The term ependymoma refers to a relatively rare tumor that occurs in the central nervous system. Ependymoma is one of the solid tumors, which occur due to cell degeneration in the brain or even in the spinal cord.
What is an ependymoma?
Because there are different ependymomas (which are all malignant), some tumors may grow rather quickly, while others may grow rather slowly. Due to the fact that there is only limited space in the skull for growing tissue, ependymoma can have fatal consequences in the course of the disease, as vital brain regions are affected. Just ten percent of all tumors that occur in the central nervous system are ependymomas, so that this type of tumor can be classified as relatively rare. Children and adolescents are primarily affected.
Causes
To date, there are no exact causes or mechanisms of development for why an ependymoma forms. However, physicians have sometimes concluded that radiation treatments in childhood – such as for malignant eye tumors or leukemia – may well promote the development of an ependymoma.
Symptoms, complaints, and signs
Symptoms and signs depend on the localization as well as spread of the tumor. Physicians distinguish between general and local or nonspecific and specific signs. General symptoms occur independent of localization and do not necessarily have anything to do with the ependymoma. Classic non-specific symptoms include back pain or headaches, loss of appetite, weight loss, dizziness, fatigue, morning vomiting, impaired concentration, and also a change in character and sometimes a delay in development. Specific or local symptoms may include gait or balance disorders if the tumor is located in the region of the cerebellum. On the other hand, if seizures occur repeatedly, the tumor may be present in the spinal cord region. Any sleep, consciousness or visual disturbances may also indicate where or where not the tumor is definitely located.
Diagnosis
If the physician – based on the medical history and physical examinations – expresses the suspicion that it could sometimes be a tumor in the central nervous system, the patient is subsequently admitted to the hospital as an inpatient. Institutions that specialize primarily in childhood cancers are chosen; these include clinics for pediatric oncology or hematology. It is important to note that only in the case of a mere suspicion, it is mainly specialists from various disciplines who are consulted when it comes to the final diagnosis and localization of the tumor. It is necessary to diagnose not only the diagnosis but also the localization and shape in case of ependymoma; those factors contribute a significant part to the treatment. The specialists perform physical as well as neurological examinations; imaging techniques (computer tomography as well as magnetic resonance imaging) are also used so that the physicians can determine whether there is a tumor on the one hand, and on the other hand, where it is located and whether metastases have already appeared that have spread to the spinal canal or to the brain. The imaging techniques also help to determine how large the tumor already is. However, in order for the diagnosis to be definitively confirmed, a tissue sample must be taken and subsequently examined for fine tissue. The prognosis depends primarily on the stage at which the diagnosis was made. If the ependymoma can be completely removed, the five-year survival rate is just under 70 percent and about 60 percent when it comes to the ten-year survival rate. However, if the tumor cannot be completely removed or if subsequent radiation helps, the 10-year survival rate is about 35 percent.
Complications
The complications and further course of ependymoma depend largely on the spread of the tumor and the particular site affected. In most cases, the usual symptoms that occur with cancer occur. The patient suffers mainly from loss of appetite, headache and severe weight loss. Often there is fatigue that cannot be compensated by sleep.The patient is exhausted and complains of a general feeling of weakness. Vomiting and diarrhea may occur, and concentration also decreases. The affected person is severely restricted in his daily life by the symptoms and can usually no longer perform his usual activities. If the cancer is widespread, visual disturbances or balance disorders may also occur. Since the cancer spreads mainly in the area of the back, there is increased pain and seizures. Treatment takes place surgically and is aimed primarily at removing the tumor. The earlier the treatment is started, the fewer complications occur. In some cases, multiple surgical procedures are also necessary to completely remove the tumor. The surgery is followed by chemotherapy. If the treatment is successful, there should be no further complications.
When should you see a doctor?
With recurrent seizures, dizziness, back pain, headache, and other symptoms of ependymon, the primary care physician should be consulted. Gait and balance problems, as well as disturbances of consciousness, sleep, or vision, also need to be clarified before further complications develop. Should serious complaints develop, a visit to the hospital is indicated. In the event of severe convulsions or accidents as a result of a seizure, it is best to alert the emergency services directly. An ependymon must be diagnosed and treated in any case, because if left untreated, the tumor causes increasingly severe symptoms and can even lead to death in the worst case. Therefore: Consult a doctor already at the first signs. If the tumor has been diagnosed, surgical treatment is usually carried out immediately. If the ependymon has already caused personality changes, therapeutic measures may also have to be taken. Close monitoring by a physician is required in any case after treatment. In addition, regular check-ups are necessary to detect and treat any recurrences at an early stage.
