A particular kind of tumor called an ependymoma can develop in the brain or spinal cord. The ependymal cells that line the pathways in the brain and spinal cord where the fluid (cerebrospinal fluid) that nourishes your brain flows are where ependymoma develops.

Although it can happen to anybody, ependymoma most frequently affects young children. Ependymoma in children can cause headaches and seizures. Adult-onset ependymoma is more likely to develop in the spinal cord and can result in weakness in the area of the body that is controlled by the nerves that are damaged by the tumor.

The main form of therapy for ependymoma is surgery. Further therapies, such as radiation therapy or chemotherapy, may be advised for more aggressive cancers or tumors that cannot be eliminated during surgery.

Ependymoma: Causes

Although ependymomas that develop in various parts of the Brain are histologically identical, their clinical behavior frequently differs. Thus, ependymomas with identical histological grades may have very diverse clinical outcomes even when they have similar histological grades. Retrospective investigations have shown that histological grading alone has not consistently and reliably predicted survival outcomes.

Current research suggests that these tumors may be divided into separate progenitor cell populations, which would explain why tumors of the same histological grade might have diverse clinical outcomes. Large genomic areas are affected by several genetic disorders that have been identified to be associated with ependymoma. According to several of these investigations, ependymomas are associated with specific carcinogenic substances and molecular subgroups that may be more reliably associated with clinical outcomes than just histologic categorization.

Ependymoma: Symptoms

Ependymomas can manifest clinically in a variety of ways, ranging from an abrupt increase in intracranial pressure that causes nausea and vomiting to a more chronic and sneaky clinical course. Ependymoma is a histological diagnostic that most frequently manifests in the supratentorial, spinal cord, and infratentorial regions of the body.

When an adult patient has a non-enhancing, clearly defined intraventricular lesion on brain imaging, isodense on computed tomography, and isointense on T1 images on magnetic resonance imaging, ependymoma is frequently suspected (MRI).

Long-term brain tumor survivors may experience a wide range of consequences, including secondary cancers, endocrine and growth abnormalities, sensorineural hearing loss, neurological impairments, and cognitive difficulties. Long-term effects, most frequently weariness, numbness and tingling, discomfort, and disrupted sleep, can occur in adult patients.

Ependymoma: Risk Factors

Several factors play an important role in the development of ependymoma which includes −

Ependymomas are mostly sporadic, not familial, however, they can occur in people with the uncommon genetic disorder neurofibromatosis type 2. Moreover, ependymoma development has been linked to anomalies on chromosome 22.

Ependymoma: Diagnosis

The diagnosis of the ependymoma is mainly done based on history and some of the tests may be required for confirmation and to rule out underlying causes −

  • Neurologic examination. Your doctor will inquire about your child's signs and symptoms during a neurological examination. He or she could assess your child's reflexes, balance, strength, coordination, vision, and hearing. Issues in one or more of these areas might reveal information about the region of your child's brain that a brain tumor might be affecting.

  • Image-based exams. Doctors can use imaging studies to pinpoint the exact location and size of the brain tumor. Brain tumors are frequently diagnosed with MRI, which may also be combined with specialist MRI imaging techniques like magnetic resonance angiography. When ependymoma is suspected, imaging procedures should be utilized to get images of the brain and spine since ependymoma can develop in either location.

  • Cerebrospinal fluid removal for testing (lumbar puncture). In this technique, which is also known as a spinal tap, a needle is inserted between two lower spine bones to drain fluid from surrounding the spinal cord. To check for tumor cells or other anomalies, the fluid is examined.

The doctor may suspect ependymoma based on your child's test findings and advise surgery to remove the tumor. The tumor cells will be taken out and examined in a lab to confirm the diagnosis. The sorts of cells and their level of aggression are identified using specialized testing, which the doctor may utilize to inform therapy choices.

Ependymoma: Treatment

The treatment is based on the severity of the symptoms. Your doctor may advise conservative or surgical treatment.

Ependymoma Removal Surgery

Neurosurgeons want to remove as much of the ependymoma as is practical. Although it is ideal to remove the entire tumor, there are instances where doing so would be too dangerous due to the ependymoma's proximity to delicate brain or spinal tissue.

Your child might not need any further treatment if the surgeon can remove the entire tumor during surgery. The neurosurgeon may advise a further procedure to try to remove the remaining tumor if part of it is still there. For more aggressive cancers or in cases when the entire tumor cannot be removed, other therapies, such as radiation therapy, may be advised.

Radiation Treatment

High-energy beams, such as X-rays or protons, are used in radiation treatment to destroy cancer cells. Your kid will lie on a table during radiation therapy as a machine travels all around them, aiming beams at specific areas of the brain. After surgery, radiation therapy could be advised to assist prevent the recurrence of more aggressive tumors or if neurosurgeons weren't able to completely remove the tumor.

Using specialized methods can guarantee that the radiation therapy targets the tumor cells while sparing as much of the surrounding healthy tissue as feasible. Radiation treatment techniques including conformal radiation therapy, intensity-modulated radiation therapy, and proton therapy enable medical professionals to deliver radiation with care and accuracy.


Stereotactic radiosurgery, which is technically a form of radiation rather than an operation, concentrates several radiation beams on specific locations to destroy the tumor cells. When ependymoma returns after surgery and radiation, radiosurgery may be employed.


Drugs are used in chemotherapy to destroy cancer cells. For the majority of ependymoma instances, chemotherapy is not particularly successful.

Ependymoma: Prevention

Sadly, ependymomas cannot be avoided. Avoiding environmental risks like smoking and excessive radiation exposure can lower your chance of getting a brain tumor.


Patients should get prognosis-related counseling. Palliative care helps those with CNS tumors who are towards the end of their lives. Early and proactive counseling will be helpful for patients and their families, in addition to comfort measures. To address the diverse range of requirements that these patients will have, palliative care should be interdisciplinary.

An interprofessional strategy that may involve surgery, radiation therapy, and chemotherapy is the cornerstone of ependymoma treatment. Neurosurgery must be consulted to confirm the tumor by tissue biopsy or tumor excision treatment. Patients who are nearing the end of their lives may require palliative care. Surgery patients may potentially experience a variety of neurological impairments. These people could benefit from occupational, speech, and physical therapy. Neurological impairments are frequently long-lasting.

Dr. Durgesh Kumar Sinha
Dr. Durgesh Kumar Sinha


Updated on: 21-Apr-2023


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