Rhabdomyosarcoma and Soft Tissue Sarcoma
A Handbook for Patients and Families • Oncology Series
Rhabdomyosarcoma and Soft Tissue Sarcoma A Handbook for Patients and Families
Author Kelly Laschinger, MSN RN CPNP CPHON®
Parent Reviewer Krisann Stenz
Reviewers Steering Council
Previous Contributors Margaret Bottcher, MN RN CPNP CPON® Violet Shen, MD
David Tishler, MD Ann Watts, RN CNP
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 rhabdomyosarcoma and soft tissue 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 rhabdomyosarcoma and soft tissue 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.
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WHAT ARE RHABDOMYOSARCOMA AND SOFT TISSUE SARCOMA? Rhabdomyosarcoma , also called rhabdo, is a cancer that develops from muscle cells (rhabdomyoblasts). It can arise from muscles in many different areas of the body, most commonly the head and neck, arms, legs, bladder, prostate gland, and vagina. Other, less common sites are the chest, abdomen, and genital and anal area. Rhabdomyosarcoma accounts for more than half of all soft tissue sarcomas diagnosed in children, making it the most common soft tissue tumor in children. The type of rhabdomyosarcoma is determined by looking at a piece of the tumor under the microscope. The most common types are embryonal and alveolar rhabdomyosarcoma. The term soft tissue sarcoma is generally used to refer to a cancer that arises from soft tissue other than muscle, such as connective tissue, fibrous tissue, blood vessels, nerves, and joint membrane tissue. Soft tissues connect, support, and surround other body parts and organs. Soft tissue sarcoma is rare in infants and children.
WHAT ARE SOME OF THE SIGNS AND SYMPTOMS OF RHABDOMYOSARCOMA AND SOFT TISSUE SARCOMA?
The signs and symptoms of rhabdomyosarcoma and soft tissue sarcoma depend on the location of the tumor and its effect on the surrounding organs and tissue. For example, if the tumor is located in the head or neck, there may be swelling around the eye ( proptosis ), facial nerve weakness, ear drainage, headache, facial pain, a lump in the neck, or signs of sinusitis. If the tumor is located in the arm or leg, there may be a tender or enlarged area in or around the muscle. Tumors in the bladder may cause bloody urine ( hematuria ) or difficulty urinating. Paratesticular tumors usually cause painless enlargement of the scrotum or groin area. Vaginal tumors may cause vaginal discharge or bleeding.
WHAT CAUSES RHABDOMYOSARCOMA AND SOFT TISSUE SARCOMA? At this time, it is not known what causes pediatric rhabdomyosarcoma and soft tissue sarcoma. We do know, however, that the tumors are not contagious and cannot be caught from another person. No behaviors or lifestyle habits, including those of the child’s mother during pregnancy, have been associated with the development of these tumors. The cancer is not caused by the presence or absence of something (such as vegetables or vitamins) in the child’s diet.
IS RHABDOMYOSARCOMA OR SOFT TISSUE SARCOMA INHERITED? Sometimes, but rarely, rhabdomyosarcoma is associated with a syndrome that has been passed down through a family (an inherited familial syndrome) such as Li-Fraumeni syndrome, neurofibromatosis type I, Beckwith- Wiedemann syndrome, cardiofaciocutaneous syndrome, and Costello syndrome. If a familial syndrome is a suspected cause of your child’s cancer, your child will be referred to a genetic specialist for further evaluation that may include bloodwork.
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WHO GETS RHABDOMYOSARCOMA OR SOFT TISSUE SARCOMA? Rhabdomyosarcoma accounts for approximately 4% of all childhood cancers diagnosed before the age of 15 years. Approximately 350 cases of rhabdomyosarcoma are diagnosed each year in the United States. The majority of children are younger than 9 years of age at the time of diagnosis. Tumors of the bladder and vagina occur more often in infants and young children; tumors of the trunk, arms, and legs are more likely to affect older children and adolescents. Rhabdomyosarcoma is slightly more common in boys than girls. Soft tissue sarcoma is rare in children, accounting for 3% of all childhood cancers, but it can occur at any age. It may be associated with other hereditary conditions or with radiation exposure. WHAT TESTS AND PROCEDURES WILL MY CHILD NEED? A number of tests and procedures may be used to diagnose rhabdomyosarcoma or soft tissue sarcoma and to determine the extent of your child’s disease. 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 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 are often sedated for biopsies; your child’s comfort and anxiety level will always be considered in planning this procedure. CYTOGENETIC TESTING Cytogenetic testing may be another important part of the diagnostic process. Your healthcare team may ask your permission to perform cytogenetic testing on the tumor sample collected during biopsy. Cytogenetic testing includes evaluation of the genes or chromosomes that make up a tumor cell to seek out specific genetic abnormalities, including PAX3-FOXO1 and PAX7-FOXO1 gene fusions, to help determine the most effective treatment decisions and identify whether there are specific medications to target the tumor. Research is ongoing to improve rhabdomyosarcoma classification through molecular testing. BONE MARROW ASPIRATION AND BIOPSY A bone marrow aspirate and biopsy is a procedure done to remove a small sample of bone marrow, which is the factory that makes our blood cells. This test is done to determine if any cancer cells have spread to 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.
