Where hope springs eternal

BASIC FACTS ABOUT JUVENILE PILOCYTIC ASTROCYTOMA (JPA) Where hope springs eternal Pediatric Brain Tumor Foundation A Resource for Families

ACKNOWLEDGEMENTS The Pediatric Brain Tumor Foundation wishes to thank Henry S. Friedman, MD, The Brain Tumor Center, Duke University Medical Center, Durham, NC, and Ian F. Pollack, MD, Department of Neurosurgery, Children s Hospital of Pittsburgh, Pittsburgh, PA, for scientific review of this publication. SOURCES Statistical data in this publication obtained from the Central Brain Tumor Registry of the United States (CBTRUS). DISCLAIMER The Pediatric Brain Tumor Foundation 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 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 review with your doctors and medical institutions to attempt to seek the most current information available. COPYRIGHT Copyright 2000 by the Pediatric Brain Tumor Foundation. Reprinted in 2004. The contents of this publication have been prepared for the exclusive use of the Pediatric Brain Tumor Foundation. It may not be reproduced in part or in its entirety without the written permission of the Pediatric Brain Tumor Foundation.

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 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 (PBTF) 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 with access to education and support services. We have much to learn about childhood brain tumors. Funding research to find causes and cure, increasing public awareness, and aiding in the early detection and treatment of childhood brain tumors are all ways the PBTF fulfills its mission. Families like yours are at the heart of our mission. If you need further information or other PBTF publications, our National Family Support Program Coordinator is on staff to take your call. Contact us at 1-800-253-6530 or e-mail: familysupportr@pbtfus.org. 2 Pediatric Brain Tumor Foundation

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. The key may even lie in areas not yet investigated by science. What does it mean when a tumor is classified as Grade I, Grade II, Grade III, or Grade IV? Tumors are graded based on their appearance when examined under the microscope 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 is considered benign (non-cancerous). Grade II is a significant step higher with cells that more easily become malignant, although this is uncommon in children. Malignant tumors invade and destroy healthy tissue. Grade III and IV tumors are malignant with increasing severity. 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. Because a juvenile pilocytic astrocytoma (JPA) is benign and generally does not spread to other sites, physicians usually do not assign a stage to this type of tumor. cerebrum thalamus (third ventricle) hypothalamus posterior fossa brainstem midbrain pons medulla oblongata fourth ventricle cerebellum tentorium Pediatric Brain Tumor Foundation 3

JUVENILE PILOCYTIC ASTROCYTOMA (JPA) What is Juvenile Pilocytic Astrocytoma? An astrocytoma is a tumor of the glial cells, or neuroglia, also called nerve glue. Glial cells have different functions: some respond to infection or damage in the nervous system, while others connect and support the neurons. Glial cells are involved in a high percentage of brain and spinal cord tumors. According to the World Health Organization s classification system, a juvenile pilocytic astrocytoma, often abbreviated JPA, is a grade I tumor. The word pilocytic means comprised of fiber-shaped cells. Well-defined, slow-growing, and usually benign, JPAs can arise from the cerebellum, cerebrum, or spinal cord. They have also been found in the structures of the eye, hypothalamus, thalamus, and the region of the third ventricle. Ventricles are hollow areas in the brain that contain cerebrospinal fluid (CSF). The third ventricle is surrounded by the thalamus. Although JPAs are typically non-cancerous, that does not mean they are harmless. Even a benign tumor can be life-threatening if it is difficult or impossible to reach safely. What are the symptoms of a JPA? Symptoms depend on the exact location of the tumor. The cerebellum, where JPAs often start, aids in coordinating voluntary movements and maintains balance and muscular tone. These are the functions most affected by a tumor in that area. A JPA in the cerebrum is likely to cause headaches, nausea, vomiting, weakness of arms or legs, visual disorders, or seizures. If the hypothalamus or pituitary gland is involved, effects may include early onset of puberty, delayed adolescence, short stature with a decreasing rate of growth, or behavioral changes. Other indications that a JPA may be present include diabetes insipidus and abnormal thinness. If the main bulk of the tumor is in the cavity of the third ventricle, the flow of CSF may be blocked, causing hydrocephalus or water on the brain. In babies and very young children whose skull bones are not firmly set, hydrocephalus can actually cause the skull to increase in size. How common are JPAs in children? JPAs most often occur in the first decade of life, with the highest incidence being between the ages of five and nine. Medulloblastoma is the only tumor type more likely to be found in that age group. JPAs appear to be equally common in boys and girls. (CBTRUS) 4 Pediatric Brain Tumor Foundation

