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“Where Hope Springs Eternal”

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“Where Hope Springs Eternal” BASIC FACTS ABOUT EPENDYMOMA “Where hope springs eternal” Pediatric Brain Tumor Foundation of the United States A Resource for Families ACKNOWLEDGEMENTS The Pediatric Brain Tumor Foundation of the United States wishes to thank Ian Pollack, MD, Department of Neurosurgery, Children’s Hospital of Pittsburgh, Pittsburgh, PA, for the scientific review of this publication. SOURCES Statistical data in this publication was obtained from the Central Brain Tumor Registry of the United States (CBTRUS) and the World Health Organization Classification of Tumors, 2000. DISCLAIMER The Pediatric Brain Tumor Foundation of the United States does not engage in rendering medical advice or professional medical services. Information contained in this publication is NOT intended to be a substitute for medical care and should not be used for the diagnosing or the treatment of a brain tumor or any other health problem. If you have or even suspect you have a problem concerning your health or that of someone else, you should consult with your healthcare provider. The materials provided by the Pediatric Brain Tumor Foundation of the United States are compiled based on current information at the time that they were written. Medical research concerning disease and treatments is an ongoing process. We endeavor to keep our materials current. However, you should contact your doctors and medical institutions to seek the most current information available. COPYRIGHT Copyright© 2002 by the Pediatric Brain Tumor Foundation of the United States, Inc. The contents of this publication have been prepared for the exclusive use of the Pediatric Brain Tumor Foundation of the United States. It may not be reproduced in part or in its entirety without the written permission of the Pediatric Brain Tumor Foundation of the United States. Mission Statement Find the cause and cure of childhood brain tumors through the support of medical research; Increase public awareness about the severity and prevalence of childhood brain tumors; Aid in early detection and treatment of childhood brain tumors; Support a national database on all primary brain tumors; and Provide hope and emotional support for the thousands of children and families affected by this life-threatening disease. Pediatric Brain Tumor Foundation of the United States 1 INTRODUCTION The news that a child has a brain tumor is nearly impossible to absorb. Naturally, when parents first receive such news, they are stunned. Gradually, the initial shock is replaced by a desire to learn more about the tumor: Exactly where is it? Which areas of the body is it affecting? What can be done about it? What lies ahead? Almost instinctively, people realize that seeking the answers to these questions will restore a sense of control. To help you in your efforts to gain understanding about your child’s illness, the Pediatric Brain Tumor Foundation of the United States (PBTFUS) has put together this booklet. It contains information about the specific type of tumor that has invaded your family’s world. We encourage you to learn as much as you can. Trust your instincts. When you have questions or concerns, voice them. Take an active part in selecting which approach will result in the best care for your child. Pediatric brain tumors require specialized treatment methods that are most often provided at comprehensive pediatric hematology/oncology centers by a multidisciplinary team of pediatric cancer specialists. This team usually includes physicians, nurses, social workers, child life specialists, psychologists and rehabilitation specialists. Such care should provide families access to education and support services. We have much to learn about childhood brain tumors. Funding research to find the cause and cure, increasing public awareness, and aiding in the early detection and treatment of childhood brain tumors are all the ways the PBTFUS fulfills its mission. Families like yours are at the heart of our mission. If you need further information or other PBTFUS publications, our Family Support Program Coordinator is on staff to take your call. Contact us at 1-800-253-6530 or e-mail: [email protected]. A FEW BASIC FACTS ABOUT BRAIN TUMORS What causes a brain tumor? The short answer is: We don’t know. Researchers believe that inherited and genetic factors may be involved. Environment may play a part. While studies have yielded valuable information, they have not provided a concrete answer about the cause of most brain tumors. The key may even lie in areas not yet investigated by science. 