Ewing Sarcoma Patient & Family Handbook

Ewing Sarcoma Family of Tumors

A Handbook for Patients and Families • Oncology Series

Ewing Sarcoma Family of Tumors

A Handbook for Patients and Families

Authors Amy R. Newman, MSN RN CPNP

Parent Reviewer Melissa Oberst

Content Reviewers Steering Council

This handbook is published by the Association of Pediatric Hematology/Oncology Nurses (APHON) for educational purposes only. The material has been developed by sources believed to be reliable. The material is not intended to represent the only acceptable or safe treatment of Ewing sarcoma . Under certain circumstances or conditions, additional or different treatment may be required. As new research and clinical experience expand the sources of information available concerning the treatment of Ewing sarcoma, adjustments in treatment and drug therapy may be required. APHON makes no warranty, guarantee, or other representation, express or implied, concerning the validity or sufficiency of the treatments or related information contained in this handbook. APHON Handbooks for Patients and Families have been developed by healthcare providers who have been educated and currently practice primarily in the United States. APHON acknowledges that treatments and practices presented in this handbook, which are commonly or widely recognized as standard in the United States, may differ from those in other countries. APHON encourages users to defer to their governing body’s preferred treatment and practice methods and healthcare providers’ advice. APHON provides this handbook for educational use. Users may copy and distribute this material in unadapted form only, for noncommercial use, and with attribution given to APHON.

WHAT IS EWING SARCOMA? Ewing sarcoma is a type of cancerous tumor arising from the bone or soft tissues. Ewing sarcoma belongs to a group of tumors called the Ewing sarcoma family of tumors (EFT). EFT consists of • Ewing sarcoma of the bone • extraosseous Ewing sarcoma (EES) • Askin tumor • primitive neuroectodermal tumor (PNET) of the bone • PNET of the soft tissue. Under the microscope, tumors in this family appear similar and are made up of small round blue cells. These tumors are generally evaluated and treated the same way. To simplify the discussion, the term Ewing sarcoma will be used throughout this handbook to refer to any one of these tumors. In 1921, Dr. James Ewing became the first to describe Ewing sarcoma. After osteosarcoma, Ewing sarcoma is the most common type of childhood bone cancer. It is usually found in the pelvic bones, the upper arm, the spine, the ribs, or the femur (one of the long bones of the leg), but it can occur in any bone in the body. Ewing sarcoma found in the soft tissues around the bone is called extraosseous or extraskeletal Ewing sarcoma (EES). EES is most commonly found in the thigh, pelvis, spine area, chest wall, or foot. Ewing sarcoma found in the chest wall may be referred to as an Askin tumor . PNET is the least common member of EFT. It is also a round-cell tumor, but it is made up of young nerve cells. PNET can occur in the bone or soft tissues.

WHAT ARE SOME OF THE SIGNS AND SYMPTOMS OF EWING SARCOMA?

The most common symptom of Ewing sarcoma is pain at the site of the tumor. The pain may wake your child up from sleep. Swelling or a soft mass may appear around the affected bone or tissue. If the tumor is located in the pelvis, signs of bowel or bladder disturbance may appear. If the tumor is located in the spine, the patient often experiences back pain, weakness in the arms or legs, or numbness. If the tumor is located in the chest wall, symptoms may include cough, shortness of breath, or chest pain. A pathological fracture (a break that occurs without trauma) is often present at diagnosis because the tumor has weakened the bone. It is important to understand that trauma did not cause the cancer. Children frequently have painful lumps as a result of normal play, so Ewing sarcoma usually is diagnosed only after a lump has persisted for several months. Other less common symptoms of Ewing sarcoma are weight loss and fever.

WHAT CAUSES EWING SARCOMA? We do not know what causes Ewing sarcoma. We know that Ewing sarcoma is not contagious—that is, it cannot be caught from another person. For many adult cancers, lifestyle-related risk factors play a large role—but no behaviors or lifestyle habits, including those practiced during the mother’s pregnancy, have been associated with the development of Ewing sarcoma. Studies of children with Ewing sarcoma have not found links to radiation, chemicals, or other environmental factors, nor is Ewing sarcoma caused by too much or too little of a particular food group in a child’s diet.

WHO GETS EWING SARCOMA? Approximately 200–250 children in the United States are diagnosed with Ewing sarcoma each year. Ewing sarcoma is found mostly in adolescents 10–20 years of age. This group accounts for a little more than 1% of all childhood cancers. About 30% of all Ewing sarcomas occur in adults over the age of 20. Most patients are white, and males are affected more often than females. Ewing sarcoma is extremely rare among those of Asian or African descent. IS EWING SARCOMA INHERITED? We have no evidence that Ewing sarcoma is genetic or inherited from a family member. It is rare for more than one person in a family to have this type of tumor. WHAT IS METASTASIS? Metastasis refers to the spread of a tumor from its original location, or the primary site, to other parts of the body. Metastases are found in approximately 30% of patients with Ewing sarcoma at the time of their diagnosis. Half of the metastases are usually found in the lungs, and half are in other places—often other bones. Metastasis in the bone marrow (inside of the bones where blood cells are made) is less common. Some signs and symptoms of metastases include fever, pain, weight loss, and fatigue. WHAT IS STAGING? Staging is the process of determining the location and amount of the cancer at the time of diagnosis. Staging for Ewing sarcoma is based on the location of the tumor and whether it has spread. A tumor is staged as either localized or metastatic. A localized tumor is one that has not spread to another part of the body; a metastatic tumor is one that has spread. The method of treatment proposed for your child will depend on the stage of the disease at the time of diagnosis.

