Ependymoma is the third most common brain cancer in children, accounting for up to 10 percent of all childhood brain tumors. Though ependymomas can also appear in adults and older children, close to half occur in children under the age of three. Ependymomas are treated with a combination of surgery, radiation therapy and chemotherapy tailored to the specific circumstances of each patient.
What Is an Ependymoma?
Ependymomas arise from the abnormal growth of ependymal cells, a kind of cell that supports and insulates the brain’s information-transmitting neurons. In children, ependymomas most often appear in the brain’s ventricles, cavities that are filled with cerebrospinal fluid.
Ependymomas are usually localized on a primary tumor site, but a small number can spread, or metastasize, to other sites in the brain or spinal cord. Very rarely, these tumors can spread to other areas in the body.
“Classic” Ependymoma
The World Health Organization grades tumors on a scale of I to IV. “Classic” ependymoma, the type most often seen in children, is a grade II tumor that usually occurs in the brain’s posterior fossa, surrounding the fourth ventricle at the back of the brain.
Anaplastic Ependymoma
A faster growing and more aggressive form of ependymoma, anaplastic ependymomas are grade III tumors on the WHO scale. These tumors have more abnormal cells and active blood vessel growth than classic ependymomas, and they can recur after treatment, which can require additional treatment and monitoring.
Diagnosing an ependymoma begins with a complete medical history and review of symptoms. Magnetic resonance imaging (MRI) or computerized tomography (CT) scans are used to reveal the tumor, and patients may also have a lumbar puncture to check for tumor cells in the spinal fluid. A biopsy of the tumor tissue can confirm the diagnosis and reveal the tumor type.
Ependymoma Symptoms
The symptoms of an ependymoma are caused by pressure and swelling that displaces healthy brain tissue. Typical symptoms resemble those of many other illnesses, and include:
- Headache
- Nausea
- Vomiting
Other symptoms depend on the tumor’s location in the brain and its effect on specific brain functions. These can include:
- Clumsiness and balance problems
- Double or blurred vision
- Problems with speech
- Difficulty swallowing
- A distinct tilt to the head
Treatments for Ependymoma
Treating an ependymoma in the brain begins with surgery, and can also include radiation or chemotherapy, depending on your child’s age and health and the nature of the tumor.
Surgery
Surgical resection of the tumor is the primary treatment for pediatric ependymomas, and surgery has several goals: removal of the tumor, collecting tissue for biopsy and relieving pressure and the accumulation of fluid in the brain.
Surgery most often begins with placement of a temporary drain to control any buildup of spinal fluid followed by a craniotomy – the removal of a section of skull to expose the brain. After surgery, this section is replaced and secured. During surgery, your child’s brain tumor surgeon works to remove the tumor from surrounding healthy tissue.
Depending on the ependymoma’s size and location, though, it may not be possible to remove it completely without damaging essential brain structures. In that case, surgeons aim to reduce it as much as possible.
After surgery, patients are monitored in the PICU for several days, which may include a post-operative MRI or CT scan. A typical hospital stay may last from 5-7 days. Any surgery on the brain can affect brain functions and structures. Your child may experience fatigue, headaches or problems with balance, coordination and speech. Postoperative care plans often include physical therapy and in home exercises to support recovery.
Radiation Therapy
For children over three years old, surgery is often followed by a course of radiation therapy. Radiation destroys remaining tumor cells, and can help to shrink any parts of a tumor that could not be removed surgically.
In all its forms, radiotherapy aims targeted beams of radiation at the tumor site. Some forms of therapy also target the spine. Radiation aimed at the tumor site can also affect surrounding healthy tissues, and this raises concerns that it might harm a very young child’s developing brain.
Radiation therapy takes place on an outpatient basis, and the number of treatments depends on a patient’s individual circumstances and the nature of the ependymoma. Side effects can include fatigue, headaches and hair loss near the radiation site.
Chemotherapy
Depending on circumstances, chemotherapy can be used to treat ependymomas, especially for children younger than three years, those with more aggressive tumors or those whose tumors were not completely removed by surgery.
Chemotherapy can be used until a child is old enough for radiation therapy, or it can be used to shrink a tumor in preparation for surgery. Chemotherapy drugs are given either orally or intravenously, and can cause temporary effects such as nausea, vomiting, loss of appetite, fatigue and hair loss. Treatment typically takes place in a series of cycles, with breaks in between.
Treatment outcomes depend on the extent of a tumor’s removal and factors including a patient’s age and overall health. After treatment for ependymoma, frequent follow-ups, including MRI scans, monitor not only the status of the tumor but also any neurological deficits related to treatment itself.
Why Choose Neurosurgeons of New Jersey?
Neurosurgeons of New Jersey combines patient-centered care with decades of experience, research and cutting edge technology. The center’s team of neurosurgeons and other specialists provide state of the art treatment designed to meet the unique needs of pediatric neurosurgery patients and their families. At Neurosurgeons of New Jersey, your brain cancer surgeon works with you to create a treatment plan that’s right for your child.