Introduction to Ependymoma Childhood

Executive Summary

Childhood ependymoma is a rare, malignant brain tumor commonly found in young children and teens between 0-19 years of age. The location of ependymomas in infants and children is intracranial, whereas, in adults, it is spinal. Ependymoma tumors can occur anywhere in the fluid-filled passageways or ventricles of the brain or spinal cord. But the tumor is most commonly seen to appear in the cerebellum. These can block the normal flow of cerebral spinal fluid (CSF) and lead to a condition called hydrocephalus, which builds up cerebral spinal fluid that will eventually cause increased pressure within the brain. Headaches, vomiting, nausea, blurred vision, walking difficulties, trouble urinating etc., are the common signs and symptoms of these brain tumors. Subependymoma, Myxopapillary ependymoma, Ependymoma, RELA fusion-positive ependymoma, and Anaplastic ependymoma are the different types of ependymoma. There is no standard staging system for ependymoma, but the tumors are classified based on their grade.

The actual cause of childhood ependymoma is still unknown. Some studies claim that the disease is associated with body genetics. Surgery, chemotherapy, and radiation therapy are the most common treatments for ependymoma brain tumors. The treatment options for ependymoma can cause many side effects. Hence, it is essential for parents or guardians of the affected child to ask the healthcare team questions and what can be expected from each treatment option. 

What is Childhood Ependymoma?

Childhood ependymoma is a rare, malignant brain tumor commonly found in young children. The disease condition is usually seen in children and teens between 0-19 years of age. Ependymomas are rare brain tumors that develop in the spinal cord or brain. The location of ependymomas in infants and children is intracranial, whereas, in adults, it is spinal ^​1​.

These tumors are known to grow from the glial cells in the brain or spine; hence they are considered glioma. Typically glial cells provide support and stability to neurons in the Central Nervous System. But these cells turn cancerous and forms brain tumors when they start to grow uncontrollably. Hence the cause of ependymoma brain tumors is primarily genetic. Ependymoma is considered a malignant tumor as it can grow and spread or metastasise to other body parts. On the other hand, a benign tumor will grow but won’t spread to other tissues or body organs.

Ependymoma tumors can occur anywhere in the fluid-filled passageways or ventricles of the brain or spinal cord. But the tumor is most commonly seen to appear in the cerebellum. The cerebellum is the vital part of the brain that coordinates the body’s movements. Only in rare cases do ependymoma tumors occur in the spinal cord or cerebrum.

Ependymoma tumors can block the normal flow of cerebral spinal fluid (CSF) and lead to a condition called hydrocephalus. Hydrocephalus condition means a build-up of cerebral spinal fluid that will eventually cause increased pressure within the brain ^​2​. Headaches, vomiting, nausea, blurred vision, walking difficulties, trouble urinating etc., are the common signs and symptoms of these brain tumors.

Ependymoma can be of different types. The world health organisation (WHO) categorizes ependymoma brain tumors into five subtypes. They include: 

• Subependymoma (WHO grade I; rare in children).
• Myxopapillary ependymoma (WHO grade I).
• Ependymoma (WHO grade II)
• RELA fusion-positive ependymoma (WHO grade II or grade III changes the RELA gene).
• Anaplastic ependymoma (WHO grade III).

There is no standard staging system for ependymoma. But the tumor can be classified based on its grade. The grade of a tumor explains to what extent the tumor cells look like or resemble normal, healthy cells when viewed under a microscope. The doctor will compare the normal healthy tissue with the tumor tissue. Healthy tissue will contain many different types of cells, all grouped. A tumor is called a low-grade (grade I) or differentiated tumor if it resembles healthy cells but has different cell groupings. If the cell grouping is entirely different, the tumor type is called a high-grade (grade II and III) or poorly differentiated tumor. Grade I tumor cells tend to grow and metastasise rapidly than grade II and grade III tumors.

The actual cause of childhood ependymoma is still unknown. Some studies claim that the disease is associated with body genetics. Since the underlying cause of the disease is unknown, it is rather impossible to state the disease’s preventive measures and related risk factors.

Surgery, chemotherapy, and radiation therapy are the most common treatments for ependymoma brain tumors. Also, many clinical trials are underway that are trying and devising new, effective treatment procedures to treat better and cure the tumor condition ^​3​. People can try clinical trials even after starting active treatment for the tumors.

The treatment options for ependymoma can cause many side effects. Hence, it is essential for parents or guardians of the affected child to ask the healthcare team questions and inquire about what can be expected out of each treatment option. Also, ask about all the pros and cons of the recommended treatment plan. People should make sure that the treatment for brain tumors like ependymoma is planned by a team of well-experienced doctors and specialists.

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