WHAT TESTS AND PROCEDURES WILL MY CHILD NEED? To diagnose Ewing sarcoma and determine the extent of your child’s disease, a number of tests and procedures are needed. A member of your child’s healthcare team will take a complete history of the illness, which includes a determination of the location, duration, and intensity of any pain. A physical examination will be performed to check for a limp, a limited range of motion in the joints, or any soft-tissue masses or lumps. In addition, blood tests and radiographic imaging studies will be needed as described below. X RAY

An X ray is a simple procedure that uses low levels of radiation to create pictures of the tissues, organs, and bones inside the body. Standard X rays, which might be called plain films , are taken of areas that might be affected by your child’s condition. Children have to be still for a moment to have X rays taken, but X rays don’t cause pain. To minimize exposure to radiation, a special gown or vest will shield parts of your child’s body that aren’t being imaged, and you may be asked to step out of the room or wear similar protection when the X ray is taken.

MRI MRI (magnetic resonance imaging) uses radio waves and magnetic fields instead of X rays to create very detailed pictures of parts of the body. A computer translates the pattern of radio waves into images. MRI images are so clear that they are sometimes more useful than X rays or CT scans. This is true for areas of the brain and spinal cord, blood vessels, bones, and some organs of the body. Certain tumors are regularly checked by MRI during and after treatment to determine how effective the treatment is and if a relapse has occurred. CT SCAN A CT (computed tomography) scan is a computer-assisted X ray that creates detailed pictures of internal organs and tumors. It used to be called a CAT (computerized axial tomography) scan. The CT machine is a doughnut- shaped X-ray camera that moves around a table that the patient lies on. Every second, the camera moves a very small distance around the patient while taking an X ray. A computer puts these X rays together to help the doctors see a cross-sectional 3-D image from all angles. CT scans are very helpful in diagnosing and following the stage and location of cancer. CT scans are very useful in diagnosing tumors, infections, blood clots, and areas of abnormal bleeding. Some tumors are regularly checked by CT scan during and after treatment to see how effective the treatment has been and if a relapse has occurred. CT scans are painless, but the patient must lie still during the scan. Some children require sedation to help them lie still. If your child’s belly is being scanned, they may be asked to drink a dye that allows organs to be seen more clearly. Some patients may be given a small amount of dye through an intravenous (IV) line, which puts the dye right into their veins. The dye can cause a warm, flushing feeling. Generally, there are no side effects from either type of dye; however, allergic reactions are possible. Because there is some radiation exposure with this test, your child’s healthcare team will try to minimize how often it is done. BONE SCAN A bone scan is used to look for abnormalities in bones. It can be helpful in diagnosing primary bone tumors, cancers that have spread to bones, or infections in the bone. A small amount of radioactive dye (isotope) is given through an IV line and travels around the body. This isotope is picked up by tumor cells, which allows for the tumor’s location in the body to be seen. Pictures are taken about 2–3 hours after the injection. The test is painless, but some children require sedation to lie still for the scan. The radioactive isotope leaves the body in the urine over 2–3 days. There is very little radiation exposure from a bone scan. PET SCAN A PET (positron emission tomography) scan can tell the medical team a lot about how cells of the body are working. It shows the chemical and functional changes within the body. Because functional changes take place before physical changes, PET scans can help diagnose cancer early. A PET scan is very helpful in diagnosing specific cancers, especially lymphomas and some solid tumors. In addition, often it can determine if enlarged lymph nodes contain cancer cells and can provide information about the stage or extent of the cancer. A PET scan is also helpful when determining the effectiveness of treatment or if a relapse has occurred and may be able to detect relapse before an MRI or CT can. For the PET scan, a glucose solution (a type of sugar solution) that contains a slightly radioactive substance is given through an IV line. The child must wait, usually 30–60 minutes, for the solution to travel throughout the body. Certain cells absorb the glucose solution more than others, and they light up in pictures taken during the PET scan. The body eventually gets rid of the glucose solution naturally, and no special precautions are needed. The PET scanner is a doughnut-shaped camera (very similar to a CT scanner) with a table that the patient lies on. The patient will move through the doughnut-shaped camera, while lying on the table, a few different times during the scan. A PET scan is painless but may take a while. The child must be able to lie still; some children may require sedation. Some food and medications may interfere with PET scan results, so your child will be given special diet instructions before the day of the scan.

