What is Ependymoma: Types, Causes, Symptoms, Treatment, Prognosis

Ependymoma is a comparatively rare type of tumor of the central nervous system (CNS) including the brain and the spinal cord. It can occur in both adults and children at any age, but in adults the frequency of this disease is more visible than in children. Experts consider ependymoma as a primary tumor, which means that it primarily occurs either in the brain or in the spine. In the central nervous system, neurons and glial cells are commonly found. There are three basic subtypes of glial cells found known as astrocytes, oligodendrocytes, and ependymal cells. Neurons generally remain unaffected by any kind of lesions. On the other hand, glial cells, particularly ependymal cells are vulnerable to abnormal cell formation, and the formation of the tumors called, ependymoma.

Following are the different types of ependymoma:

• Subependymomas: These are grade I ependymoma. These tumors grow slowly.
• Myxopapillary Ependymomas: These are also grade I tumors, and slow-growing tumors.
• Ependymomas: These are typically considered as grade II tumors. This is the most common ependymal tumor. Ependymomas is again subdivided into following subtypes:
  • Cellular ependymomas.
  • Papillary ependymomas.
  • Clear cell ependymomas.
  • Tanycytic ependymomas.
• Anaplastic Ependymomas: These are grade III ependymomas. They are faster-growing tumors.

Particular causes of ependymoma are yet to be discovered. Experts opinion is that certain damages in the brain or in the central nervous system or any deficiency in the system may cause ependymoma. Below are the mostly found causes of ependymomas till now:

• Abnormality in the blood vessels directed towards the central nervous system.
• Brain tumor.
• Stroke.
• Deteriorating neurological disorders.
• Disorders of a single nerve or group of nerves.
• Infection in the central nervous system.
• Cerebral palsy.
• Injury due to accident.

The signs and symptoms linked with ependymomas vary according to the age of the patients and the position of the lesion.

Some really visible symptoms of ependymomas may include the following:

• Ependymoma with Lesion in the Fourth Ventricle: The symptoms of this type of ependymomas include progressive lethargy, headache, nausea, and vomiting. Multiple cranial nerve palsies and cerebellar dysfunction are also visible. In children, this may lead to enlarged head circumference.
• Supratentorial Ependymomas: In this type of ependymoma, intracranial pressure increases leading to headache, nausea, vomiting, visual disturbance, aphasia (a communication disorder), hemiparesis (weakness in the left or right side of the body), and cognitive impairment. Further some changes in the cognitive functioning like changes in personality, mood, concentration, and some specific focal neurologic problems may also occur.
• Spinal Ependymomas: In spinal ependymomas several progressive neurologic deficits may occur.
• Infratentorial Ependymomas: Some symptoms in type of ependymomas are papilledema ataxia, nystagmus, etc.
• Spinal Ependymoma Tumors in the Upper Cervical Cord: Occipital or cervical pain and/or paresthesia, neck stiffness along with weakness and wasting of neck muscles are present. The other symptoms include spastic tetraplegia (spasticity of the limbs) or hemiplegia and weakness in the ventrolateral region below the lesion, and altered cutaneous sensitivity below the lesion, etc. are some of the symptoms of ependymoma occurring in the upper cervical spinal cord.
• Thoracic Ependymomas: These tumors are normally spotted by the sensory examinations. Abdominal skin reflexes lessen remarkably below the lesion.
• Lumbar Ependymomas: Lumbar ependymoma are lesions that are located from the root level of sensory loss as well as motor weakness. The symptoms include the following:
  • Radicular pain (this is a pain that spreads into the lower extremity directly along the path of a spinal nerve root).
  • Loss of cremasteric reflex (this is the reflex of the testicles in response to the patting or stroking of the thighs).
  • Loss of abdominal reflexes and increased sensation and jerks in the knee and ankle.
  • If the lesions affect the 3rd and the 4th lumbar segments, loss of patellar reflexes and oversensitive Achilles reflexes may be noticed.
  • If the occurrence is in the lumbar ependymoma, the roots of the cauda equine are affected wherein flaccid paralysis of the legs may occur.
  • If both the spinal cord and cauda equine are affected simultaneously, spastic paralysis of one leg along with ipsilateral ankle reflexes and flaccid paralysis of one leg with the loss of contralateral reflexes may occur.
• Myxopapillary Ependymomas of the Conus and Cauda Equina: Patients with this type of ependymomas may feel pain in the lower parts of the legs, rectal area, and back. Symmetric motor dysfunction will be visible for conus lesions and the same will be asymmetric for cauda equina lesions. On the other hand, autonomic dysfunction will be marked at the early stages in conus lesions, whereas the same will be found in advanced stages in case of cauda equina lesions.

In children, ependymomas is one of the most detected forms of brain and spinal cord tumor. Though the complete data are not recorded in all countries, as per the available data, almost 5% tumors in infants and children (up to 14 years) are found to be of this nature. While in the teenagers within the age group of 15-19 years, 4% of all brain and spinal cord tumors are detected to be ependymoma. In case of adults, above the age of 20 years, approximately 2% of tumors are diagnosed as ependymoma.

Experts opine, as also several studies have shown that an ependymoma develops from precursor cells to the ependymal cells. The ependymal cells are found in the ventricles. These are the spaces filled with fluid in the brain, and the central canal of the spinal cord. The tumor can occur anywhere where ependymal cells are visible.

No particular diagnosis process is available for ependymoma. However, the following examinations are undertaken to ensure the presence and location of lesions:

• CT Scan and MRI.
• EEG.
• Lumbar Puncture to collect cerebrospinal fluid for analysis.

Surgery is the foremost step in the treatment and management of ependymomas. Through the surgical process (Gross Total Resection) the tumor is removed to the maximum possible extent and then regulated radiation is given for a certain period. It is followed with medications as required.

Some cases of ependymoma may be recurrent in nature. In such cases, if the patient has already received radiation therapy, chemotherapy may be advised depending on the severity of the situations.

Any person at any age can be detected with ependymoma. The disease shows different symptoms depending on the location and type of ependymomas. There is no specific process of detecting the disease; however, the doctors depend on the symptoms and certain examinations like CT Scan and MRI to confirm the presence of ependymoma. There is no way to cure ependymomas except surgical resection, followed by radiotherapy and medications. Through proper treatment, a patient with ependymoma can be completely cured and lead a healthy life.