Ependymoma

A mass of cells grows to form a tumor in the brain or spinal cord, and is known as an ependymoma. Its inception occurs in the ependymal cells, which line the passageways that transport cerebrospinal fluid. The brain and spinal cord are embedded in fluid, which is responsible for their protection.

Statistics and extent of the cancer

• 9% of all pediatric central nervous system tumors are ependymomas
• Ependymomas are responsible for less than 2% of all adult central nervous system tumors
• The estimated annual incidence rate in the United States varies from 0.29 to 0.6 cases per 1,00,000 individuals
• More likely to occur in male patients
• 5% of intercranial tumors in children <20 years old (5-10% on the basis of the source and database)
• 54.4% of pediatric ependymomas occur in the posterior fossa, 32.5% in the supratentorial compartment and 13.1% in the spine
• In adults, 64.1% of ependymomas occur in the spinal cord

Types of ependymoma

Healthcare providers classify ependymomas on the basis of their rate of development.

Grade 1 and 2 ependymomas are usually non-cancerous and don’t spread to different organs or tissues from the site of origin.

Grade 3 ependymomas are benign or cancerous and proliferate faster and more aggressively than the comparatively lower grade tumors.

Types of ependymomas

• Subependymomas (Grade 1): They grow in the areas surrounding the brain which contain cerebrospinal fluid→ ventricles. It is more likely to occur in adults than in children.
• Classic Ependymomas (Grade 2): They are named as classic ependymomas because of their commonality. It affects both children and adults alike.
• Myxopapillary Ependymomas (Grade 2): They occur in the lower spinal chord and mostly occur in adult men.
• Anaplastic Ependymomas (Grade 3): They occur near the base of our brains, and proliferate rapidly to other tissues and organs and usually regrow after treatment (recur).

Location

Most ependymomas develop in or around the back of the brain at the bottom of the skull, called the posterior fossa. This region includes the cerebellum and the brain stem- the areas which control critical body functions and control and coordination.

They can also originate in the upper region of the brain, called the supratentorial area. This area includes the cerebrum, ventricles, choroid plexus, hypothalamus, pineal gland, pituitary gland, and optic nerve.

They also occur in the spinal cord.

Stages

Ependymomas are grouped in three grades (grade 1, 2, or 3, also written as grade I, II, or III) based on their characteristics under a microscope and their behavior:

Ependymomas are divided into three grades (Grade 1, Grade 2, Grade 3)-

• Grade 1: These ependymomas are low-grade tumors, specifically subependymomas, an ependymoma subtype that occurs in the brain or spine. They are more likely to occur in adults, when compared to children.
• Grade 2: These ependymomas are also low-grade tumors, but they are expected to recur more often than grade 1 ependymoma. They are expected to regrow, especially if these tumors cannot be completely removed through surgery. Subtypes include myxopapillary ependymoma (which usually occurs in the spine) and conventional ependymoma grade 2. (which occurs in both the brain and the spine, on the basis of the molecular subtype)
• Grade 3: These ependymomas are cancerous and fast-growing. They occur both in the brain and spine.

Symptoms

When the tumor is in the brain, symptoms may include:

• Blurry vision.
• Confusion.
• Headaches.
• Irritability.
• Nausea.
• Seizures.
• Vomiting.

When the tumor is in the spinal cord, symptoms may include:

• Bowel changes.
• Loss of balance.
• Pain in the neck or back.
• Trouble walking.
• Weakness in the legs.

Risk factors and causes

What are the risk factors for ependymoma?

Since the exact cause of ependymoma is not well understood, doctors are often unsure about specific factors that increase the risk of developing this tumor.

However, certain inherited genetic conditions may raise the likelihood of ependymoma, such as:

• Neurofibromatosis type 240
• Turcot syndrome type B
• Multiple Endocrine Neoplasia type 1 (MEN1) syndrome

Causes

The exact cause of ependymoma is unknown. This tumor begins when cells in the brain or spinal cord start to grow abnormally.

Ependymoma develops because the DNA in these cells changes. DNA contains the instructions that guide how a cell grows, divides, and dies. In normal cells, these instructions regulate controlled growth and programmed cell death. However, in tumor cells, DNA mutations alter these instructions, causing the cells to grow and multiply rapidly and avoid dying when they should. This leads to an excessive buildup of cells, forming a tumor.

Prevention

Can You Prevent Ependymoma?

Unfortunately, there is no known way to prevent ependymoma. These tumors develop spontaneously, and their exact cause is unknown, so no specific preventive measures exist. While it's natural to worry about "what ifs" and "whys," it's important to focus on what you can control and take good care of your health.

Certain lifestyle habits may lower the risk of some cancers and help improve recovery if diagnosed with ependymoma. Here are some recommendations:

• Maintain a healthy diet: Eat a balanced diet rich in fruits, vegetables, and whole grains. Limit processed foods, red meats, and saturated fats.
• Stay physically active: Aim for at least 150 minutes of moderate exercise or 75 minutes of vigorous exercise weekly, such as walking, jogging, cycling, or swimming.
• Avoid tobacco and limit alcohol: Smoking is a major cancer risk and should be avoided.46 Limit alcohol to no more than one drink per day for women and two for men, as excessive consumption can raise cancer risk.
• Limit exposure to toxins and radiation: Avoid harmful chemicals and protect yourself from too much sun exposure. If your job involves radiation, follow safety protocols carefully.
• Regular medical check-ups: Routine visits with your healthcare provider allow for early detection of any health concerns and provide a chance to discuss any symptoms or risks.

If you have a family history of brain or spinal cord tumors, talk with your doctor. They may suggest tailored monitoring or screening to detect any potential problems early.