2 Pediatric Brain Tumor Foundation of the United States What does it mean when a tumor is classified as Grade I, Grade II, Grade III, or Grade IV? Tumors are graded to help determine the best treatment approach. The more aggressive and dangerous a tumor is, the higher the grade it is assigned. Some types of tumors, such as medulloblastoma, are always considered high grade. Grade I tumors are the most benign appearing under the microscope. Grade II is a significant step higher with cells that more easily become malignant. Malignant tumors invade and destroy healthy tissue. Grade III and IV tumors are malignant with increasing severity. Grade alone does not determine prognosis, however. Other factors, such as tumor location, can influence how easy or difficult a tumor is to treat. What is staging? The “stage” of a cancerous tumor is based on its size and the extent to which it has metastasized, or spread, beyond the site of its origin. EPENDYMOMA What is an ependymoma? Ependymomas arise from cells that line the ventricles (hollow areas in the nervous system that contain cerebrospinal fluid) of the brain, the central canal of the spinal cord, and the filum terminale (a thread-like structure within the lower end of the spinal cord). They are considered a type of glioma. Most appear benign under the microscope, but nonetheless can show malignant behavior because they tend to be locally invasive. Some tumors do appear malignant and these tend to have a somewhat worse prognosis. Types of ependymoma: Ependymoma: A slowly growing tumor of children and young adults, most are benign or low-grade, but some are capable of malignant behavior. Anaplastic Ependymoma: A higher grade malignant tumor growing more rapidly. Sub-Ependymoma: Benign, slow growing. Myxopapillary Ependymoma: Benign, slow growing tumors of the spinal cord that occur mostly in young adults. Pediatric Brain Tumor Foundation of the United States 3 cerebrum subarachnoid space posterior fossa brainstem midbrain pons tentorium medulla oblongata fourth ventricle cerebellum LOCATION: In the brain, the most common location for ependymomas is the posterior fossa and the floor of the fourth ventricle. Tumors arising in the posterior fossa often infiltrate the brain stem to involve the medulla oblongata and upper spinal cord. Seeding, or spreading, to the ventricles and subarachnoid space is not uncommon. What are the symptoms of an ependymoma? Many symptoms associated with ependymomas are caused by increased intracranial pressure (ICP). They may vary depending on the age of the child and exact location of the tumor. In children younger than two years of age, symptoms may include vomiting, irritability, lethargy, gait disturbance, feeding problems, involuntary eye movement, and increased head size due to hydrocephalus, increased cerebrospinal fluid inside the brain. In children older than two, vomiting, headache, and gait disturbance are the most common symptoms. How common are ependymomas in children? Ependymomas represent just over 6 % of childhood brain and CNS (Central Nervous System) tumors. Incidence rates for ependymoma are similar in children and adults; however, ependymomas are more likely to occur in infants and very young children than in older children. Ependymomas are less common in children between the ages of 5 and 14, with an average 4 Pediatric Brain Tumor Foundation of the United States incidence rate of 1.4 per million. The rate of occurrence is slightly higher in males than females. (SEER 1986-94; CBTRUS 1992-1997) DIAGNOSIS Diagnosis is the process of finding out which disease is causing a person’s symptoms. Only after this is done can the best treatment plan be determined. In many cases, making the diagnosis of a brain tumor can be challenging because the early symptoms can mimic those of other less serious conditions. However, once a diagnosis of a brain tumor is considered, an imaging evaluation of the brain is required to confirm the suspicion. Following examination and evaluation of signs and symptoms, MRI and CT scans are useful in determining the presence of a brain or spinal cord tumor, but biopsy is the only reliable way to confirm a specific diagnosis. MRI Suite MRI (Magnetic Resonance Imaging) An MRI scan combines high-frequency radio waves and a strong magnetic field to produce a picture of the inside of the body. No x-ray radiation is involved. Before the test begins the doctor may inject a special dye called contrast material into the patient’s vein. The dye makes it easier to see abnormal tissue. The MRI procedure usually takes from thirty minutes to an hour. To have an MRI, the patient lies on a mechanical table. The table is then moved into a large structure with a Pediatric Brain Tumor Foundation of the United States 5 donut-shaped opening. If a “closed MRI” is used, it’s a little like going into a tunnel. In contrast, an “open MRI” is open on the top and sides. Children are never left alone during an MRI. The child is continually monitored during the procedure. Some MRI centers will allow parents to remain with their children as well. Although the scan should not cause any pain, the thumping noise that occurs in the machinery from time to time during the procedure may startle young children. An accurate scan can only be obtained if the patient is still.
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