Introduction to Childhood Ependymoma

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Childhood ependymoma is a rare, malignant brain tumour 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 tumours that develop in the spinal cord or brain. The location of ependymomas in infants and children is intracranial, whereas, in adults, it is spinal.

These tumours 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 form brain tumours when they start to grow uncontrollably. Hence the cause of ependymoma brain tumours is primarily genetic. Ependymoma is considered a malignant tumour as it can grow and spread or metastasise to other body parts. On the other hand, a benign tumour will grow but won’t spread to other tissues or body organs.

Ependymoma tumours can occur anywhere in the fluid-filled passageways or ventricles of the brain or spinal cord. But the tumour 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 tumours occur in the spinal cord or cerebrum.

Ependymoma tumours 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. Headaches, vomiting, nausea, blurred vision, walking difficulties, trouble urinating etc., are the common signs and symptoms of these brain tumours.

Ependymoma can be of different types. The world health organisation (WHO) categorises ependymoma brain tumours 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 tumour can be classified based on its grade. The grade of a tumour explains to what extent the tumour cells look like or resemble normal, healthy cells when viewed under a microscope. The doctor will compare the normal healthy tissue with the tumour tissue. Healthy tissue will contain many different types of cells, all grouped. A tumour is called a low-grade (grade I) or differentiated tumour if it resembles healthy cells but has different cell groupings. If the cell grouping is entirely different, the tumour type is called a high-grade (grade II and III) or poorly differentiated tumour. Grade I tumour cells tend to grow and metastasise rapidly than grade II and grade III tumours.

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 tumours. Also, many clinical trials are underway that are trying and devising new, effective treatment procedures to treat better and cure the tumour condition. People can try clinical trials even after starting active treatment for the tumours.

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 tumours like ependymoma is planned by a team of well-experienced doctors and specialists.