Diagnosis

To diagnose ependymoma, doctors use several tests and procedures:

• Neurological exam: A healthcare provider assesses vision, hearing, balance, coordination, strength, and reflexes. Difficulties in these areas can help identify which part of the brain or spinal cord might be affected by a tumor.
• Imaging tests: These create pictures of the body to show the tumor's location and size. MRI scans are commonly used for brain tumors, sometimes combined with specialized techniques like magnetic resonance angiography. Since ependymomas can occur in both the brain and spinal cord, imaging of both areas might be performed.
• Cerebrospinal fluid testing: Also called a lumbar puncture or spinal tap, this test involves inserting a needle into the lower spine to collect fluid around the spinal cord. The fluid is examined for the presence of tumor cells.

Your provider may use some of the following tests to diagnose an ependymoma:

• Biopsy
• CT scan
• Lumbar puncture
• MRI

If these tests suggest ependymoma, surgery may be recommended to remove the tumor. After removal, the tumor tissue is analyzed in a lab to confirm the diagnosis and determine the tumor's cell type, which helps guide treatment decisions.

Treatment

Treatment for ependymoma may involve several approaches:

Surgery:

Neurosurgeons aim to remove as much of the tumor as possible, ideally the entire tumor. However, if the tumor is near critical brain or spinal tissue, complete removal might be too risky.

Radiation therapy:

This treatment uses powerful energy beams—such as X-rays or protons—to destroy tumor cells. Radiation may be given after surgery to prevent recurrence or if the tumor cannot be completely removed.

Radiosurgery:

A focused form of radiation that delivers multiple low-dose beams from different angles, concentrating a high dose at the tumor site to destroy cancer cells without affecting surrounding tissue as much.

Chemotherapy:

This treatment uses drugs to kill tumor cells but is less commonly used for ependymoma. It may be considered if the tumor returns after surgery and radiation.

Targeted therapy:

These drugs attack specific molecules involved in tumor growth, helping to kill tumor cells. Targeted therapy might be an option if the tumor recurses after initial treatments.

Clinical trials:

Experimental treatments are tested through clinical trials. These can offer access to new therapies, but potential risks and side effects might not be fully known. Discuss with your healthcare team if participation in a trial is of interest.

Side effects

Ependymoma is a tumor that arises in the brain or spinal cord, and its symptoms and side effects can differ depending on its location, size, and whether it is benign or malignant.

Common Side Effects

Recovery varies from person to person. After treatment, some individuals may experience:

• Tiredness or lack of energy
• Cognitive difficulties, including problems with memory, focus, or speech
• Pain or discomfort at the surgical site
• Neurological issues such as weakness or numbness

These symptoms often improve gradually as the brain or spinal cord heals.

In some cases, additional support may be needed, such as:

• Physical therapy – to regain strength and mobility
• Speech therapy – to address communication or swallowing difficulties
• Rehabilitation – to improve coordination and overall function

Palliative care might be needed for patients nearing the end of life. Individuals who have surgery can experience various neurological impairments. To support recovery, they may need physical therapy, speech therapy, and occupational therapy. However, in many cases, these neurological issues are long-lasting or permanent.

FAQs About Ependymoma

What is ependymoma?

Ependymoma is a rare tumor that begins in the brain or spinal cord. It can appear in various parts of the central nervous system (CNS), including the brain’s upper regions, the back of the brain (posterior fossa), and the spinal cord. It affects both children and adults.

How are ependymomas classified?

They’re graded based on how abnormal the cells look and how fast they grow:

• Grade I: Subependymoma, myxopapillary (slow-growing)
• Grade II: Classic ependymoma
• Grade III: Previously called anaplastic (more aggressive)

Classification also includes tumor cell features, invasion, and genetic markers.

What age groups do ependymomas affect?

Ependymomas can occur at any age.

• In children, they're more often found in the posterior fossa (back of the brain).
• In adults, they’re more common in the spinal cord.

Is ependymoma cancer?

Ependymomas can be low-grade (I or II) or high-grade (III).

High-grade tumors are malignant. Low-grade tumors grow slowly but can still cause serious symptoms and may return. Both types require careful treatment and monitoring.

What treatment is available for an individual diagnosed with an ependymoma?

• Low-grade tumors: Surgery is the main treatment, often followed by radiation if complete removal isn’t possible.
• High-grade tumors: Often need surgery and radiation. Chemotherapy may also be recommended, especially if the tumor is aggressive or comes back.

What happens if there is a recurrence?

Recurrent tumors may require:

• Additional surgery
• Radiation or chemotherapy
• Participation in clinical trials for new treatments

Treatment decisions depend on tumor type, grade, location, previous treatment, and patient’s overall health

Citations and Sources

1. Introduction- Mayo Clinic: Overview (https://www.mayoclinic.org/diseases-conditions/ependymoma/symptoms-causes/syc-20580744)
2. Statistics and Extent- Cancer Therapy Advisor: Epidemiology (https://www.cancertherapyadvisor.com/ddi/ependymoma/)
3. Statistics and Extent- Pediatric Hematology Oncology Journal: Tumor origins and epidemiology (https://www.sciencedirect.com/science/article/pii/S2468124523000232)
4. Types- Cleveland Clinic: Types of Ependymoma (https://my.clevelandclinic.org/health/diseases/23147-ependymoma)
5. Stages- National Cancer Institute: What are the Grades of Ependymomas? (https://www.cancer.gov/rare-brain-spine-tumor/tumors/ependymoma#:~:text=Ependymomas are grouped in three,the brain or the spine)
6. Symptoms- Mayo Clinic: Symptoms (https://www.mayoclinic.org/diseases-conditions/ependymoma/symptoms-causes/syc-20580744#:~:text=This tumor can form in,Causes)