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What is Childhood Central Nervous System?
A central nervous system (CNS) tumor begins when normal cells in the brain or the spinal cord 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).
A CNS tumor is especially problematic because a person's thought processes and movements can be affected. This type of tumor also may be challenging to treat because the tissues surrounding the tumor may be vital to the body's functioning.
The brain is the center of thought, memory, and emotion. It controls the five senses (smell, touch, taste, hearing, and sight), movement, and other basic functions of the body, including heartbeat, circulation, and breathing. The spinal cord consists of nerves that carry information back and forth between the body and the brain.
Anatomy of the brain
The brain is made up of four major parts: the cerebrum, cerebellum, brain stem, and meninges.
The cerebrum, the largest part of the brain, contains two cerebral hemispheres and is divided into four lobes where specific functions occur.
The frontal lobe controls reasoning, emotions, problem solving, and parts of speech and movement
The parietal lobe controls the sensations of touch, pressure, pain, and temperature
The temporal lobe controls memory and the sense of hearing
The occipital lobe controls vision
The cerebellum, or "little brain," is located underneath the cerebrum. The cerebellum controls coordination and balance.
The brain stem, which is the lowest portion of the brain and connects to the spinal cord, controls involuntary functions essential for life, such as a person's heartbeat and breathing.
The meninges are the membranes that surround and protect the brain and spinal cord. There are three meningeal layers, called the dura mater, arachnoid, and pia arachnoid.
Types of CNS tumors
There are many different types of CNS tumors. Some are cancerous and very likely to grow and spread (called very aggressive or high grade), others are less aggressive (low grade), and others are noncancerous, which means they are not likely to grow and spread. The following types of CNS tumors are most common among children:
Astrocytoma
Brain stem glioma
Ependymoma
Germ cell tumor
Medulloblastoma
Symptoms and Signs
Children with a CNS tumor may experience the following symptoms or signs. Sometimes, children with a CNS tumor do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not a tumor. If you are concerned about a symptom or sign on this list, please talk with your child's doctor.
The symptoms of a CNS tumor can affect any of the brain's functions and depend on where the tumor is located. A CNS tumor may cause the following symptoms:
A seizure or convulsion (sudden involuntary movements of a person's muscles)
Staring or repetitive automatic movements (a neck tilt or a squint)
Unexplained, persistent nausea and projectile vomiting
Weakness or clumsiness that seems to get worse (difficulty walking and balancing)
Early or delayed puberty, or delayed or abnormal growth
Sleep apnea (breathing that stops periodically while asleep)
Vision problems
Headache, which may wake a child up at night or develop early in the morning
Pain, especially back pain
Irritability, listlessness, or changes in personality
In a baby, the only symptom may be that the head is growing too fast. An infant's skull can expand to make room for a growing tumor, so the baby may have a larger than normal head.
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 your child has been experiencing 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 the health care team about the 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 a tumor. Although risk factors often influence the development of a tumor, most do not directly cause a tumor. Some people with several risk factors never develop a tumor, while others with no known risk factors do.
The cause of most CNS tumors in children is not known. Researchers are studying a variety of possible causes of CNS tumors, including viruses. Different types of CNS tumors occur in different age groups. For example, some types of CNS tumors are more common in children younger than three. Head injury does not appear to cause a CNS tumor.
A small number of CNS tumors occur in families. However, only a small percentage of children with a brain tumor have an identifiable genetic cause, usually in association with a familial disease called neurofibromatosis. Neurofibromatosis is an inherited disorder that causes neurofibromas (noncancerous tumors) to form on peripheral nerves in the body, brown spots on the skin, and deformities of tissues and bones. Neurofibromatosis is also called Recklinghausen's disease or von Recklinghausen's disease. Other, less common genetic conditions associated with a higher risk of a CNS tumor include Li-Fraumeni syndrome, tuberous sclerosis, nevoid basal cell syndrome, and Turcot syndrome.
Diagnosis
Doctors use many tests to diagnose a tumor, determine if it is cancerous, and find out if it has metastasized (spread). Some tests may also determine which treatments may be the most effective. For most types of tumors, a biopsy is the only way to make a definitive diagnosis. 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 a CNS tumor:
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 patient's 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.
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 during the biopsy is analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease). The type of biopsy performed depends on the location of the tumor.
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 to decide what kind of treatment is best and can help predict a patient's prognosis (chance of recovery).
Several types of childhood CNS tumors can spread through the spinal fluid that surrounds the brain and the spine. For most types of CNS tumors, the tumor is described as either high-grade or low-grade, depending on how much of the tumor is left after surgery, the child's age, and whether the tumor has spread. In general, a child with a low-grade tumor has a better prognosis.
A recurrent tumor is a tumor that comes back after treatment. If there is a recurrence, the tumor may need to be staged and graded again.
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