Ependymoma: Symptoms, Causes, Diagnosis and Treatment

Written by Medicover Team and Medically Reviewed by Dr Krishna Haskar Dhanyamraju , Neurologists

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1. Causes 2. Find a Doctor 3. Diagnosis 4. Treatment 5. FAQs

Ependymoma is a rare tumor of the central nervous system (CNS) that develops from ependymal cells, which line the fluid-filled ventricles of the brain and the central canal of the spinal cord. It can occur in both children and adults, with tumors arising in the brain being more common in children and spinal cord tumors more common in adults. Symptoms depend on the tumor's location and may include headaches, nausea, vomiting, balance problems, weakness, seizures, or changes in bowel or bladder function. Early diagnosis and prompt treatment, typically involving surgery with or without radiation therapy, are essential to preserve neurological function, reduce the risk of recurrence, and improve long-term outcomes.

What are the Types of Ependymoma?

Ependymomas are categorized based on their location, histological features, and aggressiveness. The World Health Organization (WHO) classifies ependymomas into several grades:

Grade I (Subependymoma and Myxopapillary Ependymoma): These are slow-growing tumors often found in the spinal cord.
Grade II (Classic Ependymoma): These tumors can occur in both the brain and spinal cord and have a relatively moderate growth rate.
Grade III (Anaplastic Ependymoma): These are high-grade, aggressive tumors with a higher likelihood of recurrence and metastasis.

What are the Symptoms of Ependymoma?

The symptoms of ependymoma vary depending on the tumor's location and size. Common symptoms include:

Intracranial Ependymomas

Headaches: Often worse in the morning due to increased intracranial pressure.
Nausea and Vomiting: Caused by increased pressure within the skull.
Balance and Coordination Issues: Tumors in the posterior fossa can affect balance and coordination.
Seizures: Tumors in the cerebral hemispheres may lead to seizures.
Changes in Vision or Hearing: Depending on the tumor's proximity to cranial nerves.

Spinal Ependymomas

Back Pain: Persistent pain that may radiate to other parts of the body.
Weakness or Numbness: In the arms or legs, depending on the tumor's location.
Bladder or Bowel Dysfunction: Tumors in the lower spinal cord can affect autonomic functions.

What are the Common Causes and Risk Factors of Ependymoma?

The exact cause of Ependymoma is unknown. It develops due to genetic changes in ependymal cells that cause uncontrolled growth. Most cases occur sporadically, although rare inherited conditions may increase the risk.

There are no proven methods to prevent Ependymoma, but prompt evaluation of persistent neurological symptoms can help with early diagnosis and treatment.

Causes

Genetic mutations affecting ependymal cells
Abnormal cell growth within the central nervous system
Unknown underlying cause in most cases

Risk Factors

Childhood age for intracranial ependymomas
Adult age for spinal ependymomas
Neurofibromatosis Type 2 (NF2)
Rare inherited genetic disorders affecting the nervous system

When to See a Doctor for Ependymoma?

Persistent headaches, repeated vomiting, unexplained balance problems, seizures, back pain with weakness, or changes in bladder or bowel function should be evaluated promptly by a Neurosurgeon or Neurologist.

You should see a doctor if you experience:

Persistent headaches that worsen over time
Progressive weakness or numbness in the arms or legs
Difficulty walking or maintaining balance

Seek emergency medical attention immediately if you:

Develop seizures or lose consciousness
Experience sudden paralysis or inability to walk
Have severe headache with repeated vomiting, confusion, or vision changes

These symptoms may indicate increased pressure within the brain or spinal cord compression requiring urgent medical treatment.

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How is Ependymoma Diagnosed?

Diagnosing ependymoma involves a combination of imaging studies, neurological examinations, and biopsy procedures.

Imaging Techniques

Magnetic Resonance Imaging (MRI): The primary imaging modality for diagnosing ependymomas, providing detailed images of the brain and spinal cord.
Computed Tomography (CT) Scan: Often used in emergency settings to quickly assess brain structures.
Positron Emission Tomography (PET) Scan: Occasionally used to evaluate metabolic activity and differentiate between tumor types.

Biopsy and Histopathological Examination

A definitive diagnosis is made through a biopsy, where a tissue sample is examined under a microscope. Histopathological analysis determines the tumor's grade and guides treatment decisions.

What are the Treatment Options for Ependymoma?

Treatment strategies for ependymoma depend on factors such as the tumor's location, grade, and the patient's overall health. The primary treatment modalities include:

Surgical Resection

Surgical removal of the tumor is the first-line treatment for ependymoma. The goal is to achieve a complete resection while preserving neurological function. However, complete removal may not always be possible, especially in tumors located in critical areas of the brain or spinal cord.

Radiation Therapy

Post-operative radiation therapy is often recommended to target residual tumor cells and reduce the risk of recurrence. Advanced techniques such as conformal radiation therapy and proton therapy allow precise targeting of the tumor while minimizing damage to surrounding healthy tissue.

Chemotherapy

Chemotherapy is less commonly used for ependymomas but may be considered in certain cases, particularly for high-grade tumors or when surgical resection and radiation therapy are insufficient.

Clinical Trials

Participation in clinical trials may provide access to novel therapies and advanced treatment options. Patients should discuss potential eligibility for clinical trials with their healthcare team.

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What is the Recovery Process for Ependymoma?

Recovery depends on the tumor location, grade, extent of surgical removal, and response to additional therapies. Many individuals recover well after treatment, although some may require rehabilitation to regain neurological function. Lifelong follow-up may be necessary to detect recurrence and manage treatment-related effects.

Following the prescribed treatment plan, attending regular neurosurgical and oncology follow-up appointments, participating in rehabilitation, maintaining a healthy lifestyle, and reporting new neurological symptoms promptly help support long-term recovery and quality of life.