TUMOR BIOPSY A tumor biopsy is the removal of a small amount of tissue to be looked at under a microscope. This test is needed to confirm the diagnosis and decide what the best treatment is. The biopsy most often is performed in the operating room by a surgeon who removes an enlarged lymph node or a small part of a tumor. In some medical centers the biopsy is done in the radiology department, where a needle is guided into the lymph node or tumor to obtain the biopsy sample (fine needle aspiration biopsy). In other cases, a surgical incision is made. Patients often are sedated for biopsies; your child’s comfort and anxiety level will always be considered in planning for this procedure. BLOOD TESTS Blood tests help diagnose medical problems and monitor for side effects of treatment. A specially trained nurse or technician uses a needle to collect blood from a vein. The blood samples are collected in tubes that are sent to a laboratory to be closely examined. In some cases, a finger prick may give enough blood for a test. These tests are usually done at the time of diagnosis to rule out other diseases and serve as a baseline for your child. Complete Blood Count Blood is made up of plasma (the liquid part of the blood) and cells floating in the plasma. There are three types of blood cells: white blood cells, red blood cells, and platelets. The white blood cells fight infection as part of the body’s immune system. The red blood cells carry oxygen throughout the body. The platelets stop bleeding by rushing to the site of an injury to form a clot. A complete blood count , or CBC, is a blood test that measures the amount and health of these three types of cells. The CBC is useful in diagnosing blood disorders. It also is used to diagnose cancers of the blood system, such as leukemia. Your child’s CBC results may change with each test because of other things going on in the body, such as infections or nutritional problems, or in response to medications like chemotherapy. Blood Chemistry A blood chemistry test measures the balance of fluid, electrolytes, and blood sugar. These measurements can provide information about the health and function of the kidneys and liver and about the body’s metabolism; they can also help diagnose certain diseases, such as diabetes. They can indicate how a disease is responding to treatment and monitor for possible side effects. Blood tests may be done to • measure creatinine and blood urea nitrogen (BUN) to monitor changes in kidney function • detect changes in liver function by measuring bilirubin • measure erythrocyte sedimentation rate (ESR) and levels of C-reactive protein (CRP), which are sometimes elevated by certain cancers. BONE MARROW ASPIRATION AND BIOPSY A bone marrow biopsy is a test done to determine whether tumor cells are in the bone marrow. A needle is inserted into the marrow inside the bone. Some of the bone marrow (the biopsy) and the fluid in the bone marrow (the aspirate) are collected. It is usually necessary to obtain bone marrow samples from both hip bones. Patients undergoing bone marrow aspirations and biopsies may experience pain, so children often are sedated for this procedure. If your child is uncomfortable after the test, a mild pain medication such as acetaminophen (Tylenol ® ) may be given, but not aspirin or ibuprofen (Motrin ® or Advil ® ). A specially trained doctor (a pathologist or hematologist) examines the biopsy and aspirate under a microscope to see if there is Ewing sarcoma detected. If the bone marrow is positive for Ewing sarcoma, it may be checked periodically during treatment to determine response to the treatment. Other tests—including immune-phenotyping, cytogenetics, and fluorescent in situ hybridization (FISH)—also may be done to detect Ewing sarcoma. A bone marrow aspirate and biopsy also is used to evaluate the white blood cells, red blood cells, and platelets to see that they are being produced normally.

ECHOCARDIOGRAM AND ELECTROCARDIOGRAM Echocardiogram , often referred to as a cardiac echo or simply an echo, is an ultrasound of the heart. An echocardiogram reveals the size and shape of your child’s heart and how well the heart is functioning. This serves as a baseline evaluation because some chemotherapy drugs can weaken the heart muscle. Echocardiograms will be repeated throughout your child’s treatment and beyond to monitor for any signs of heart dysfunction. An electrocardiogram , or EKG, is a recording of the electrical activity of the heart. Electrodes , which are soft stickers connected to wires, are attached to the surface of the skin on your child’s chest. Impulses from the heart are sensed through the electrodes and recorded or displayed by a machine placed next to the patient. This is another way to measure how well the heart is working, and it will be repeated throughout your child’s treatment. VENOUS ACCESS DEVICE A venous access device (VAD), sometimes called a central venous catheter or central line, is a temporary or long-term intravenous (IV) tube that can be used for the duration of your child’s therapy (keep in mind that “therapy” can be used interchangeably with “treatment”). It is placed in a large vein in the chest or the arm. These long, flexible catheters empty into or near the heart, allowing treatments to be given within seconds. They can be used to administer medications, fluids, chemotherapy, blood products, and nutritional support as needed and to draw blood for testing. A surgery is performed to insert the VAD, and your child will be sedated or given general anesthesia for the procedure. The VAD will be placed either before chemotherapy begins or within the first month of treatment. Not all children will need a VAD. This will be determined by you and your child’s healthcare team. The VAD will be removed when it is no longer needed. There are different types of VADs, such as an implanted port inserted under the skin near the collarbone; a tunneled central venous catheter (Broviac or Hickman) that has a tube; a lumen (either single or double), which comes out of your child’s chest; or a PICC (peripherally inserted central catheter) line, a central line inserted through a vein in the arm. Your child’s healthcare team will discuss each type with you and help you choose the best device for your child’s treatment. Each type of catheter requires slightly different care. A port requires almost no care at home, but a special needle needs to be inserted through the skin into the port each time it is used. Tubing can then be attached to the needle if needed to give medication or IV fluids. An external central line requires routine changes to the dressing that covers the exit site of the line and keeps it in place to prevent infection and to care for the skin around the tube. The external lines also must be routinely flushed with a solution to keep them working properly. You will be taught how to care for the device at home.

HOW IS EWING SARCOMA TREATED? Three types of therapy are commonly used to treat Ewing sarcoma: surgery, chemotherapy, and radiation. The type of therapy chosen depends upon the age of your child, the location of the cancer, and the extent of the disease. In most cases, a combination of chemotherapy, surgery, and radiation is needed. Ewing sarcoma is radiosensitive, so radiation may be used if no surgical option is available. Radiation also may be needed if microscopic cells are left behind after surgery. Your child’s healthcare team will talk with you about the best treatment for your child. You, your child, and the healthcare team then will make decisions about your child’s treatment.