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. Initially, MRI and CT scans are most useful in determining the presence of a JPA. 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 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 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 MRI Suite Pediatric Brain Tumor Foundation 5

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 be obtained only if the patient is still. For this reason, a mild sedative may be used to calm a child who seems very upset. At times they may play music during the scan to help with relaxation. CT (Computerized Tomography) A CT scan uses a sophisticated x-ray machine combined with a computer to create a picture of the inside of the body. 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. When it is time for the scan, a technologist positions the patient on a movable padded table. The table inches slowly through a donut-shaped scanner ring, stopping about every half-inch for a picture to be taken. During the scan, the technologist watches through a glass window and talks to the patient on an intercom. The sound of motors and gears can be heard as the scanner takes the pictures. A CT scan normally takes less than an hour. PET (Positron Emission Tomography) A PET scan provides a picture of brain activity. It may be used when trying to tell whether a CT or MRI scan is showing the return of a tumor or tissue damage caused by radiation. PET may be used in addition to CT or MRI. SPECT (Single Photon Emission Tomography) SPECT provides information similar to the PET scan, but is more widely available. Biopsy A biopsy is a procedure in which a sample of tumor tissue is removed so that doctors can study its characteristics. The sample can be taken through an open or surgical biopsy, or a needle biopsy. If the tumor is difficult or impossible to resect (remove) safely, a doctor may perform a biopsy to identify the type of tumor and determine what treatment would be most useful. If the tumor is surgically resectable, tissue removed during the resection process can be examined to confirm the diagnosis. 6 Pediatric Brain Tumor Foundation

TREATMENT FOR JPA Treatment for a JPA often begins with surgery. The surgeon s goals are to biopsy the tumor and to resect (remove) as much of the tumor as possible with minimal harm to the patient. Because a JPA is usually a well-defined, distinct capsule, complete removal is achieved in many cases. However, if the tumor has grown into an area that is difficult or impossible to reach safely, such as the brain stem, radiation therapy (RT) or chemotherapy may be used following surgery to destroy the remaining tumor cells. Chemotherapy may be advised, depending on how much of the tumor has been removed, the age of the child, the location of the tumor, and whether the tumor has spread. Surgery To remove a JPA, the pediatric neurosurgeon must first gain access to the brain. This is usually done by craniotomy or craniectomy. Cranio means skull. Otomy means surgical incision. Ectomy means surgical removal. For a craniotomy, the surgeon creates a bone flap in the skull which is removed and put back in place when the operation is over. For a craniectomy, the bone is removed in pieces and not replaced. During surgery, the doctor may find that the JPA is blocking the cerebrospinal fluid (CSF) pathway. In most cases, removing the tumor will correct the problem. Sometimes, however, even when the CSF pathway is open, it does not function properly. In that case, the surgeon may insert a small plastic tube called a ventriculostomy or external ventricular drain (EVD) to avoid fluid buildup around the time of surgery. Later, a more permanent internal shunt may be inserted to keep the fluid circulating around the brain. Another way to keep the fluid from building up is to create a small hole in the floor of the third ventricle (a third ventriculostomy). Sometimes steroids are given to reduce swelling before, during, and after surgery. The brain is a complex, sensitive organ, and doctors and parents must understand and deal with the risks of brain surgery. Despite these risks, surgery may be essential to remove as much of the tumor as possible. Studies show that two out of every ten children who have brain surgery may develop serious aftereffects. Examples include loss of speech, balance difficulties, hormonal disorders, or growth disorders. These problems may last for weeks, months, or longer. Speech therapy, physical therapy, or occupational therapy can help restore function to its maximum potential. Pediatric Brain Tumor Foundation 7