Treatment and therapy
Due to the fact that pediatric oncology facilities employ expert nurses and specialized physicians, treatment should definitely be carried out in such a facility. This is the only way to be sure that not only the best treatment is given to the patient, but also that relatives are gently introduced to the subject. Furthermore, such facilities are also known for being able to combat ependymoma with the latest therapeutic and procedural methods. In almost all cases, ependymoma is treated surgically; doctors refer to this as tumor resection or tumor removal. In recent years, tumor resection has impressively shown that it can positively benefit the course of the disease and also significantly improve the prognosis (in terms of the five- or ten-year survival rate). However, if the ependymoma is diagnosed only at an advanced stage, so that only a partial resection is possible, not only the course of the disease worsens, but also the prognosis. The problem arises mainly when the ependymoma is located in the 4th ventricle of the brain or grows into the cerebellopontine angle; in these cases, only partial resection is possible. If the physician would decide for a complete removal, this could sometimes cause that vital (and healthy) brain tissue would be damaged. If complete removal of the tumor is not achieved during the first surgery, a second surgery may be scheduled down the road so that tumor resection can be achieved in the second attempt. Surgery is followed by radiation therapy; in some cases, chemotherapy may also be prescribed.
Outlook and prognosis
Ependymoma can have a very different outcome, depending on the cause and location of the tumor. If the tumor is localized in the superficial tissue, it can be completely removed in most cases. Further treatment measures are then not necessary. However, the patient must undergo comprehensive follow-up care so that any recurrences and other complications can be detected quickly. If the tumor could be completely removed, there is a 60 to 75 percent chance that the patient will survive for more than ten years. The prerequisite for this is that the disease does not progress.If the ependymoma cannot be completely removed, for example because it is located under a vital organ, there is a 30 to 40 percent chance that the patient will survive longer than ten years. The prospect of recovery can be improved by self-help measures as well as comprehensive follow-up care. Nevertheless, complications can always occur, which worsen the prognosis. In principle, however, an ependymoma offers a good prognosis. If the tumor has not yet spread, the prospect is good that the patient can live a long, symptom-free life.
Prevention
Preventive measures are not known to date. Due to the fact that no definitive causes that could trigger ependymoma have been found so far either, no preventive accents are possible so that ependymoma does not occur.
Follow-up
In most cases of ependymoma, the options for follow-up care are relatively limited. Here, the patient is primarily dependent on direct and medical treatment by a specialist to prevent further complications and further spread of the tumor. In general, therefore, follow-up care for an ependymoma is directed toward regular monitoring of the body for further tumors in order to detect and remove them at an early stage. In some cases, however, this disease also limits the life expectancy of the affected person, and treatment is not always possible either. In the case of an ependymoma, the affected person is also dependent on the help and support of friends and his or her own family. This can also prevent psychological complaints. In some cases, contact with other people affected by the ependymoma can also be useful. Since a complete treatment of the disease is not always possible, the affected persons are often dependent on the help of other people in their everyday life. Here, loving care in particular has a positive effect on the further course of the disease. The focus is also on early detection of ependymoma.
What you can do yourself
In any case, an ependymoma requires specialist treatment. The measures that parents of affected children can take themselves always depend on the individual circumstances of the tumor disease. In principle, the child must be informed about the disease. Any questions are best answered in consultation with a physician. Since those affected and their relatives usually experience great anxiety, therapeutic advice should also be sought. Attending a self-help group allows for exchange with other sufferers and can thus provide a better perspective on the disease and how to deal with it. If chemotherapy or radiation therapy has to be carried out, an individual therapy should be worked out together with a nutritionist and the doctor in charge. An adapted diet, physical exercise and other measures can support the treatment and improve well-being. After the treatment, the most important measures are regular check-ups. An ependymon can form recurrences, which must be detected and treated promptly to exclude a negative progression. If the tumor disease is already far advanced, further therapeutic measures are indicated. In the event of death, professionally guided grief management is needed.
