Brain ependymoma is a tumor from the ependymal tissue of the ventricular system of the brain. It is clinically manifested, first of all, by signs of increased intracranial pressure, as well as ataxia, visual and hearing disorders, convulsive seizures, speech and swallowing disorders. Diagnosis is carried out mainly with the help of MRI of the brain, spinal puncture, EEG, histological examination. The basis of treatment is radical removal of the ependymoma followed by radiation or chemotherapy. Stereotactic radiosurgery is used in adults.
ICD 10
C71.5 Malignant neoplasm of the brain (ventricles of the brain)
General information
Brain ependymoma is a CNS tumor, originating from ependymocytes. The latter are epithelial—like cells that form the ependyma – the thin epithelial lining of the cerebral ventricles and the central spinal canal. Ependymomas account for up to 8% of all brain tumors, belong to gliomas.
Ependymomas are more common in childhood, about 60% of patients are children of the first five years of life. According to its prevalence, brain ependymoma ranks 3rd among all cerebral tumors in children. In childhood, its malignant variants are observed more often than in adults. Treatment with ependym is an urgent problem of modern oncology, neurology and neurosurgery.
Causes
Studies have made it possible to detect the SV40 virus, which is in the active state of ependymal cells. However, its role in the development of neoplasms is still not clear. It is assumed that brain ependymoma occurs due to the effects of oncogenic factors common to most tumors: chemical carcinogens, radioactive radiation, long-term adverse environmental conditions (for example, hyperinsolation), oncogenic viruses (herpes virus, human papilloma, etc.).
In addition, it is impossible to exclude the participation of genetic factors in the development of ependyma. There are known combinations of cerebral ependyma with Recklinghausen’s neurofibromatosis, multiple endocrine neoplasia syndrome (MEN), familial colon polyposis.
Pathogenesis
Brain ependymoma is more often formed in the posterior cranial fossa. In many cases, it is characterized by slow growth, lack of germination into the surrounding cerebral tissues. However, as the neoplasm grows, it can cause a mass effect – compression of the brain. Ependymoma metastasis occurs mainly along the cerebrospinal fluid pathways and, as a rule, does not go beyond the central nervous system. Retrograde metastases to the spinal cord are most often noted.
Classification
According to the modern classification, there are 4 types of ependyma, differing in the degree of goodness and the nature of the course of the tumor process:
- Myxopapillary ependymoma is the most benign, localized mainly in the spinal cord in the area of the ponytail. It occurs mainly in adults.
- Subependymoma is characterized by slow benign growth, often asymptomatic course and relatively rare recurrence.
- Classical ependymoma is observed most often. It can cause occlusion of the cerebrospinal tract. It often recurs, and over time it can transform into anaplastic ependyma.
- Anaplastic ependymoma accounts for about a quarter of all ependymomas. It is characterized by accelerated growth and metastasis, for which it has received synonymous names: ependymoblastoma, malignant ependymoma, undifferentiated ependymoma.
Symptoms
Brain ependymoma manifests signs of intracranial hypertension: an increasing headache, a feeling of pressure in the eyeballs, nausea independent of food intake, vomiting. Along with this, many patients experience irritability, some changes in habitual behavior, ataxia — clumsiness and shakiness of walking, discoordination of movements, which makes it difficult, first of all, small actions with hands, including writing. In school-age children, there is a noticeable deterioration in the results of studies.
The above symptoms are a consequence of cerebrospinal hypertension syndrome and worsen as intracranial pressure increases. If an ependymoma becomes an obstacle to the circulation of cerebrospinal fluid and causes obturation of the cerebrospinal tract, obturation hydrocephalus develops. In the clinical picture, this is manifested by a sharp increase in the existing symptoms — the headache becomes very intense and is accompanied by repeated vomiting without relieving the condition. Convulsive seizures are possible.
The focal symptoms accompanying cerebral ependyma vary according to the location of the tumor. Tumors of the posterior cranial fossa are characterized by vestibular ataxia, double vision. If the brain ependymoma is located in the lower part of the IV ventricle, then hearing loss, dysarthria, cerebellar ataxia, dysphagia are possible.
Diagnostics
A pronounced cerebrospinal hypertension syndrome, the appearance of other neurological symptoms or a first-time epiprime are a reason for a neurologist to conduct a comprehensive examination of the patient. The range of ongoing research includes:
- MRI or CT scan of the brain. An MRI of the brain is preferable because it provides more complete information and avoids radiation exposure, especially when it comes to examining a child.
- NSG. For children under one year of age, the stage of primary diagnosis can be neurosonography, which is performed through a still unclosed fontanel.
- Cytomorphological diagnostics. To determine the prevalence of the process on the spinal canal, a lumbar puncture and examination of the cerebrospinal fluid are performed. The final verification of the diagnosis is possible only after morphological examination of the ependymoma material obtained intraoperatively or by stereotactic biopsy.
- Additional methods. Electroencephalography, consultation of an ophthalmologist, direct ophthalmoscopy are performed.
Treatment
Neurosurgical operations
The optimal way to treat ependymoma is its total removal. The frequent location of the tumor in the posterior cranial fossa causes certain difficulties of access to it, which the neurosurgeon faces. If complete removal of the tumor is not possible, the operation may be palliative. In such cases, a bypass operation can be performed to improve the outflow of cerebrospinal fluid.
In adults, tumor destruction is possible by stereotactic radiosurgery. Recently, the “Cyber-knife” installation has been increasingly used, which allows achieving a large total dose of ependymoma irradiation by converging numerous beams emanating from different points of the device on the tumor. However, due to the high radiation load, this method is not recommended for children under 14 years old.
Antitumor therapy
In order to reduce the likelihood of recurrence of cerebral ependymoma after surgical treatment, radiation therapy is performed. In young children whose brains are particularly susceptible to radioactive radiation, radiation therapy should be reduced or replaced with chemotherapy. Treatment with cytostatics (cisplatin, carboplatin) can be carried out after radiation therapy or instead of it. Its volume depends on the nature and prevalence of epindymoma.
Forecast
Mixopapillary ependymoma and subepindymoma after their radical removal usually have a favorable prognosis. In the case of a classical ependymoma, complete removal is not always possible. The neoplasm recurs, as a rule, at the place of its initial appearance. With successful removal, the 5-year survival rate is estimated at 67-80%.
Anaplastic ependymoma has the most negative prognosis, which is associated with its rapid spread through the cerebrospinal tract. It should be noted that even after successful treatment, patients may subsequently retain various neurological symptoms, hearing loss, visual impairment; in children, mental retardation is possible.