Staging Staging determines if a tumor has spread beyond the site of its origin and if so, to what extent? To stage a tumor, the doctor reviews scans taken before and after surgery, and may also analyze samples of cerebrospinal fluid (CSF) obtained through a spinal tap. The extent of surgical removal is also used to stage a tumor. Surgeons commonly designate surgical resection as being total or near total, subtotal, partial, or biopsied only. Total resection means no visible residual tumor although there are always some tumor cells left behind. Near total means a small amount (less than 10%) of residual tumor left behind. Subtotal means 50%-90% tumor removal and partial means less than 50% tumor removal. Ask your doctor to more fully explain the term being used to describe the stage of your child's tumor. Doctors usually do not stage a JPA. JPAs generally do not spread to other sites and staging is not meaningful in most cases. Radiation Therapy (RT) Radiation therapy, sometimes called radiotherapy, uses x-rays to attempt to destroy tumor cells. RT is highly effective for JPA, but is used selectively for tumors that are progressive after surgery or that are symtomatic but can not be removed surgically due to the extent and/or location of the tumor. RT is usually not needed if a JPA is completely removed. The side effects of RT vary from child to child and are most related to the dose received by the brain and the age of the child. Radiation effects can be temporary or permanent, occurring during radiotherapy or not until months or years later. Problems that can occur during radiation therapy include fatigue, nausea or diminished appetite, skin changes (like sunburn), hair loss, or soreness swallowing. RT can cause low white blood cell counts (leaving the child susceptible to infection) or low levels of platelets (important for clotting and healing). Your radiation oncologist can often suggest ways to relieve some of the symptoms seen during radiotherapy. Later effects of RT include: RT to the entire brain can cause learning problems noted several years after therapy. The effects of irradiation on learning or on memory are quite variable, but in general are more pronounced when higher doses are used in younger children. When the disease presentation permits lower radiation doses to the brain, 8 Pediatric Brain Tumor Foundation

the likelihood of encountering later learning difficulties and the degree of the deficits are both diminished. Changes in growth can result from direct effects on spinal bone growth or more commonly from a reduction in growth hormone. Growth hormone, or GH, is an important substance produced in the pituitary gland. GH is often diminished when measured a year or more after radiation therapy. In a child without evidence of disease, growth hormone can be replaced as a medicine. Changes in other hormones, including thyroid hormone and sometimes the hormones responsible for sexual maturation during puberty, can be noted at or beyond the one-year post-therapy point. When hormone levels are inadequate, the hormones can be replaced with medication. Chemotherapy Chemotherapy is the use of a single medicine or combination of medicines to attack tumor tissue. Chemotherapy can be taken by mouth, by injection, or through an intravenous line (IV). Some therapies can be given on an outpatient basis, while others require one or more days in the hospital. A JPA may be treated with chemotherapy if the tumor cannot be completely resected (removed) surgically and the disease seems to be progressing. Chemotherapy may be particularly useful if the doctor wishes to delay or avoid RT. If your child requires chemotherapy, it is helpful to keep the following two facts in mind: (1) children do not normally experience all possible side effects, and (2) there are ways to relieve or offset many of them. Among the side effects caused by chemotherapy are hair loss, skin problems, nausea, vomiting, diarrhea, constipation, changes in taste, fatigue, mouth sores, seizures, hearing loss, kidney and liver problems, and reduction of platelets, red cells, or white cells in the blood. Remember: some of these problems may not occur at all. Some may be temporary, while others may be permanent. Research is ongoing in an attempt to improve the effectiveness and reduce the negative side effects of RT and chemotherapy. If you are interested in learning about clinical trials or studies that are testing new approaches, ask your doctor, contact the National Cancer Institute at http://www.cancer.gov, call the PBTF at 1-800-253-6530, or e-mail: familysupport@pbtfus.org. Pediatric Brain Tumor Foundation 9

PROGNOSIS AND OUTCOME The likely outcome, or prognosis, for a child with a brain tumor depends on many factors, including: The age of the child. The child s overall health. How much the tumor has already affected the child s ability to function. Degree of metastasis, especially the extent to which the tumor has already spread when first found. Degree of surgical resection. The type of treatment received. When a JPA is completely resected, long-term results are excellent and complete cures are possible without RT or chemotherapy. Relative survival rates are currently 95 percent at two years, over 90 percent at five years, and over 88 percent at ten years in people under age 19 at the time of diagnosis. (CBTRUS) WHEN A TUMOR RETURNS The majority of JPAs that have been completely resected do not recur. Nevertheless, regular follow-up with MRI or CT scans is recommended for several years following surgery. If only part of the tumor is removed (as in near total, subtotal, or partial resection), recurrence is more likely. If tumor progression is discovered, RT or chemotherapy may be recommended. IN THE LONG TERM More children than ever are surviving brain tumors. Having survived, however, they and their families must often deal with long-term effects caused by the tumor or by the therapy used to treat the tumor. Among the possible issues are: decreased levels of growth hormone decreased levels of thyroid hormone delayed growth reduced ability to think and reason impaired vision or hearing physical disabilities fatigue depression personality and attitude changes hydrocephalus seizures 10 Pediatric Brain Tumor Foundation

