Ependymomas can develop in all age groups but are more common in children. The exact cause of these tumors is unknown.
In this article, we look at the types, symptoms, diagnosis, and treatment of ependymoma cancer.
- Ependymoma is a glial tumor of the cells that line the spinal cord and ventricles of the brain.
- It is a rare cancer. It is also uncommon for the cancer to spread.
- Ependymoma cancer is more common in children than adults.
- Grading of the tumor is in 3 stages of severity.
- Treatment involves surgical removal of the tumor followed by radiotherapy to remove any remaining cancer cells.
What is ependymoma cancer?
Ependymoma is a rare tumor.
An ependymoma is a glial tumor of the ependymal cells that line the center of the spinal cord and the ventricles of the brain.
Ependymomas are physically soft, have a gray or red color, and may contain mineral calcifications or cysts within them. A tumor can occur anywhere where ependymal cells are present in the central nervous system.
Ependymoma cancer is rare. It occurs in both children and adults, and in men and women relatively equally. However, ependymomas are more common in children than in adults.
There are several different types of ependymoma cancers, which are also classified by grade. The three grades used for ependymoma cancers are determined by how closely the cancer cells resemble normal cells. The lower the grade, the more closely the cancer cells resemble normal cells. The different types of ependymomas appear in different locations within the spinal column or brain.
Grade I tumors - These include subependymomas and myxopapillary ependymomas, and are typically slow growing. Myxopapillary ependymomas tend to appear at the lower end of the spinal column. Subependymomas appear near a ventricle in the brain. These ventricles are involved in the creation of cerebrospinal fluid.
Grade II tumors - These are the most common ependymomas. Many ependymoma subtypes exist in this grade, including cellular, papillary, tancytic, RELA fusion-positive, and clear cell ependymomas. These may appear anywhere along the ventricular system where cerebrospinal fluid is produced.
Grade III tumors - These are known as anaplastic ependymomas and are typically faster growing than the other grades. Anaplastic ependymomas are usually found in the skull, brain, and brain stem. They are rarely found in the spinal cord.
Tumor cells can sometimes spread through the cerebrospinal fluid. If this occurs, tumors may appear in multiple areas of the brain and spine. It is uncommon, however, for this type of cancer to spread to other parts of the body.
In children versus adults
Ependymoma is more common in children than adults.
Ependymomas develop in all age groups but are more common in children than adults.
Ependymomas may differ depending on an individual's age. In children, the tumor is most commonly located in the brain. In adults, this type of cancer is more likely to be seen along the spine.
Infants with ependymoma may be irritable and have difficulty sleeping, and a child's head may grow irregularly.
Children may also develop slower than expected, either physically or mentally.
Age can also affect treatment methods. Younger children may not be able to undergo certain forms of cancer treatment.
Symptoms of ependymoma can vary from case to case. Many people experience noticeable symptoms along with the tumor, but some show little or no symptoms that can be identified.
Symptoms of ependymoma may include:
- neck pain
- vision changes, such as blurred vision or vision loss
- jerky eye movements
- nausea and vomiting
- difficulty with balance or walking
- seizures and convulsions
- tingling, numbness, or weakness in the limbs
Persistent symptoms should be reported to a doctor as soon as possible for screening and diagnosis.
Doctors will use many tests to find and diagnose ependymoma. Depending on the situation, doctors will also run tests to see if tumors have spread, or metastasized, to other parts of the body.
The tests that doctors recommend will vary based on the type of tumor suspected, the person's health, and any symptoms they are showing.
Most tumor types will require a biopsy. This involves removing a small sample of tissue from the tumor area for testing. If a biopsy is impossible or too dangerous to perform, doctors will often suggest other tests to help make their diagnosis.
As part of the diagnostic process, some form of imaging will be used. These tests allow doctors to look at and take pictures of the inside of the body to inspect and measure the tumor. This is usually done with a CT scan or MRI.
A CT scan provides a more complete picture of the inside of the body, by creating a series of X-ray images taken from different angles. An MRI creates a similar image, using magnetic fields instead of X-rays. Sometimes a special dye or other contrast medium is injected into the individual to get a clearer image.
A lumbar puncture, or spinal tap, may also be necessary in cases of ependymoma. A doctor will insert a needle to take a small sample of cerebral spinal fluid from the lower back to inspect it for tumor cells.
Many cases of ependymomas will require a neurological exam. Doctors will ask a series of questions and administer tests designed to check the brain and a person's nerve function.
Diagnosing ependymoma can take time, but every step is important. A thorough diagnosis helps doctors find the best way to treat this type of cancer.
Radiotherapy can be used to destroy any cancer cells not removed by surgery.
In cases where the tumor is very small and not causing any immediate danger, doctors may suggest waiting and monitoring it for progression. Otherwise, the first step in active treatment is usually to remove as much of the tumor as possible.
The entire tumor can be removed in certain instances, while others may be in more delicate areas that allow only part of it to be removed.
Doctors usually recommend radiation therapy to help get rid of any lingering tumor cells after surgery. The use of chemotherapy may also help with some types of ependymoma, though its usefulness can vary.
People will be observed closely during treatment to monitor any changes that come about, so that alterations to treatment can be made, if necessary.
New clinical trials are currently underway to develop different treatment methods for ependymomas, and doctors may recommend one of these if they think it might help. It is important to remember that the choice to participate in clinical trials is always up to the individual.
Doctors may prescribe medications or recommend certain therapies to help manage symptoms of both the tumor and the cancer treatments, the latter of which can have adverse side effects.
Long-term or late side effects from ependymoma or surgery may appear in some cases. These side effects may develop months or years after surgery and can occur anywhere in the body.
Physical long-term effects can include heart problems, lung problems, and secondary cancers. Other long-term effects might include trouble with memory or thinking, anxiety, depression, or learning difficulties.
Doctors will want to monitor a person regularly to check for any lasting effects. Additional tests or exams may be needed to detect certain issues.
Some people may need different forms of rehabilitation, such as physical therapy, hearing assistance, or cognitive therapy. Children may also experience developmental issues and hormone irregularities.
It may be helpful for an individual who has been treated for ependymoma cancer to keep a personal health record to note any new symptoms. These can then be reported to a doctor to help them prescribe any further treatments.
The outlook for ependymoma depends on many individual factors. The type, grade, and location of the tumor can affect how easily it is removed.
Individual outlook also depends on the age and health of the person being treated, as well as whether any cancer cells still remain after surgery. Recurring tumors will also have a different outlook than tumors that do not come back.
According to statistics provided by the Central Brain Tumor Registry of the United States (CBTRUS), the overall 5-year relative survival rate for cases of ependymoma is 83.4 percent.