Ependymoma - Types, Causes, Symptoms, Diagnosis, and Treatment

Important Points:

1. Ependymoma is a rare tumor of the central nervous system (glial tumor).

2. It originates from the tissue lining the spinal cord and brain ventricles.

3. It usually does not spread.

4. This cancer affects children more often than adults.

5. This tumor is graded in 3 stages based on the severity.

6. Treatment includes surgical tumor removal with radiation therapy.

What Is Ependymoma?

Ependymoma is a rare type of cancer that originates in the brain or spinal cord. It can occur at any age but is more common in children. Doctors still do not know the exact cause of this cancer. The ependymal cells, which are the cells that line the spinal cord and the brain ventricles, divide uncontrollably and give rise to a tumor. Ependymomas are gray or red in color and soft. In some cases, they might contain calcifications or cysts within.

What Are the Types of Ependymoma?

Ependymoma can be divided into different types based on how closely the abnormal cancer cells resemble normal brain or spinal cord cells, their location, and aggressiveness. The types of ependymoma are:

1. Grade I - This type is usually slow-growing. It includes:

   1. Subependymomas - are commonly seen near a ventricle in the brain.

   2. Myxopapillary ependymomas - are commonly seen at the lower end of the spinal column.

2. Grade II - This is the most common type of ependymomas. They can be seen anywhere in the ventricles of the brain where cerebrospinal fluid (CSF) is produced. This type includes:

   1. Cellular ependymoma.

   2. Papillary ependymoma.

   3. Tanycytic ependymoma.

   4. RELA fusion-positive ependymoma.

   5. Clear cell ependymoma.

3. Grade III - They are a faster-growing type of ependymomas and are malignant. Otherwise called anaplastic ependymomas, they are commonly found in the skull, brain, brain stem, and rarely in the spinal cord.

This tumor does not usually spread, but in some cases, the tumor cells spread through the CSF and can form tumors in multiple areas of the brain and spinal cord. But this is very rare.

What Are the Causes of Ependymoma?

The exact cause of ependymoma is not known still.

Risk Factors:

• This tumor is more common in children than in adults.

• It is mostly diagnosed in infants.

• It is believed to be genetic or inherited as they tend to appear in families.

• Spinal ependymomas commonly affect people with neurofibromatosis type 2 (NF2), which is a condition that results in nonmalignant tumors in the nervous system.

What Are the Signs and Symptoms of Ependymoma?

Ependymomas can occur in the ventricles of the brain and spinal cord, the symptoms depend on the part affected. This tumor commonly form in the fourth ventricle, cerebellum, brain stem, and less commonly in the cerebrum and spinal cord.

The normal functions that are affected by ependymoma in that part are:

1. Cerebellum - controls movement, posture, and balance.

2. Brain stem - controls breathing, nerves and muscles needed for eyesight, hearing, heart rate, eating, and talking.

3. Cerebrum - controls thinking, helps in solving problems, speech, learning, emotions, reading, and writing.

4. Spinal cord - helps carry messages between other parts of the body and brain.

The common signs and symptoms caused by ependymoma include:

• Headaches.

• Seizures.

• Nausea.

• Vomiting.

• Trouble walking.

• Leg weakness.

• Confusion.

• Irritability.

• Blurry eyesight.

• Change in bowel movement.

• Neck or back pain.

• Problems with urination.

How Is Ependymoma Diagnosed?

Your doctor will perform the following tests to diagnose this tumor:

1. Neurological examination - The doctor will ask you about the symptoms or about your child’s symptoms. Then the neurologist will check the vision, balance, hearing, strength, coordination, and reflexes.

2. MRI of the brain - This imaging test will help the doctor determine the tumor’s size and location. In some cases, along with an MRI, specialized MRI imaging like magnetic resonance angiography is also done.

3. Lumbar puncture - In this test, cerebrospinal fluid is collected for testing. Lumbar puncture is also called a spinal tap. Here, a needle is inserted between the bones in the spine and CSF around the spinal cord is collected. The collected fluid is then tested for any abnormalities or cancer cells.

4. Biopsy - Depending on the location of the tumor, the doctor will collect a small sample from the tumor using a needle or surgically. This sample is then sent to the lab to check for cancer cells.

Based on the test results, the doctor will diagnose this tumor and suggest appropriate treatment.

How Is Ependymoma Treated?

The following are the treatment options for ependymoma:

1. Surgery - A neurosurgeon will try to remove as much of the tumor as possible without damaging the surrounding brain structures. When the tumor is located near a sensitive brain and spinal cord tissues, the entire tumor is not resected as it can become risky. In case the entire tumor is removed, no other treatment is required. But if a portion of the tumor is left behind by the surgeon, then the surgeon might suggest another surgery using a different approach. If the tumor is inoperable or aggressive, then the doctor will suggest radiation therapy.

2. Radiation therapy - Radiotherapy is the use of high-energy X-rays or protons to kill abnormal cancer cells. The beams are directed precisely at the part of the brain where the tumor is present. Sometimes when the surgeon leaves a part of the tumor behind or to prevent an aggressive tumor from recuring, radiotherapy is used. Newer radiation therapy methods, such as conformal radiation therapy and proton therapy, the damage to surrounding healthy tissue from radiation is significantly reduced.

3. Stereotactic radiosurgery - This is a type of radiation therapy, which is used in case the tumor recurs after radiation or surgery. Here, multiple beams of radiation are focused on the tumor to kill the cells.

4. Chemotherapy - The use of drugs to kill cells in the tumor is called chemotherapy. This is not a very effective treatment option.

Recovering from any type of cancer can be painful and challenging. The side effects of chemotherapy and radiation therapy can make the patient weak. Brain surgery can also make the patient feeling tired, weak, dizzy, and confused. But these symptoms usually go away after a while.

If the surgeon was able to remove the tumor completely, then the cure rate is about 65 %. But it can recur later, which is challenging to treat. Myxopapillary ependymoma has a better prognosis as compared to the anaplastic type. And the prognosis is slightly better in adults than children. With the help of proper treatment, the 5-year survival rate is 82 %. For more information on ependymoma, consult a neurosurgeon now.