As distressing as these side effects can be, it is important to remember that untreated children have little chance of survival. The choice to do what it takes to extend life carries with it a choice to accept the risk that complications may occur. Your best defense is a good offense. Educate yourself. Seek out other people who are going through similar experiences, or who have already been down the road you are traveling. Be prepared. Don t hesitate to ask questions, express concerns, and seek more explanation when needed. Many of the problems caused by brain tumors or treatment can be offset. Hormone Replacement Therapy (HRT) is used to overcome hormone deficiencies. Physical therapy and occupational therapy are helpful to children with impaired coordination. If a child has difficulty talking, speech therapy may be useful. Specialists can address vision and hearing problems. The whole family can benefit from social support programs, including individual, couples or group counseling. The Pediatric Brain Tumor Foundation offers Family Support Programs. For more information on family support programs offered by the Pediatric Brain Tumor Foundation call (800) 253-6530 or e-mail: familysupport@pbtfus.org. Even if there are no apparent complications, a child who has had a brain tumor must be monitored closely. Regular MRI or CT scans may be recommended by your doctor for several years following surgery. Your doctor may also recommend hormone evaluation and neuropsychological testing. Talk with your doctor about post-treatment monitoring of your child s tumor. Your child may sustain late effects from the JPA itself and/or from treatments for the disease. After completing treatment, there will be concern for appropriate reintegration into school. This will require school placement and interventions to deal with the child s particular learning needs. It is important for parents to work with educators, the medical care team, and the child to identify individual needs, which can then be addressed with an Individualized Education Plan (IEP). Information is available about this process from the PBTFUS at (800) 253-6530 or e-mail: familysupport@pbtfus.org. Ongoing interaction will help to assure that your child s needs are addressed. Consultation with a neuropsychiatrist or neuropsychologist may be helpful in evaluating learning needs. Pediatric Brain Tumor Foundation 11

CONCLUSION: HOPE FOR TODAY AND TOMORROW By now, you have probably discovered that when you are dealing with a brain tumor, you do not travel in a straight line from beginning to end. Instead, one step forward can be followed by two steps backward, followed by three steps forward, and so on. An attempt to treat one complication may create other problems, which then must be solved. Yet you also experience small triumphs along the way, and you are always aware that a major victory may be just around the corner. As you continue on this difficult journey, we encourage you to ask questions and express your concerns. At the same time, seek out people and places that give you energy and hope. Only when you are nourished can you give of yourself to those who need you. We are making progress against juvenile pilocytic astrocytoma. Every study and clinical trial increases our understanding of this tumor s origin and behavior, improving our ability to destroy it. One day, we will learn how to prevent it. And with each advance, more and more children will be able to enjoy a long and productive life. 12 Pediatric Brain Tumor Foundation

Pediatric Brain Tumor Foundation Reference Library The following resource literature and archived Internet Conference audio compact discs are available to patient families, medical professionals and social services specialists at no charge by calling (800) 253-6530 or by e-mailing familysupport@pbtfus.org. 1. Questions For Your Medical Care Team When Your Child Has a Brain Tumor 2. Basic Facts About Pediatric Brain and Spinal Cord Tumors 3. Basic Facts About Medulloblastoma/PNET 4. Basic Facts About Juvenile Pilocytic Astrocytoma 5. Basic Facts About Astrocytoma 6. Basic Facts About Glioma 7. Basic Facts About Ependymoma 8. Helping Hand National Newsletter 9. Caring Hand National Newsletter 10. Informed Parent Internet Series - The Importance of a Multi-Disciplinary Approach to Treating Children with Brain Tumors 11. Informed Parent Internet Series - The Clinical Trials Process 12. Informed Parent Internet Series - School Re-entry Following the Diagnoses and Treatment of Your Child s Brain Tumor 13. Informed Parent Internet Series - Healing the Family 14. Informed Parent Internet Series - Growth and Development: Endocrine Issues Facing Pediatric Brain Tumor Survivors 15. Informed Parent Internet Series - Post Traumatic Stress: Helping Families Survive Childhood Cancer 16. Informed Parent Internet Series - Siblings Issues: The Impact of Cancer on Healthy Siblings 17. Informed Parent Internet Series - Brothers & Sisters & Brain Tumors: A Child s Point of View of Coping with Cancer in the Family 18. Informed Parent Internet Series - Combining Curative and Pallative Care for Children with Brain Tumors Pediatric Brain Tumor Foundation 302 Ridgefield Court Asheville, NC 28806 (828) 665-6891 (828) 665-6894 (fax) (800) 253-6530 e-mail familysupport@pbtfus.org research@pbtfus.org donations@pbtfus.org ride4kids@pbtfus.org website http://www.pbtfus.org