 Ependymoma
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An ependymoma is a tumor that arises from cells of the ependymal lining of the ventricular system of brain or spinal cord. The ventricular system contains cerebrospinal fluid (CSF).
The average age at diagnosis is 5 years old, and 25-40 percent of patients are less than 2 years old. Ependymomas occur equally in males and females.
Ependymomas account for 5-10 percent of pediatric brain tumors. Approximately 60 percent are located in the region of the cerebellum and brain stem known as the posterior fossa, 10 percent are located in the spinal cord, and the remaining 30 percent in other parts of the brain. Ependymomas account for 25 percent of all spinal cord tumors. Most patients with tumors of the spinal cord are older than 12 years.
As you read further below, you will find general information about ependymomas. If you would like to view summary information about brain tumors first, see the overview on brain tumors.
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Symptoms vary depending on size and location of tumor. Tumor can block the normal flow of CSF, causing increased pressure on the brain (hydrocephalus) and enlargement of the skull and a variety of symptoms. Common symptoms may include:
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- headaches ( especially upon awakening)
- nausea and vomiting (especially upon awakening)
- lethargy and irritability
- problems feeding or walking
- enlarged head size or fontanels
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The symptoms of a brain tumor may resemble other conditions or medical problems - ranging from the simple to the serious. Always consult your child's physician for diagnosis and treatment.
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Microscopically, ependymomas can appear different, called either standard or anaplastic ependymoma. These two types are treated the same and have approximately the same prognosis.
A third type, which occurs at the base of the spine, called myxopapillary cauda equina ependymoma, tends to be much less malignant than any of the other forms. A fourth type, called ependymoblastoma, is a more aggressive form of this tumor and more difficult to treat. Its diagnosis and management are similar to that of primitive neuroectodermal tumor (PNET) and is discussed in the section on PNET.
Ependymomas can metastasize, meaning they can spread into adjacent areas of the brain or, less commonly, to distant parts of the central nervous system. Approximately 12 percent of patients will have evidence of spread of disease at diagnosis. In these situations, more extensive treatment is used to cure the disease.
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Diagnostic procedures for an ependymoma, like other brain tumors, are necessary to determine the exact type of tumor and whether the tumor has spread. These may include:
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- physical exam - your child's physicians can test neurologic function including: reflexes, muscle strength, eye and mouth movement, coordination, and alertness.
- scans - magnetic resonance imaging (MRI) scans are obtained using computerized, large magnetic fields to produce detailed images of organs and structures within the body. This will provide information on the location, size and characteristics of the tumor. The magnetic fields are not harmful and are safer than even routine x-rays. Computerized tomography (CT) or CAT scans using x-rays are also occasionally used.
- biopsy - a tissue sample from the tumor taken during surgery to remove part or all of the tumor. The sample will provide definitive information about the type of tumor.
- lumbar puncture (spinal tap) - to remove a small sample of cerebrospinal fluid (CSF), a special needle is placed into the lower back, into the spinal canal, the area around the spinal cord. CSF is the fluid that bathes the brain and spinal cord. The sample is sent for testing to determine if any tumor cells have started to spread. In children, this procedure is safely performed under sedation, and is less difficult and less painful than placing an intravenous (IV) catheter.
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Specific treatment to cure ependymoma will be determined by your child's physician based on:
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- type, location, and size of the tumor
- extent of the disease
- your child's age, overall health, and medical history
- how your child's doctors expect the disease to progress
- your child's tolerance for specific medications, procedures, or therapies
- your preference and judgement
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Treatment approaches include:
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- surgery - usually the first step and mainstay in the treatment of brain tumors. The goal is to remove as much of the tumor as possible while preserving neurological function. Tumor specimens are examined by neuropathologists to determine the exact diagnosis. Complete resection or surgical removal of the entire tumor is ideal when this is feasible. However, tumor location and other characteristics may limit removal to a partial or sub-total resection. A biopsy is the surgical removal of a sample of the tumor for diagnostic purposes. This is frequently done if the tumor is in an area with sensitive structures around it that may be injured during removal. In general, the more complete the resection, the better the chances for cure.
- ventriculo-peritoneal shunt (VP shunt) - when a tumor causes blockage of CSF flow leading to symptoms, special tubing can be surgically implanted in the ventricles to drain excess CSF into the abdomen. This bypasses the tumor blockage and relieves symptoms of hydrocephalus.
- radiation therapy - precisely targeted and dosed radiation is used to kill cancer cells left behind after surgery. This therapy is important to control the local growth of tumor. If the tumor has spread, radiation to the whole brain and spinal cord is important to treat ependymoma.
- chemotherapy - the role of anti-tumor medications in treating ependymoma is actively being studied. New agents are under investigation.
- follow-up care - to manage disease complications, to screen for early recurrence of tumor and to manage late effects of treatment, regular visits with physical exams, laboratory testing, and scans are routine follow-up. In addition to neurosurgeons, radiation oncologists, pediatric oncologists and neurologists, the endocrinologists, neuro-psychologists, alternative/complementary therapy specialists, school liaison and psychosocial personnel continue to serve as members of the neuro-oncology team.
- rehabilitation - speech, physical, and occupational therapists are also part of the neuro-oncology team, and may be involved during and after treatments.
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Within the brain and posterior fossa, patients do best when their tumor can be completely removed surgically, and if high dose focal radiation therapy is given. For spinal cord ependymomas, a similar approach is used, but with lower radiation doses. A metastatic tumor (ependymoma that has spread) requires additional whole brain and spine radiation.
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Ependymoma of the brain treated with complete resection and radiation to the tumor area can result in an approximately 50 percent cure rate. Spinal cord ependymomas are often easier to treat, and have an excellent prognosis. Metastatic, progressive or recurrent ependymoma is more difficult to cure.
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Children's Hospital and Dana-Farber Cancer Institute are conducting numerous research studies that will help clinicians better understand and treat ependymoma. For more information on current research, see the Brain Tumor Program.
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