CHEMOTHERAPY Chemotherapy includes several medicines that kill cancer cells, shrink tumors, and prevent cancer cells from spreading. Ewing sarcoma can spread early in your child’s illness, making chemotherapy an important part of your child’s treatment. Several chemotherapy medications can kill the cells that cause Ewing sarcoma, but no single chemotherapy medicine alone can control the cancer. As a result, chemotherapy medications are usually given in combination. Each cycle of treatment usually lasts several days. The treatment may be given in the hospital, which requires an overnight stay, or in an outpatient clinic, which requires daily visits to the clinic. Your child’s healthcare team will discuss with you the specific chemotherapy medications your child will receive and how and when they will be given. Most medications are given through the veins or a VAD. Your child’s healthcare team also will explain the possible side effects of the specific chemotherapy treatment that your child will receive. SURGERY It is sometimes possible to remove the entire tumor by surgery at the time of diagnosis. More commonly, Ewing sarcoma requires a combination of chemotherapy and surgery, and possibly radiation therapy. Typically treatment starts with chemotherapy. Chemotherapy may shrink the size of the tumor, which helps to decrease the risk of surgical injury to the healthy tissues surrounding the tumor. When the surgeon removes the tumor, the goal is to take out the entire tumor with a small amount of healthy surrounding tissue, called the margin. This reduces the chance that the tumor will regrow in this area. Once the tumor is removed, a pathologist will look at it under a microscope to ensure that no tumor cells are at the edge of the margin. If tumors cells are seen at the margin, cells may have been left behind in your child’s body, and radiation therapy will likely be needed. Your medical team will discuss these results and decisions with you. If your child’s cancer is metastatic or if tumors are in certain difficult-to-access locations, surgery may not be an option. RADIATION Radiation therapy is a very precise kind of X-ray treatment. Radiation may be used alone or in combination with surgery, depending on the location of the Ewing sarcoma. It is given in carefully measured amounts by radiation therapy experts. If radiation is necessary for your child, the radiation therapy doctor will discuss with you exactly how the radiation will be given and how long the treatments will last. Radiation therapy is usually given once daily, Monday through Friday, for several weeks in a row. If your child is young or unable to stay still, daily sedation may be required to assist your child through the radiation experience. During radiation some children become more tired than usual or have decreased appetites or nausea. Irritation to the skin within the radiation field may occur. Other possible side effects will be discussed in detail with you and your child prior to the start of therapy. ARE THERE CONCERNS TO ADDRESS PRIOR TO INITIATING TREATMENT? Yes. Short- and long-term side effects may occur as a result of treatment. Therefore, before starting any chemotherapy, surgery, or radiation, it is important to obtain baseline information about your child’s health and organ function. Some potential long-term side effects include the following: • Damage to the muscle of the heart—Doxorubicin is a chemotherapy agent that may cause damage to the heart muscle. It is important to obtain a baseline echocardiogram to establish baseline function and to evaluate whether it is safe to proceed. Your child’s healthcare team should inform you about possible heart-protectant medications.

• Disturbance in sexual development and adult infertility—Ifosfamide and cyclophosphamide, which comes from a class of chemotherapy drugs called alkylating agents, may cause disturbance in sexual development and infertility as an adult. Boys are affected more than girls. Methods to bank female eggs or male sperm should be explored when it is age appropriate. These methods should be reviewed with your child’s healthcare team. Your healthcare team also will review if it is safe to postpone the start of chemotherapy for these fertility procedures. Information concerning infertility and fertility preservation may be found on the LiveStrong website at Fertile Hope website at https://www. livestrong.org/what-we-do/program/fertility and in APHON’s Plan Now, Parent Later resources, available at https://aphon.org/education/patient-family-resources . • Weakened bones and impaired growth—Radiation may have long-term side effects on bones exposed to radiation. Side effects include weakening and slowed or limited bone growth. This can result in differences in the lengths of the extremities, as well as altered function of the affected extremity. Your child’s healthcare team will discuss with you ways to improve bone health and how to compensate for limitations in bone growth. • Hardware failure and infection—If your child underwent surgery to remove the tumor from a bone, internal hardware (also called a prosthetic) was likely placed inside your child’s body. Internal prosthetics generally need to be revised in the future through surgical procedures to account for the child’s growth. Internal prosthetics also come with a lifelong potential risk of infection and hardware failure, which require close follow-up care with your surgeon. For children undergoing amputation, external prosthetics often are used to aid in function and mobility. External prosthetics also require future changes to be made to accommodate the child’s growth and for other reasons.

HOW LONG WILL MY CHILD’S THERAPY LAST? Treatment for Ewing sarcoma typically lasts about 8–10 months. After treatment is finished, frequent physical exams and imaging are required for several years to monitor for the return of the cancer and for any side effects of treatment. Yearly follow-ups will be needed throughout your child’s life to monitor for possible late side effects from treatment, such as heart and lung problems, slowed or decreased growth and development of the bones, changes in sexual development and ability to have children, changes in intellectual ability or learning problems, and development of secondary cancers.

