Introduction To Childhood Ependymoma
Childhood ependymoma is a disease in which malignant cells form in the tissues of the brain and spinal cord. These tumours can occur anywhere within the neuraxis, but distribution and molecular characteristics are fairly site-dependent, divided into three groups depending on the anatomical compartment in which they are found
The brain controls vital functions such as memory and learning, emotion, and the senses such as hearing, sight, smell, taste, and touch. The spinal cord is made up of bundles of nerve fibres that connect the brain with nerves in most parts of the body. Ependymomas form from ependymal cells that line the ventricles and passageways in the brain and the spinal cord. Ependymal cells make cerebrospinal fluid (CSF). There are different types of ependymomas.
The Ependymoma tumours are grouped into five main subtypes:
- Sub ependymoma (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 with change in the RELA gene).
- Anaplastic ependymoma (WHO grade III).
The part of the brain that is affected depends on where the ependymoma forms as these can form anywhere in the fluid-filled ventricles and passageways in the brain and spinal cord. Most of the forms in the fourth ventricle affect the cerebellum and the brain stem. Ependymomas are formless commonly in the cerebrum and rarely in the spinal cord as per my experience. The cause of most is unknown. The signs and symptoms of childhood ependymoma are not the same in every child.
Signs and symptoms caused by childhood ependymoma are as follows
- Frequent headaches.
- Nausea and vomiting.
- Pain in the neck or back.
- Loss of balance or trouble walking.
- Weakness in the legs.
- Blurry vision.
- A change in bowel function.
- Trouble urinating.
- Confusion or irritability.
Tests that examine the brain and spinal cord are used to detect childhood ependymomata such as physical exam and health history, neurological exam, MRI (magnetic resonance imaging) with gadolinium, and Lumbar puncture. Once it is diagnosed and removed in surgery.
The prognosis and treatment options depend on:
- Where the tumour has formed in the central nervous system (CNS).
- Whether there are certain changes in the genes or chromosomes.
- Whether any cancer cells remain after surgery to remove the tumour.
- The type and its grade
- The age of the child when the tumour is diagnosed.
- Whether the cancer has spread to other parts of the brain or spinal cord.
- Whether the tumour has just been diagnosed or has recurred (come back).
- Prognosis also depends on whether radiation therapy was given, the type and treatment dose, and whether chemotherapy alone was given.
Stages are not defined as there is no standard staging system for childhood ependymoma.
There are different types of ependymoma Treatments for children
Three types of treatment are used:
- Radiation therapy
- New types of treatment are being tested in clinical trials.
- Targeted therapy