BASIC FACTS ABOUT ASTROCYTOMA 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 Henry S. Friedman, MD, The Brain Tumor Center, Duke University Medical Center, Durham, NC for 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 Surveillance, Epidemiology, and End Results (SEER) program of the National Cancer Institute. 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 review with your doctors and medical institutions to attempt to seek the most current information available. COPYRIGHT Copyright 2001 by the Pediatric Brain Tumor Foundation of the United States. 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 as well as the most up-to-date methods. There are medical centers that specialize in the treatment of pediatric brain tumors. We have much to learn about childhood brain tumors. Funding research to find causes and cures, increasing public awareness, and aiding in the early detection and treatment of childhood brain tumors are all ways the PBTFUS fulfills its mission. Families like yours are at the heart of that mission. If you need further information or other PBTFUS publications, a social worker is on staff to take your call. Contact us at 1-800-253-6530 or e-mail: socialworker@pbtfus.org. 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 to help determine the best treatment approach. The more aggressive and dangerous a tumor is, the higher the grade it is 2 Pediatric Brain Tumor Foundation of the United States
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. Malignant tumors invade and destroy healthy tissue. Grade III and IV tumors are malignant with increasing severity. ASTROCYTOMA What is 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. Astrocytomas (astrocytic tumors) arise from starshaped neural glial cells called astrocytes, which are found in the nervous system. In children, common locations include the brain stem, cerebrum, and cerebellum. Astrocytomas also arise deep within the brain in the basal ganglia, thalamus, hypothalamus, and third ventricle. Ventricles are hollow areas in the brain that contain cerebrospinal fluid, or CSF, a clear, watery liquid that bathes and cushions the brain and spinal cord. There are a total of four ventricles in the brain; all are connected to each other. The thalamus surrounds the third ventricle. cerebrum thalamus (third ventricle) basal ganglia tentorium hypothalamus brain stem cerebellum Astrocytomas can be low-grade (grade I or II), midgrade (grade III), or high-grade (grade IV). Low-grade astrocytomas are well-differentiated, less malignant, and slow-growing. Mid-grade astrocytomas, often called Pediatric Brain Tumor Foundation of the United States 3
anaplastic astrocytomas, contain cells with malignant traits. High-grade astrocytomas are fast growing, invasive, and very malignant. Most variants of astrocytoma are capable of progressing to a malignant phase. The most aggressive astrocytoma is glioblastoma multiforme. They are rare in children. Names of specific types of astrocytomas are based on location and other characteristics. For example, cerebellar astrocytomas occur in the cerebellum. There are two types of cerebellar astrocytomas. Pilocytic cerebellar astrocytomas often have malignant features but usually behave in a benign fashion (a separate booklet on juvenile pilocytic astrocytoma is available from the PBTFUS). Another is diffuse or fibrillary cerebellar astrocytomas which are more likely to develop into an anaplastic (malignant) form, spreading into surrounding areas of the brain. These tumors are able to change into more malignant forms, the most aggressive and dangerous being glioblastoma multiforme. Astrocytomas that do not fit into any other established category are grouped together and called Astrocytoma, NOS. The letters NOS stand for Not Otherwise Specified. What are the symptoms of an astrocytoma? Increased intracranial pressure (ICP) is the main symptom, but other symptoms can occur, depending on the exact location of the tumor. The cerebellum aids in coordinating voluntary movements and maintains balance and muscular tone. These are the functions most affected by a tumor in that area. An astrocytoma in the cerebrum is likely to cause headaches, nausea, vomiting, weakness of arms or legs, visual disorders, or seizures. Other symptoms may include paralysis of a single part of the body or one side of the body, tremors, hormonal abnormalities, or diabetes insipidus. Hydrocephalus, an abnormal accumulation of cerebrospinal fluid (CSF) within the brain, may occur. 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 is astrocytoma in children? Astrocytomas represent about one-third of all brain tumors in people under age 19. Juvenile pilocytic astrocytomas represent a little more than 15 percent of all pediatric brain tumors, occurring most often in children between the ages of five and nine. They occur at the same rate in males and females. Anaplastic astrocytomas and glioblastomas are considerably less 4 Pediatric Brain Tumor Foundation of the United States
