  • No prior treatment other than surgical intervention and corticosteriods
View Trial

PBTC48: Study of the Optune Device in Children with Certain High-Grade Glioma and Ependymoma Brain Tumors

Feasibility Trial of Optune for Children with Recurrent or Progressive Supratentorial High-Grade Glioma and Ependymoma

Diseases Treated:

High-grade glioma (HGG), ependymoma

Eligibility:

  • Between 5 and 21 years old
  • Diagnosis of supratentorial high-grade glioma (HGG) or supratentorial ependymoma that is recurrent, progressive or refractory
  • Head circumference of at least 44 cm
  • Failed standard therapy with no other available treatment options
  • Recovered from prior chemotherapy, immunotherapy or radiotherapy
  • Willing and able to use the device at least 18 hours a day for at least 23 days and keep head shaved throughout treatment
View Trial

RERTEP: Surgery and Second-Course Radiation Therapy in Treating Younger Patients With Recurrent Ependymoma

A Phase II Trial of Surgery and Fractionated Re-irradiation for Recurrent Ependymoma

Diseases Treated:

Recurrent ependymoma

Eligibility:

  • Progressive intracranial ependymoma after prior focal irradiation
  • At least 1 year old and younger than 21 years old
  • Adequate performance status (ECOG less than 3)
  • Does not require mechanical ventilation
  • Interval from start of initial radiation therapy to enrollment greater than 9 months
View Trial

SJDAWN: Molecular-Based Therapy for Aggressive Brain and Spinal Cord Tumors in Children and Young Adults

Molecularly-Driven Doublet Therapy for All Children with Refractory or Recurrent CNS Malignant Neoplasms and Young Adults with Refractory or Recurrent SHH Medulloblastoma

Diseases Treated:

Brain tumor, medulloblastoma, ependymoma

Eligibility:

Ages for Study Enrollment

  • Stratum A (ribociclib and gemcitabine):  Between 1 and 24 years old with recurrent, progressive or refractory non-WNT,  non-sonic hedgehog (SHH) (NWNS) medulloblastoma or ependymoma
  • Stratum B (ribociclib and trametinib):  Between 1 and 24 years old with recurrent, progressive, or refractory central nervous system (CNS) tumors, including:
    • High-grade glioma
    • Atypical teratoid rhabdoid tumor (ATRT)
    • SHH and WNT medulloblastoma
    • CNS embryonal tumors [previously called PNET])
  • Stratum C (ribociclib and sonidegib):  Between 10 and 39 years old with recurrent, progressive, or refractory SHH medulloblastoma and copy number loss of 9q or PTCH1 mutation. (This stratum is only open to patients with SHH medulloblastoma who are fully grown.  Patients younger than 18 years old will have bone age to determine if fully grown)

Screening Phase Eligibility

Inclusion Criteria:

  • Participants with recurrent, progressive, or refractory brain tumors
  • At least 1 year old and younger than 25 years old at the time of screening. Exception: Participants with recurrent, progressive or refractory medulloblastoma who are at least 1 year old and younger than 40 years of age at the time of study screening are eligible for screening.
  • Participants and/or guardian have the ability to understand and the willingness to sign a written informed consent document according to institutional guidelines.

Exclusion Criteria:

  • Recurrent, progressive or refractory low grade glioma (LGG)
  • Prior exposure to a CDK4/6 inhibitor
  • History of clinically significant, uncontrolled heart disease and/or repolarization abnormalities
  • History of QTc prolongation
View Trial

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