An ependymoma is a tumor that develops from the cells lining the ventricular system of the brain or spinal cord. Ependymal cells make cerebrospinal fluid. Ependymomas in children usually appear in the brain, most commonly in the fourth ventricle (or back part of the brain). In rare cases, an ependymoma can arise in the spinal cord.
- Ependymoma accounts for five to ten percent of pediatric brain tumors. It’s the third most common childhood brain tumor.
- Though ependymoma only rarely occurs in the spinal cord, it accounts for about 25 percent of all spinal cord tumors.
- Most patients with spinal cord tumors are older than 12.
- It occurs equally in boys and girls.
Children with ependymomas receive expert care at Dana-Farber/Boston Children's Cancer and Blood Disorders Center through the Childhood Brain Tumor Center. Our pediatric oncologists have extensive expertise in treating all types of brain and spinal tumors, including ependymomas.
Symptoms of Childhood Ependymoma
Brain tumors can cause a variety of symptoms in children, depending on their size and location. Ependymoma symptoms are most often associated with increased pressure in the brain and can include:
- Headache, generally upon awakening in the morning
- Hydrocephalus, a buildup of fluid in the brain, which can result in increased head size in infants or headaches and vomiting in older children
- Nausea and vomiting, often worse in the morning and improving throughout the day
- Problems eating or walking
How We Diagnose and Classify Childhood Ependymoma
Doctors most commonly diagnose ependymoma with comprehensive physical and neurological exams, advanced imaging studies, biopsy, and lumbar puncture.
Successfully treating your child’s brain tumor depends on the tumor’s location, whether the disease has spread, and what type of ependymoma it is. Doctors will classify your child's tumor as one of the following types:
- Myxopapillary ependymoma
Classic and anaplastic ependymomas are treated the same way and have similar prognoses. The third type, myxopapillary ependymoma, which usually occurs at the base of the spine, tends to be much less aggressive than the other forms.
After completing all necessary tests, our experts meet to review and discuss what they have learned about your child's condition. Then we will meet with you and your family to share the results and outline the best possible treatment options.
How We Treat Childhood Ependymoma
Your child's physician will determine a specific course of treatment based on several factors, including your child's age and overall health, the tumor type, location, and size of the tumor, and the extent of the disease.
Ependymoma treatments include:
- Surgery: Surgery is commonly the first step in treating ependymoma. During surgical removal, the surgeon will remove as much of the tumor as possible. Sometimes the tumor's location or other factors may make it impossible to remove it altogether.
When a tumor causes blockage of cerebral spinal fluid flow, our surgeons may perform one of two procedures to relieve symptoms of hydrocephalus, the buildup of fluid inside the skull.
- Endoscopic third ventriculostomy (ETV): In an endoscopic third ventriculostomy, surgeons create a small hole that allows fluid to flow around the blockage. About 90 percent of children with hydrocephalus symptoms will receive this procedure.
- Ventriculo-peritoneal shunt (VP shunt): In some cases, children may have an alternative procedure in which a tube is placed in the ventricles to the abdomen to drain excess fluid into the stomach.
- Radiation therapy: This treatment uses high-energy waves from a specialized machine to damage or shrink tumors. We use precisely targeted and dosed radiation therapy to destroy cancer cells left behind after surgery. Your child may receive this treatment to control the local growth of the tumor. If an ependymoma has spread, your child's physician may recommend radiation to the whole brain and spinal cord.
- Chemotherapy: Chemotherapy is a drug treatment that interferes with a cancer cell’s ability to grow or reproduce. We may use it before surgery to shrink the tumor. Different groups of chemotherapy drugs work in different ways and are generally systemic treatments. Your child may receive chemotherapy:
- Orally, as a pill to swallow
- Intramuscularly, as an injection into the muscle or fat tissue
- Intravenously, as a direct injection into the bloodstream, or IV
- Intrathecally, as a direct injection into the spinal column through a needle
Ependymoma treatments can depend on the site of the tumor and whether it is recurrent (doesn't resolve after initial treatment) or not.
Within the brain and posterior fossa, outcomes are best when a combination of complete surgical removal and high dose focal radiation therapy is possible. For spinal cord ependymomas, we use a similar approach. An ependymoma that has spread, or metastasized, may require additional chemotherapy and radiation depending on your child’s age.
Follow-up cancer care is critical to your child’s physical, mental, and emotional health. Beyond initial treatment, we continue to care for the physical, mental, and emotional health needs of children through our extensive pediatric cancer survivorship programs, including the Stop & Shop Family Pediatric Neuro-Oncology Outcomes Clinic for pediatric brain tumor survivors. These services address health and social issues, ranging from motor function evaluation and physical therapy to return-to-school and learning programs.
Research and Clinical Trials for Childhood Ependymoma
At Dana-Farber/Boston Children’s, we have an unparalleled depth of experience in developing new treatments for brain tumors through clinical trials.
Our researchers investigate pediatric brain tumor topics such as anti-tumor medications, genetic markers on tumor cells, and the long-term effects of treatment and ways to minimize these effects.
In addition to launching our own clinical trials, we also offer trials available through collaborative groups such as the Children's Oncology Group (COG) and the Pacific Pediatric Neuro-oncology Consortium (PNOC). We are also the New England Phase I Center of the Children's Oncology Group. If your child has a progressive or recurrent tumor, she may be eligible for a number of clinical trials available through these groups, or from one of our independent clinical investigators.
If you have questions about clinical trials, your child’s doctor can explain more about what’s available and if your child is eligible.
Long-term Outcomes for Children with Ependymoma
The cure rate for ependymoma with complete surgical removal is 65 percent.
Metastatic, progressive, or recurrent ependymomas are most challenging to cure. In about 12 percent of patients, the disease has spread at the time of diagnosis, and more extensive treatment is necessary to cure the disease. When ependymomas recur, they usually do so at the original tumor site.
Childhood Ependymoma Treatment Team
Our doctors have extensive experience in treating children with ependymoma. See a complete list of the specialists in our Childhood Brain Tumor Center.