common in children than in adults but equally common in males and females. (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 an astrocytoma, but biopsy is the only reliable way to confirm a specific diagnosis. All tests may not be necessary in the diagnosis of your child; your medical care team will make the best decision for your child. DIAGNOSTIC METHODS MRI (Magnetic Resonance Imaging) MRI Suite 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 Pediatric Brain Tumor Foundation of the United States 5
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 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. 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. 6 Pediatric Brain Tumor Foundation of the United States
Biopsy A biopsy is a procedure in which a sample of tumor tissue is removed so 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. TREATMENT FOR ASTROCYTOMA Treatment for an astrocytoma usually begins with surgery. The surgeon s goal is to resect (remove) as much of the tumor as possible with minimal harm to the patient. The location of the tumor and level of risk must be taken into account. Treatment can also be difficult because certain regions of the tumor may be sensitive to a specific treatment, while other regions of the same tumor are resistant. Radiotherapy (RT) and chemotherapy may be used in an effort to destroy tumor cells not removed during surgery. Chemotherapy is usually not considered unless the age of the child makes the risks of RT unacceptable. Surgery To remove an astrocytoma, 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 astrocytoma is blocking the cerebrospinal fluid (CSF) pathway. In some cases, removing at least a portion of 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. Pediatric Brain Tumor Foundation of the United States 7
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. 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 or bone marrow obtained through a bone marrow needle biopsy. 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. Radiation Therapy (RT) Radiation therapy, sometimes called radiotherapy, uses x-rays to attempt to destroy tumor cells. In cases where a tumor cannot be completely resected, survival rates improve when RT is used following surgery. 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. 8 Pediatric Brain Tumor Foundation of the United States
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, 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 at the base of the brain. 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. An astrocytoma may be treated with chemotherapy to destroy cells that remain after surgical removal of the tumor. Chemotherapy is particularly useful if the patient is a child under three years old and 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. Pediatric Brain Tumor Foundation of the United States 9
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://cancernet.nci.gov, call the PBTFUS at 1-800-253-6530, or e-mail: socialworker@pbtfus.org. 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. Exact location of the tumor. How much the tumor has already affected the child s ability to function. Degree of surgical resection. The type of treatment received. In people diagnosed before age 19, the two-year relative survival rate for pilocytic astrocytoma is 95 percent; the five-year rate is nearly 91 percent; and the ten-year rate is close to 89 percent. Anaplastic astrocytoma has a two-year relative survival rate of almost 63 percent in children, with a five-year rate of about 53 percent and a ten-year rate of almost 49 percent. Relative survival rates for glioblastoma in people diagnosed before age 19 are 30 percent at two years, almost 20 percent at five years, and 17 percent at ten years. (CBTRUS 1992-1997; based on SEER data, 1973-1996) Prognosis for recovery from an astrocytoma is usually poorest when the brain stem is involved. WHEN A TUMOR RETURNS 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: LIVING WITH ASTROCYTOMA 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. 10 Pediatric Brain Tumor Foundation of the United States
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 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 of the United States offers a Family Support Program that includes establishing family support groups. For more information on family support groups in your area or starting a family support group, contact the Pediatric Brain Tumor Foundation of the United States at 1-800-253-6530 or e-mail: socialworker@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 astrocytoma 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 Pediatric Brain Tumor Foundation of the United States 11
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 1-800-253-6530 or e-mail: socialworker@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. 12 Pediatric Brain Tumor Foundation of the United States
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 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. Pediatric Brain Tumor Foundation of the United States 13
Pediatric Brain Tumor Foundation of the United States 302 Ridgefield Court Asheville, NC 28806 (828) 665-6891 (828) 665-6894 (fax) (800) 253-6530 e-mail socialworker@pbtfus.org research@pbtfus.org donations@pbtfus.org ride4kids@pbtfus.org websites http://www.pbtfus.org http://www.ride4kids.org This publication made possible through a generous grant from the Janirve Foundation.