Brain and spinal cord tumors represent one of the most challenging areas of medicine, owing to their location and behavior. Among these tumors, anaplastic ependymoma demands particular attention — both for the difficulty of recognizing it and for the complexity of its treatment. Although rare, understanding it correctly and intervening early can be life-saving.

What Is the Difference Between Ependymoma and Anaplastic?

Among the cells lining the brain and spinal canal, there is a specialized group known as ependymal cells. These cells coat the inner surface of the ventricles — the cavities where cerebrospinal fluid (CSF) is produced and circulated. Tumors arising from these cells are collectively referred to as ependymomas.

When examined under a microscope, tumors are graded according to how “aggressive” they appear. The World Health Organization classifies them on a scale from Grade I to Grade IV. Anaplastic ependymoma is classified as Grade III, meaning the cells appear markedly abnormal, multiply rapidly, and tend to invade surrounding tissue. In short, the word “anaplastic” indicates that the tumor is high-grade and exhibits more aggressive behavior.

Where Does It Occur?

Anaplastic ependymoma can arise anywhere along the brain and spinal cord, but the most common locations include:

• The posterior fossa (around the cerebellum and the fourth ventricle): the most common site in children.
• The spinal canal: more frequently seen in adults.
• The cerebral hemispheres (upper regions of the brain): less common, but possible in adults.

Who Is at Risk?

Anaplastic ependymoma can occur at any age, but there are two distinct peaks. It is more common in childhood — particularly in children under five — where it represents a significant proportion of brain tumors in this age group. In adults, it most often presents in the thirties and forties.

No definitive environmental cause is known. However, genetic predisposition syndromes such as neurofibromatosis type 2, associated with NF2 gene mutation, are recognized as risk factors for the development of ependymoma.

What Are the Symptoms?

Symptoms vary considerably depending on the location of the tumor. That said, the most frequently encountered findings include:

• Headache that is more pronounced in the morning and worsens when bending forward
• Nausea and vomiting
• Balance problems and difficulty walking
• Visual disturbances or double vision
• Epileptic seizures
• Numbness or weakness in the arms or legs
• Loss of bladder and bowel control (in cases involving the spinal cord)
• In children: enlargement of head circumference, irritability, and developmental delay

The insidious onset of these symptoms and how easily they can be mistaken for other conditions may delay diagnosis.

How Is It Diagnosed?

The diagnosis of anaplastic ependymoma is established through a multi-step process.

Imaging: MRI (magnetic resonance imaging) is the gold standard for identifying the tumor’s location, size, and relationship to surrounding structures. Contrast-enhanced scans provide sharper delineation of tumor margins.

Surgery and Biopsy: A definitive diagnosis is only possible through pathological examination of tumor tissue. A sample obtained by the surgeon is evaluated under a microscope by a neuropathologist to determine the cell type and grade.

Molecular Analysis: Today, genetic and molecular studies of tumor tissue are also performed. Findings such as 1p/19q codeletion, TERT promoter mutation, and CDKN2A/B deletion not only refine the diagnosis but directly influence treatment planning.

What Are the Treatment Options?

The treatment of anaplastic ependymoma requires a multidisciplinary approach — neurosurgeon, radiation oncologist, and medical oncologist working together to reach a decision.

Surgery: Surgery is the first and most critical step in treatment. Removing the tumor as extensively as possible both relieves symptoms and enhances the effectiveness of subsequent therapies. The surgical goal is to achieve maximum tumor resection without causing damage to neurological structures.

Radiotherapy: Radiotherapy, applied as a standard following surgery, targets any remaining tumor cells. Focal radiotherapy or craniospinal irradiation is planned based on the extent of tumor spread.

Chemotherapy: The role of chemotherapy in anaplastic ependymoma is more limited compared to other high-grade tumors. In recurrent or refractory cases, agents such as temozolomide, carboplatin, or etoposide may be used.

Proton Therapy: Particularly in pediatric patients, proton therapy may be preferred to minimize radiation damage to healthy tissue. This method allows radiation to be directed at the tumor with far greater precision.

Prognosis: What Can Be Expected?

The course of anaplastic ependymoma varies considerably from patient to patient. The tumor’s location, the completeness of surgical resection, the patient’s age, and molecular characteristics are the primary factors determining prognosis.

In general, five-year survival rates range between 50 and 75 percent, though this figure varies significantly depending on whether the tumor was completely removed. Regular MRI follow-up after treatment is essential, as the tumor may recur — in which case additional treatment planning may be necessary.

Follow-Up and Quality of Life

Completing the treatment process is only the beginning. Long-term follow-up is critical not only for monitoring tumor recurrence but also for managing the potential late effects of treatment. Neurophysiological rehabilitation, neuropsychiatric support, and physical therapy where needed all play a decisive role in preserving the patient’s quality of life.

Many patients, with the support of a multidisciplinary team and a well-structured follow-up program, continue to lead productive and meaningful lives.

Conclusion: Knowledge Is the Most Powerful Weapon

Anaplastic ependymoma is a challenging diagnosis, given its complex nature and high-grade characteristics. Yet the advances medicine has made in this field — particularly in molecular classification and targeted therapies — paint an increasingly hopeful picture. Early diagnosis, surgical intervention at an experienced center, and a comprehensive treatment plan are the most important factors determining outcomes.

If you or a loved one has received this diagnosis, do not hesitate to seek out an experienced neuro-oncology team. Having the right information and the right team by your side is the most powerful step you can take on this journey.

Prof. Dr. Mehmet Şenoğlu Neurosurgeon, İzmir

Note: This article is intended for informational purposes only. Please consult a qualified physician for diagnosis and treatment.