WHAT NEW METHODS OF TREATMENT EXIST? Most advances in the treatment of childhood cancer have been made through processes called clinical trials. During clinical trials, the best known, standard treatment for a particular cancer is compared with a new, experimental treatment. This experimental treatment is believed to be at least as good as, and possibly better than, the standard treatment. Clinical trials allow doctors to determine whether promising new treatments are safe and effective. Participation in clinical trials is voluntary. Because clinical trials involve research into new treatment plans, all risks and side effects cannot be known ahead of time. However, children who participate in clinical trials can be among the first to benefit from new treatment approaches. Before making a decision about your child’s participation in a clinical trial, you should discuss the potential risks and benefits with your child’s healthcare team.

COMPLEMENTARY AND ALTERNATIVE THERAPIES Complementary therapy refers to medicines or modalities that are used along with the recommended treatment. Many complementary therapies are safe and may help relieve symptoms or side effects, ease pain, provide relaxation, and help your child enjoy life. Examples include herbal medications, massage, music, dance, acupuncture, meditation, and yoga. Consult your child’s healthcare team prior to starting any of these therapies. It is especially important to prevent possible harmful interactions between chemotherapy and herbs. Alternative therapies are sometimes used in the place of standard medical care. These therapies have not been tested in clinical trials and have not been proven safe or effective. If you chose an alternative therapy method for your child before seeking standard medical care, the delay in treatment may give the tumor time to grow, lowering your child’s chances of being cured. HOW CAN I WORK WITH MY CHILD’S HEALTHCARE TEAM? The care of your child requires a team approach. You are a key player on your child’s healthcare team. You know your child better than anyone else, and your input is important. Other members of your child’s healthcare team include doctors, nurses, pharmacists, and social workers.

Always communicate openly with the members of your child’s healthcare team. If there is anything you are not sure about, ask the team. Write down your questions as you think of them, so you’ll remember to ask them during your next meeting with your healthcare team. HERE ARE SOME QUESTIONS YOU MIGHT WANT TO ASK: • What stage is the disease, and what does that mean? • What treatment choices are available? • What treatment do you recommend and why? • What are the risks or side effects during the treatment? • What are the risks and side effects after the treatment? • What are my child’s chances for survival? • What are the chances that the disease or condition will recur? Use this space to write down additional questions.

ARE MY FEELINGS NORMAL, AND WHAT CAN I DO ABOUT THEM? Hearing that your child has cancer is shocking and overwhelming. Many parents say, “I didn’t remember anything after the words ‘Your child has cancer.’” Parents often feel numb and have a hard time

believing the diagnosis. It is important to know that this is normal and expected. Most families have difficulty processing all of the information the healthcare team is providing in the beginning. However, over time, the information will be repeated and absorbed. Many families feel somehow responsible for their child’s disease. Feelings of guilt are also common because they could not protect their child from illness or because of the amount of time it took to

diagnose the child. This disease is not caused by anything that you did or did not do. The cause of childhood cancer is not known. It often takes time for the cancer diagnosis to be made because symptoms are often the same as those of many childhood illnesses. Feelings of sadness, anger, and helplessness about your child’s diagnosis also are common. These feelings are normal. Each member of the family may express these emotions in different ways and at different times. Talking honestly with each other about these feelings, emotions, and reactions will help everyone in the family. Keep in mind that there is no right or wrong way to feel. Each member of the family needs the chance to express their feelings when they are ready and in their own way. Talking to friends, family, and members of the healthcare team can be difficult at times—however, sharing your feelings can help you cope. Your child will benefit from family and friends showing their care through communication and support. HOW CAN I HELP MY CHILD? Children often think that something they did caused their cancer. You can help your child by reinforcing that this is not the case. Make sure your child understands that your feelings of anger and sadness are directed at the cancer, not at them. This will help you keep a close relationship with your child. Your child will need to share their feelings with someone that they trust, and they may choose someone other than a parent to avoid upsetting the parent, which is OK. Don’t be afraid to ask your child about their feelings—it may be what your child is waiting for. Keep in mind that your child is still a child first in spite of the disease. As your child goes through treatment, remember that they still have all the needs of a growing child. All children need love, attention, the opportunity to learn and try new skills, and limits. Do not avoid talking to your child about therapy. Use explanations that your child will understand. Children tolerate treatment better if they understand it and are allowed to help make decisions about their care, when appropriate. The things children can imagine on their own are often more frightening than what is actually happening. As a parent you may find it difficult to watch your child go through treatment. Your child may sometimes seem sicker during treatment than before treatment. Your feelings about what your child is going through must be balanced with the knowledge that treatment provides the chance of curing the disease and making it possible for your child to live a full and meaningful life. It’s difficult to accept how cancer and treatment change your child, but most of these changes, like hair loss, are temporary. Despite outward changes, your child is still the same person on the inside. Most healthcare centers have a psychosocial team that can support you and your child through their illness.

IS MY CHILD’S DIET IMPORTANT DURING TREATMENT? Yes, your child’s diet is important. Research has shown that well- nourished children tolerate treatment better and experience fewer treatment delays. Nausea and vomiting can be distressing side effects of treatment, and your child’s healthcare team will prescribe medications to help prevent and treat these side effects. Your child may have difficulty eating normal meals during treatment. Foods that are appealing to your child will be tolerated better. Offer foods that are high in calories, protein, and carbohydrates. Small frequent meals are often better tolerated than three larger meals. When possible, choose more nutritious foods. Your child’s nurse or dietitian can help you put together a list of possible food choices.

