Ependymoma - Childhood

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What is Childhood  Ependymoma?

Childhood ependymoma is a type of brain tumor. A tumor begins when normal cells change and grow uncontrollably, forming a mass. A tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body). Ependymoma is a rare, cancerous brain tumor.                    

A brain tumor is named after the cells from which it develops. Ependymoma starts in the ependymal cells that line the ventricles (fluid-filled spaces) in the brain and the central canal of the spinal cord. The brain and spinal column make up the central nervous system (CNS), where all vital functions are controlled, including thought, speech, and body strength.

Ependymoma belongs to a group of brain tumors called gliomas. A glioma is a tumor that grows from a glial cell, which is a supportive cell in the brain. Even though ependymoma can occur in any part of the brain or spine, it most commonly occurs in the cerebrum, which is the largest part of the brain. The cerebrum is the top part of the brain and controls motor skills (muscle coordination) and talking.

Symptoms & Signs

Children with ependymoma may experience the following symptoms or signs. Sometimes, children with ependymoma do not show any of these symptoms. Or, these symptoms may be cause by a medical condition that is not a tumor. If you are concerned about a symptom or sign on this list, please talk to your child’s doctor.

Headaches

Seizures

Nausea and vomiting

Changes in vision, such as blurriness

Difficulty with walking or balance

Swelling of the nerve at the back of the eye

Jerky eye movements

Neck pain

Your child’s doctor will ask you questions about the symptoms your child is experiencing to help find out the cause of the problem, called a diagnosis. This may include how long he or she has been experiencing the symptom(s) and how often.

If a tumor is diagnosed, relieving symptoms and side effects remains an important part of care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with your child’s health care team about symptoms your child experiences, including any new symptoms or a change in symptoms.

Risk Factors

A risk factor is anything that increases a person’s chance of developing cancer. Although risk factors often influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do.

The causes of childhood ependymoma are unknown. There is little known about the risk factors or ways to prevent the disease.

Rarely, children with neurofibromatosis type 2 (NF2) have an increased risk of developing ependymoma. NF2 is a hereditary syndrome that causes tumors of the central nervous system that are usually noncancerous.

Diagnosis

Doctors use many tests to diagnose a tumor and find out if it has metastasized (spread). Some tests may also determine which treatments may be the most effective. For most tumors, a biopsy is the only way to make a definitive diagnosis of cancer. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Imaging tests may be used to find out whether the tumor has metastasized. Your child’s doctor may consider these factors when choosing a diagnostic test:

Age and medical condition

Type of tumor suspected

Severity of symptoms

Previous test results

In addition to a physical examination, the following tests may be used to diagnose ependymoma:

Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. Sometimes, a contrast medium (a special dye) is injected into a vein to provide better detail.

Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A contrast medium may be injected into a patient’s vein to create a clearer picture. A spinal MRI may be used to determine if the tumor has spread to the spine.

Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. Other tests can suggest that a tumor is present, but only a biopsy can make a definite diagnosis. The sample removed from the biopsy is analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease). For ependymoma, surgery  is needed to get a sample of tissue.

Lumbar puncture (spinal tap). A lumbar puncture is a procedure in which a doctor uses a needle to take a sample of cerebral spinal fluid (CSF) to look for cancer cells, blood, or tumor markers (substances found in higher than normal amounts in the blood, urine, or body tissues of people with certain kinds of cancer). CSF is the fluid that flows around the brain and the spinal cord. Doctors generally give an anesthetic to numb the lower back before the procedure. This procedure is generally done after ependymoma is diagnosed and the tumor is removed.

After these diagnostic tests are done, your child’s doctor will review all of the results with you. If the diagnosis is a tumor, these results also help the doctor describe the tumor; this is called staging.

Stages

Staging is a way of describing where the tumor is located, if or where it has spread, and whether it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the tumor's stage, so staging may not be complete until all of the tests are finished. Knowing the stage helps the doctor decide what kind of treatment is best and can help predict a patient's prognosis (chance of recovery). There are different stage descriptions for different types of tumors.

There is no formal staging system for ependymoma, however, it can be classified based on where in the brain the tumor is located and whether it has spread.

Supratentorial: The tumor is above the membrane that covers the cerebellum, known as the tentorium cerebella.

Infratentorial: The tumor is growing below the tentorium cerebella.

Spinal: The tumor is growing in the central canal of the spinal cord.

Recurrent: A recurrent tumor is a tumor that comes back after treatment. If there is a recurrence, the tumor may need to be staged again (called re-staging) using the system above.

Grading

A tumor may also be described by its grade, which is used to describe how much the tumor appears like normal tissue when viewed with a microscope. In a tumor that resembles normal tissue, doctors can clearly see different types of cells grouped together (called well differentiated). In a higher-grade tumor, the cells usually look less like normal cells, or “wilder” (called poorly differentiated or undifferentiated). In general, a child with a more differentiated, lower-grade tumor has a better prognosis.

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