The ependymoma is a very rare tumor in the brain or spinal cord. It forms in the brain waterways, often causing increased intracranial pressure with headache and nausea. Most children are affected by the age of 6 years. The tumor is operated on or irradiated. The prognosis depends on various factors and varies greatly. Here you read everything important to an Ependymom.
Ependymoma: description
Ependymoma is a tumor that can form in both the brain and spinal cord. Mostly children are affected in their first ten years of life. Ependymoma accounts for ten percent of childhood brain tumors, making it the third most common tumor of the central nervous system in this age group. Every year about two in a million children contract an ependymoma. But even adults can develop this tumor, but much rarer: In them, the ependymoma makes up only two to three percent of all brain tumors.
An ependymoma forms from ependymal cells. These cover the inner wall of the ventricles and the spinal canal, where the cerebrospinal fluid (cerebrospinal fluid) is located. In principle, the tumor can form at any point of these inner walls. In children and also in all age groups, it develops preferentially in the 4th ventricle of the posterior fossa. From there, the ependymoma often spreads to the cerebellum, brainstem, or upper cervical cord. An ependymoma in the spinal canal is more common in adults.
Metastasis formation
In certain cases, individual tumor cells may secrete and pass through the brain waterways to another location such as the spinal cord. Grow at this new site and multiply there, it is called a metastasis. Some scientific studies estimate that about two percent of all ependymomas form metastases, others say about 30 percent. If the ependymoma returns after a successful therapy (relapse), it metastasizes in almost every second patient.
WHO classification
The World Health Organization (WHO) divides the ependymoma into different degrees of severity like all other brain tumors:
- Grade I: subependymoma and myxopapillary ependymoma
- Grade II: Ependymoma
- Grade III: Anaplastic ependymoma
The subependymoma is a benign tumor. It archs nodulously into the inner brain chambers and is often discovered only by chance.
The myxopapillary ependymoma is also benign. It grows only in the lowest part of the spinal canal and occurs in adults.
An anaplastic ependymoma is found mainly in children. It grows very fast and has a less good prognosis, as it grows into the adjacent tissue – in contrast to the tumor types of the 1st and 2nd degree, which are usually well differentiated from the healthy brain tissue.
Ependymoma: symptoms
Due to its spatial proximity, an ependymoma can very quickly move the cerebral waterways. But if the brain water does not drain properly, the pressure on the brain increases. This causes severe headaches, which can hardly be alleviated by medication. Most headaches occur at night or early in the morning and improve during the day. They return regularly and become more intense over days and weeks. If the intracranial pressure continues to rise, those affected are often sick. Some have to pass over. In extreme cases, they can become tired and sleepy or even fall into comatose states.
Especially in younger children, the head circumference can enlarge by an ependymoma (macrocephaly). In some patients, the tumor is noticeable by a seizure. In addition, discomfort may occur while walking, seeing, sleeping and concentrating. An ependymoma can also cause paralysis.
Ependymoma: causes and risk factors
Why an ependymoma arises is not yet fully understood. An increased risk of disease have people who were irradiated in the context of cancer therapy. Children are partially irradiated with leukemia (white blood cancer) or the malignant eye tumor retinoblastoma and develop a brain tumor more frequently even years after the radiation.
Ependymomas in the spinal cord are associated with the hereditary disease neurofibromatosis type 2.
Ependymoma: examinations and diagnosis
Patients with brain tumor symptoms often visit their family doctor or pediatrician first. This inquires after the exact complaints and their course, after possible pre and basic illnesses and the general health condition (collection of the medical history = anamnesis). If he finds evidence of a malignant tumor in the central nervous system, he usually transfers the patient for further investigation in a specialized center for cancer (oncology). Doctors from different disciplines work together to make the right diagnosis. This usually requires various technical examinations.
An ependymoma can best be visualized in magnetic resonance imaging (MRI). Most often, the patient is injected with a contrast agent into a vein before the examination. The tumor absorbs this contrast agent and illuminates irregularly bright in the MRI image. This allows you to determine its location, size and spread very well.
The brain water examination (CSF diagnostics) complements a detailed diagnosis of ependymoma. In this case, individual tumor cells can be detected. In addition, a tissue sample of the tumor is usually taken and examined under the microscope. This is necessary in order to optimally tailor the subsequent therapy to the patient.
Ependymoma: treatment
The first therapeutic step in an ependymoma is the most complete surgical removal of the tumor. In tumors in the spinal cord, this goal can often be achieved nowadays. In most cases, tumors in the brain can not be completely removed. If tumor remains in the body or if the tumor has already spread, this is followed by radiotherapy. In a second or third degree ependymoma, this is also done with complete tumor removal. This can reduce the risk that the tumor will recur. Chemotherapy is rarely used in studies of ependymoma. It is debatable whether it brings a decisive advantage.
Symptoms of the tumor such as headache or nausea are treated concomitantly with medication. An ependymoma is also a therapy with cortisone preparations. They allow the brain tissue to subside and thus reduce existing intracranial pressure.
Examination and treatment
Further information on examinations and therapy can be found in the article Brain Tumor.
Ependymoma: disease course and prognosis
The prognosis of an ependymoma depends on various factors and can therefore be quite different. The decisive factor is, among other things, where the tumor is located, whether it can be surgically removed completely and whether it has already spread.
For a complete removal with re-irradiation of the tumor region, 60 to 75 percent of the patients live five years later and 50 to 60 percent ten years later – prerequisite: The disease does not progress. Could that ependymoma can not be completely removed and those affected have received a re-irradiation, the 10-year survival rate is only 30 to 40 percent.