If your child is on steroid medications, such as prednisone or Decadron (dexamethasone), their appetite will likely be increased. These medicines may make your child feel constantly hungry and crave unusual foods. Your child may want one particular food constantly. Try to keep healthy snacks on hand. Steroids may cause your child to gain weight and develop a round face and puffy appearance, but these side effects are temporary. It is important to offer your child fluids to drink frequently throughout the day to avoid dehydration. Signs of dehydration include decreased urine output, dizziness, and a dry mouth. Multivitamins, herbs, and other supplements should be avoided unless approved by your child’s healthcare team because they can interact with your child’s treatment. Please ask your healthcare team if you are considering any of these types of supplements. A dietitian may be a member of your child’s healthcare team. Dietitians are trained in the calorie and nutritional needs of children. The dietitian can provide you with education regarding your child’s dietary needs and ideas about how to meet these needs during treatment. Your child’s healthcare team will monitor your child’s nutritional status, height, and weight during and after treatment to try to prevent problems. When a concern about nutritional problems arises, your child’s healthcare team will discuss with you the ways to manage and improve your child’s nutrition.

CAN MY CHILD ATTEND SCHOOL DURING TREATMENT? Your child’s ability to go to school during therapy will depend on the intensity of the treatment and your child’s

response to the treatment. There may be extended periods when your child is unable to go to school because of hospitalizations or treatments. However, it is important that your child keep up with schoolwork, even during these times. Federal laws protect children with disabilities or illnesses to ensure that they receive the same education as their peers. Talk with your child’s healthcare team and school staff about these laws. Then work with them in arranging for a tutor or finding other ways to help your child stay on track with schoolwork.

Discuss with the healthcare team your child’s ability to attend school. Some children are able to attend school between hospitalizations or treatments. Many hospitals and clinics have resources such as educational liaisons to assist in coordinating school services and educating teachers and classmates to prepare them for your child’s return to school.

RESOURCES CureSearch for Children’s Cancer. (2022). Ewing sarcoma in children . https://curesearch.org/ Ewing-Sarcoma-in-Children National Cancer Institute. (2020). Ewing sarcoma treatment (PDQ ® )—Patient version . https://www.cancer.gov/ types/bone/patient/ewing-treatment-pdq#_1 American Cancer Society. Ewing family of tumors . h ttps://www.cancer.org/cancer/ewing-tumor.%20html INFORMATION ON CLINICAL TRIALS National Cancer Institute. (2016). Taking Part in Cancer Treatment Research Studies . Publication No. 16-6249. https://www.cancer.gov/publications/patient-education/crs.pdf National Cancer Institute. (2020). Deciding to take part in a clinical trial . https://www.cancer.gov/ about-cancer/treatment/clinical-trials/taking-part

IMPORTANT PHONE NUMBERS

NOTES

8735 W. Higgins Road, Suite 300 Chicago, IL 60631 847.375.4724 • Fax 847.375.6478

info@aphon.org www.aphon.org

Tumores de la Familia del SARCOMA DE EWING

Manual para pacientes y familiares • Serie Oncología

A Handbook for Patients and Families Tumores de la familia del SARCOMA DE EWING

Autora Amy R. Newman, MSN RN CPNP

Revisor por parte de los padres Melissa Oberst

Revisor de contenido Consejo Directivo

La Association of Paediatric Hematology/Oncology Nurses (APHON) publica este manual únicamente con fines educativos. El material ha sido desarrollado por fuentes que se consideran confiables. El material no pretende representar el único tratamiento aceptable o seguro del sarcoma de Ewing. Bajo ciertas circunstancias o condiciones, se puede requerir un tratamiento adicional o diferente. A medida que las nuevas investigaciones y la experiencia clínica amplíen las fuentes de información disponibles sobre el tratamiento del sarcoma de Ewing, es posible que se requieran ajustes en el tratamiento y la farmacoterapia. APHON no ofrece ninguna garantía, seguridad u otra representación, expresa o implícita, con respecto a la validez o suficiencia de los tratamientos o la información relacionada contenida en este manual. Los manuales APHON para pacientes y familiares han sido desarrollados por proveedores de atención médica con formación y que actualmente ejercen en los Estados Unidos, principalmente. APHON reconoce que los tratamientos y prácticas presentados en este manual, comúnmente o ampliamente reconocidos como estándar en los Estados Unidos, pueden diferir de los de otros países. APHON alienta a los usuarios a referirse a los métodos de tratamiento y prácticas preferidos de su organismo rector y al consejo de los proveedores de atención médica. APHON proporciona este manual para uso educativo. Los usuarios pueden copiar y distribuir este material sólo en su forma no adaptada, para uso no comercial y con atribución otorgada a APHON.

