Ependymoma - Treatment, Survival Rate, Symptoms, Causes, Diagnosis
What is Ependymoma?
Ependymoma is a brain or spinal cord tumor that originated from the cells that lines the ventricular system of the brain containing the cerebro spinal fluid. It is regarded as the third most common type of brain tumor among children although adults can also get affected. The difference however between children and adults is that, Ependymoma in children occurs in the intracranial while it occurs in the spinal in adults and older children. Ependymoma is defined as the tumor of the ependymal cells that is usually found on the fourth ventricle. The nature of Ependymoma in children is intracranial and rarely spinal.
The ependyma is composed of ependymal cells that form the lining of the ventricles of the brain. The cell is best seen in younger brain and which is usually cuboidal and surrounded by cilia. The ependyma is among the four types of neurolagia in the central nervous system and function together with other cells to produce the cerebrospinal fluid.
Ependymoma is seen in all parts of the world although Grades II and III are commonly seen among black individuals. It equally affects both male and female but is more common in children. The mean age of onset among children is at age 4 with a few percentages of case in children at age 2. Children are mostly seen with Intracranial Ependymoma while Spinal Ependymoma is common in 15 to 40 years age group.
The presentation of Ependymoma depends on the age, location and size of the tumor with the duration of symptoms that vary from 1 month to 3 years prior to diagnosis. Most patients however have symptoms that linger for 1 to 3 months.
A progressive lethargy, nausea, vomiting and headache are common symptoms in Ependymoma that arises in the fourth ventricle. The symptoms are secondary to the increase in intracranial pressure due to the impeding hydrocephalus. A cerebral dysfunction and multiple cranial-nerve palsies may occur if Ependymoma extends to the floor of the fourth ventricle.
An increase in the size of the head is the initial symptom of Ependymoma in babies. The increase in head size is secondary to the obstructive hydrocephalus where Ependymoma developed prior to the closure of the cranial sutures. As the tumor grows babies with Ependymoma may suffer from sleeplessness, vomiting and an increased irritability.
Other signs and symptoms of intracranial Ependymoma include the following:
- Visual loss
- Sensory loss
- Cognitive impairment
Spinal Ependymoma is common among adults and is rare in children. The signs and symptoms of Ependymoma depend on the location of the growth in the spine and these include the following:
- Pain in the cervical or occipital region
- Neck stiffness
- Wasting of muscles in the neck
- Spastic tetraplegia
- Paralysis of the flexors of the wrists and finger extensors
- Pain in the lower legs, rectal region and the lower back
- Paralysis of the legs
- Wasting of muscles in the legs
- Foot drop
Ependymoma is a type of brain tumor that belongs to the group of brain tumors known as gliomas. The glioma is a tumor that originates from glial cell that act as a supportive cell in the brain. Like any other type of brain tumor which is named after the cell from which it originated, Ependymoma is a brain tumor that develops from the ependymal cell that lines the ventricles of the spinal cord and the brain.
The tumor can be malignant or benign and usually develop when there is an existing change in the normal cells that uncontrollably grow. And like any other form of tumor, Ependymoma has no exact etiology or the exact cause of changes in the ependymal cells remains unknown.
Numerous genetic mutations have been considered to the onset of Ependymoma although the exact relationship between tumor growth and genetic mutations has remained vague and unidentified. No environmental cause has also been identified that can be directly implicated in Ependymoma.
Neurofibromatosis type 2 is a hereditary syndrome characterized by a development of tumor in the central nervous system although it is normally noncancerous. This syndrome is being linked with the development of Ependymoma yet Ependymoma seldom occurs in children with neurofibromatosis type 2.
The initial step in diagnosing Ependymoma is through the establishment of basis of diagnosis through gathering medical history of the patient including other pertinent information. Important factors are being considered to determine which type of diagnostic procedure is appropriate and will best identify or confirm suspicion.
Factors considered include the following:
- Age of the patient
- Overall health condition
- Medical history
- Signs and symptoms presented
- Type of tumor considered
- Cross reference to previous diagnostic test results
The physical examination involves testing the reflexes of the patient including the strength and sensory in the arms and the legs.
Additional test and other diagnostic procedure are required to further confirm the suspicion. A biopsy is usually recommended to give a definite conclusion. Lumbar puncture may also be done to look for tumor markers.
The imaging test is further required after confirmation of Ependymoma. The imaging test is to determine the exact position and size of the tumor and whether it has metastasized or not. This is also the basis for establishment of appropriate treatment necessary. Such imaging test may include the following:
Magnetic Resonance Imaging (MRI)
Magnetic resonance imaging is the most preferred diagnostic tool after microscopic examination of the tumor. An MRI through its magnetic fields provides detailed images which can clearly locate the tumor including its size.
Computed Tomography Scan
Computed tomography scan creates a three dimensional to give a detailed view. A CT scan can provide the size of the tumor.
The treatment of Ependymoma is reliable on the size, location and extent of the tumor growth if it has spread to the surrounding areas from its original. The overall health condition of the patient is also being considered prior to treatment program. The set of treatment is usually planned by a group of specialists who work together to formulate an appropriate treatment.
The pressure buildup in the skull is the primary concern prior to any treatment. A medication indicated for reducing the swelling in the brain is usually given to relieve the pressure. A pressure buildup due to the cerebro-spinal fluid is addressed with an insertion of a shunt to drain excessive fluid thereby relieving the pressure buildup.
Surgical removal of the tumor is usually the first line of treatment after which the multidisciplinary team will formulate a treatment plan based on the diagnosis after the tumor has been removed. The goal of the surgery is to eradicate as much tumor as possible. The tumor resection plays an important role in the prognosis and long-term survival of the patient with nonmalignant growths.
Medical management of Ependymoma includes the conventional radiotherapy, chemotherapy and radiosurgery. Anticonvulsant medication is also given for supratentorial Ependymoma to prevent seizures. Radiation therapy is especially recommended for adults and older children post surgery.
Spinal Ependymoma has a better prognosis compared with intracranial Ependymoma. Recent studies showed that pediatric patients cover about 60% of five year survival rate regardless of age, position of the tumor and grade of Ependymoma. Older children however have a better prognosis when the case is viewed independently.
The overall five-year survival rate among children accounts for roughly 55% for those who underwent surgery and radiotherapy. A high survival rate on the other hand can be achieved if the treatment program is carefully followed.
The five-year survival rate for adult patients with Ependymoma is a bit higher than in pediatric patients. Spinal Ependymoma is more common in older children and adults and seldom with intracranial Ependymoma. Spinal tumor is given a better prognosis than with intracranial Ependymoma. The prognosis however is worse for adult patients with Grade III Ependymoma. An improved prognosis is ideal in spinal tumors that has been removed totally through surgery and if there is no spread of tumor on the brain and other areas of the spine.