The ependymoma is a rare tumor in the brain or spinal cord. Here you will learn everything about its creation, treatment & more!


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.

ICD codes for this disease: ICD codes are internationally valid medical diagnosis codes. They are found e.g. in doctor's letters or on incapacity certificates. D43C71D33

Product Overview


  • Ependymoma: description

  • Ependymoma: symptoms

  • Ependymoma: causes and risk factors

  • Ependymoma: examinations and diagnosis

  • Ependymoma: treatment

  • Examination and treatment

  • Ependymoma: disease course and prognosis

Ependymoma: description

Ependymoma is a tumor that can form in both the brain and spinal cord. Mostly children are affected in the first ten years of life. The 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 clad the inner wall of the ventricles and the spinal canal, where the cerebrospinal fluid (CSF) 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 predict that about two percent of all ependymomas form metastases, others say about 30 percent. If the ependymoma returns after a successful therapy (recurrence), 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 bulges into the inner brain cavities 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 because it grows into the adjacent tissue - unlike the tumor types of the 1st and 2nd degree, which are usually well demarcated 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 is no longer flowing 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 drowsy 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, looking, 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 has 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 disorder 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 to a specialized center for cancer (oncology) for further investigation. Doctors from different disciplines work together to make the right diagnosis. For this, various technical investigations are usually necessary.

An ependymoma can best be visualized in magnetic resonance imaging (MRI). In most cases, 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.

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Ependymoma: treatment

The first therapeutic step for 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. For ependymoma, therapy with cortisone preparations is also recommended. They allow the brain tissue to subside and thereby reduce existing intracranial pressure.

Read more about the investigations

  • MRI
  • lumbar puncture
  • Neurological examination
  • perimetry

Examination and treatment

Further information on examinations and therapy can be found in the article Brain Tumor.

Read more about the therapies

  • craniotomy

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 completely removed 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 post-irradiation, the 10-year survival rate is only 30 to 40 percent.

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