¿QUÉ ES EL SARCOMA DE EWING? El sarcoma de Ewing es un tipo de tumor canceroso que se origina en los huesos o en los tejidos blandos. Pertenece a un grupo de tumores denominado tumores de la familia del sarcoma de Ewing (EFT) que consiste en • Sarcoma de Ewing del hueso • Sarcoma de Ewing extraóseo (EES) • Tumor de Askin • Tumor neuroectodérmicos primitivo (PNET) del hueso • PNET de partes blandas. Bajo el microscopio, los tumores de esta familia parecen similares y están formados por pequeñas células redondas de color azul. Estos tumores generalmente se evalúan y tratan de la misma manera. Para simplificar, el término sarcoma de Ewing se utilizará a lo largo de este manual para referirse a cualquiera de estos tumores . En 1921, el Dr. James Ewing describe por primera vez este sarcoma. Después del osteosarcoma, es el tipo de cáncer de hueso más común en niños. Puede desarrollarse en los huesos pélvicos, la parte superior del brazo, la columna vertebral, las costillas o el fémur (uno de los huesos largos de la pierna), de hecho, se puede presentar en cualquier hueso del cuerpo. Al sarcoma de Ewing ubicado en los tejidos blandos alrededor del hueso se le llama extraóseo o extraesquelético (EES). Y se localiza más comúnmente en el muslo, la pelvis, el área de la columna vertebral, la pared torácica o el pie. El sarcoma de Ewing que se encuentra en la pared torácica puede denominarse tumor de Askin . El PNET es el miembro menos común de la EFT. Es un tumor de células redondas, pero está formado por células nerviosas jóvenes y puede presentarse en el hueso o en los tejidos blandos .

¿CUÁLES SON ALGUNOS DE LOS SIGNOS Y SÍNTOMAS DEL SARCOMA DE EWING?

El síntoma más común es dolor en el sitio del tumor. El dolor puede despertar a tu hijo(a) del sueño. Puede aparecer una hinchazón o una masa blanda alrededor del hueso o tejido afectado. Si está en la pelvis, pueden aparecer signos de alteración intestinal o vesical. Si está en la columna, se puede experimenta dolor de espalda, debilidad en los brazos o piernas o entumecimiento. Si está en la pared torácica, los síntomas pueden incluir tos, dificultad para respirar o dolor en el pecho. A menudo hay una fractura patológica (sin un traumatismo) al momento del diagnóstico debido a que el tumor ha debilitado el hueso. Los traumatismos no causan el cáncer. Los niños suelen tener bultos dolorosos como resultado del juego, por lo que el sarcoma de Ewing generalmente se diagnostica sólo después de que el bulto haya persistido durante varios meses. Otros síntomas menos comunes son la pérdida de peso y la fiebre.

¿QUÉ CAUSA EL SARCOMA DE EWING? No se sabe qué causa el sarcoma de Ewing, pero se sabe que no es contagioso, o sea que no se puede contraer de otra persona. En muchos tipos de cánceres en adultos, los factores de riesgo relacionados con el estilo de vida juegan un papel importante, pero ningún comportamiento o hábito, incluidos los practicados durante el embarazo, se ha asociado con el desarrollo de este sarcoma. Los estudios en niños con sarcoma de Ewing no lo vinculan con la radiación, productos químicos u otros factores ambientales; tampoco con el exceso o insuficiencia de un grupo de alimentos en particular en la dieta del niño .

¿QUIÉN PUEDE CONTRAER SARCOMA DE EWING? Unos 200 a 250 niños en los EE.UU. son diagnosticados con sarcoma de Ewing cada año. Se presenta sobre todo en adolescentes de 10 a 20 años de edad, grupo que representa poco más de 1% de todos los cánceres infantiles. Cerca del 30% de todos los sarcomas de Ewing se dan en adultos mayores de 20 años. La mayoría de los pacientes son blancos, afecta más a hombres que a mujeres, y es muy raro entre los descendientes de asiáticos o africanos. ¿EL SARCOMA DE EWING ES HEREDITARIO? No hay evidencias de que el sarcoma de Ewing sea genético o que se herede de un miembro de la familia. Es raro que más de una persona en la familia tenga este tipo de tumor. ¿QUÉ ES UNA METÁSTASIS? Metastasis se refiere a la diseminación de un tumor desde su ubicación original, o sitio primario, a otras partes del cuerpo. Se presentan en alrededor del 30% de los pacientes con sarcoma de Ewing en el momento de su diagnóstico. En general, el 50% de las metástasis se localizan en los pulmones y el otro 50%, en otros sitios, a menudo otros huesos. La metástasis en la médula ósea (dentro de los huesos donde se producen las células sanguíneas) es menos común. Algunos signos y síntomas incluyen fiebre, dolor, pérdida de peso y fatiga. ¿QUÉ ES LA ESTADIFICACIÓN? Estadificación es el proceso de indica la ubicación y cantidad de cáncer en el momento del diagnóstico. La estadificación del sarcoma de Ewing se basa en la ubicación del tumor y si es que se ha propagado. Un tumor se clasifica como localizado o metastásico. El localizado es el que no se ha diseminado a otra parte del cuerpo; el metastásico es el que se ha diseminado. El tratamiento de tu hijo(a) dependerá de la etapa de la enfermedad en el momento del diagnóstico.

¿QUÉ PRUEBAS Y PROCEDIMIENTOS NECESITARÁ MI HIJO(A)? Para diagnosticar el sarcoma de Ewing y determinar el alcance de la enfermedad, se necesitan varias pruebas y procedimientos. Una persona del equipo de atención médica realizará un historial completo de la enfermedad para determinar la ubicación, la duración y la intensidad de cualquier dolor. Se hará un examen físico para ver si hay cojera, movimiento limitado en las articulaciones o cualquier masa o bulto en los tejidos blandos. Además, se realizarán análisis de sangre y estudios de imágenes radiográficas, como se describe a continuación. RAYOS X

Es un procedimiento simple que utiliza bajos niveles de radiación para crear imágenes de los tejidos, órganos y huesos dentro del cuerpo. Se toman rayos X estándar, llamados también placas simples, de las áreas que pudieran estar afectadas por la condición de tu hijo(a), quien debe permanecer quieto por un momento para que se tomen los rayos X, pero éstos no causan dolor. Para que la exposición a la radiación sea menor, un vestido o chaleco especial protegerá las partes del cuerpo que no están siendo analizadas, y es posible que te pidan que salgas de la habitación o que uses una protección similar.

RESONANCIA MAGNÉTICA (MRI) La MRI usa ondas de radio y campos magnéticos en lugar de rayos X para crear imágenes detalladas de partes del cuerpo. Una computadora traduce el patrón de ondas de radio en imágenes tan claras que a veces son más útiles que las de los rayos X o de las de CT, especialmente para las áreas del cerebro y la médula espinal, los vasos sanguíneos, los huesos y algunos órganos del cuerpo. Ciertos tumores se revisan regularmente durante y después del tratamiento para determinar cuán efectivo es el tratamiento y si se ha producido una recaída. TOMOGRAFÍA COMPUTARIZADA (CT) La CT son rayos X asistidos por computadora que crea imágenes detalladas de órganos y tumores internos. Solía llamarse CAT (tomografía axial computarizada). La máquina CT es una cámara de rayos X en forma de dona que se mueve alrededor de una mesa en la que se encuentra el paciente. La cámara se mueve cada segundo una distancia muy pequeña alrededor del paciente mientras toma las imágenes. Una computadora las reune para ayudar a los médicos a ver una imagen 3-D transversal desde todos los ángulos. La CT es muy útil para diagnosticar y seguir la etapa y ubicación del cáncer, al igual que para diagnosticar tumores, infecciones, coágulos de sangre y áreas de sangrado anormal. Algunos tumores se revisan regularmente durante y después del tratamiento para ver cuán efectivo ha sido el tratamiento y si se ha producido una recaída . La tomografía computarizada es indolora, pero el paciente debe permanecer quieto durante la exploración. Algunos niños requieren sedación para permanecer quietos. Si se escanea el vientre de tu hijo(a), deberá beber un tinte que permite ver los órganos con mayor claridad. Algunos pacientes reciben el tinte a través de una línea intravenosa (IV) que va directamente a las venas. El tinte puede causar una sensación de calor y enrojecimiento pero, en general, no hay efectos secundarios con ninguno de los dos tipos de tinte; aunque, las reacciones alérgicas son posibles. Se minimizará la frecuencia de la prueba por la exposición a la radiación. GAMMAGRAFÍA ÓSEA Se usa para buscar anomalías en los huesos. Diagnostica tumores óseos primarios, cánceres que se han propagado a los huesos o infecciones óseas. Se da una pequeña cantidad de tinte radiactivo (isótopo), vía intravenosa, que viaja por todo el cuerpo. El isótopo es recogido por las células tumorales, señalando la ubicación del tumor. Las imágenes se toman alrededor de 2 a 3 horas después de la inyección. La prueba es indolora, pero algunos niños son sedados durante la exploración para que no se muevan. El isótopo radiactivo sale por la orina durante 2 a 3 días. Hay muy poca exposición a la radiación en una gammagrafía ósea. PRUEBA PET (TOMOGRAFÍA POR EMISIÓN DE POSITRONES) Una PET le dice mucho al equipo médico sobre cómo funcionan las células del cuerpo, mostrando los cambios químicos y funcionales dentro del él. Ya que los cambios funcionales se dan antes que los físicos, las pruebas PET ayudan a diagnosticar el cáncer de manera temprana. La PET ayuda a diagnosticar cánceres específicos, especialmente linfomas y algunos tumores sólidos. Además, puede determinar si los ganglios linfáticos agrandados contienen células cancerosas y proporcionar información sobre el estadio o la extensión del cáncer. También ayuda a determinar la eficacia del tratamiento o si se ha producido una recaída, detectándola antes de que se pueda realizar una MRI o una CT. Para la PET, se administra por vía intravenosa una solución de glucosa (un tipo de solución de azúcar) con una sustancia ligeramente radiactiva. Se debe esperar, generalmente de 30 a 60 minutos, para que la solución se desplace por todo el cuerpo. Algunas células absorben la solución de glucosa más que otras y se iluminan en las imágenes tomadas durante la prueba PET. Con el tiempo, el cuerpo se deshace de la solución de glucosa de forma natural y no se necesitan precauciones especiales. El aparato para la prueba PET es una cámara en forma de dona (parecido a un escáner CT) con una mesa sobre la cual está acostado el paciente, que se moverá a través de la cámara varias veces durante la exploración. La PET es indolora, pero puede tardar un poco y algunos niños requieren ser sedados para permanecer quietos. Hay alimentos y medicamentos que pueden interferir con los resultados de la PET, por lo que tu hijo(a) recibirá instrucciones dietéticas antes del día de la exploración.

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