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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. Blood tests are used throughout treatment to monitor for possible side effects and your child’s response to treatment. 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 chemistry 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 BLOOD CULTURE Blood may be drawn for a blood culture if there is a suspected bacterial infection in your child’s blood. Laboratory staff observes the blood over time to see if bacteria grow. The blood culture can confirm a bacterial infection and give important information about the type of bacteria involved to help the medical team A lumbar puncture is a procedure done to obtain a small sample of cerebral spinal fluid (CSF), which is the fluid that surrounds and protects our brain and spinal cord. CSF is made continually by our bodies, so the small amount removed is quickly replaced. This test is done to determine if any cancer cells have spread to the brain or spinal cord or if there is an infection in the central nervous system. In a lumbar puncture, also referred to as a spinal tap, a needle is inserted into the back between the spaces of the spinal bones (vertebrae) to obtain a sample of CSF. The needle enters the spinal column below the spinal cord to avoid injuring the cord. This test can be uncomfortable, so some children are sedated to help them lie still. If a bone marrow biopsy is ordered or due at the same time as the lumbar puncture, these procedures will be done together to limit your child’s exposure to sedation. determine how to treat it. LUMBAR PUNCTURE
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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 or if a relapse has occurred. For an MRI, the patient lies on a table that is rolled into a machine. The machine surrounds them on all sides, like a tube. The MRI is painless, but the machine makes loud noises. Your child may use ear plugs or a headset during the MRI. They must be able to lie completely still, and some children require sedation to do this. The MRI usually takes 1–2 hours. Often, a dye is injected into a vein to improve the MRI images. Because they use strong magnets, MRI machines cannot have metal objects inside them. Before an MRI, your child will be asked to remove all metal objects such as jewelry and clothes with metal snaps or zippers. An MRI does not expose your child to any form of radiation. CT SCAN A CT (computerized or 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 useful 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 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, and 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 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 it may be able to detect relapse before an MRI or CT can. A glucose solution (a type of sugar solution) that contains a slightly radioactive substance is given through the child’s 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
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scan. The body eventually gets rid of the glucose solution naturally, and no special precautions are needed. The PET scan machine is a table surrounded by a doughnut-shaped camera that is moved up and down around the body. A PET scan is painless, but the test may take a while and 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. 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. CHEST X RAY Chest X rays have many uses. For instance, they can be used to look for enlarged lymph nodes inside the chest or lungs, to diagnose infections or spread of disease in the lungs, or to check the placement of tubes that were Ultrasound is a technique that uses the echoes from high-frequency sound waves to produce a picture of internal organs or tumors. It has many uses, including finding tumors in the belly, checking the health of the kidneys and bladder, and measuring the size of some organs (like the liver, gall bladder, and spleen). A type of ultrasound that looks at the way blood flows and the condition of the blood vessels, Doppler , can be used to diagnose clots in blood vessels. An ultrasound is a painless test that does not use radiation. The child lies on a table for a short time, and a clear jelly is smeared on the area being examined. (The jelly wipes off easily after the test.) A small, round handle is moved around on the jelly to get a clear picture of the tissue or organ being examined. Depending on the location of the ultrasound, the child may not be allowed to eat or drink for several hours or may need to have a full bladder before the test. WHAT IS STAGING? Staging is the process of determining the location of the tumor and amount of tumor present at diagnosis. Staging for rhabdomyosarcoma and soft tissue sarcoma is based on the location of the tumor, the size of the tumor, whether the tumor has spread from the primary site, and the type of tumor cell. If your child has surgery, the amount of tumor remaining after the surgery will be a factor in staging. The method of treatment proposed for your child will depend upon the stage of the disease at the time of diagnosis. Types of Rhabdomyosarcoma: • Embryonal: most common type, more common in younger children • Alveolar: second most common type, more common in adolescents • Other: spindle cell/sclerosing, pleomorphic/anaplastic, and mixed type are uncommon, and seen in less than 2% of cases in children put in for treatment. ULTRASOUND The staging of rhabdomyosarcoma is done in three parts. A stage, clinical group, and risk group will be assigned to your child’s rhabdomyosarcoma, as explained below. Your doctor can discuss the staging system for your child’s soft tissue sarcoma.
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STAGE OF RHABDOSARCOMA
CLINICAL GROUP
RISK GROUP
Stage I: The tumor is located in a “favorable” site such as the eye, head, neck, biliary tract or liver, or near the sex organs or bladder (not including the bladder or prostate) and may have spread to lymph nodes near the tumor but has not spread to other areas of the body. Stage II: The tumor is located in only one area (a location not included in Stage I), is smaller than 2 inches (5 centimeters) across, and may have spread to lymph nodes near the tumor but not to other areas of the body. Stage III: The tumor is located in only one area (a location not included in Stage I), is larger than 2 inches (5 centimeters) across, and may have spread to lymph nodes near the tumor but not to other areas of the body. Stage IV: The cancer has spread from the primary site and is found in more than one other place in the body at the time of diagnosis.
Clinical Group I: The tumor is confined to one area and has been completely removed with surgery; no lymph nodes were involved. Clinical Group II: The tumor and lymph nodes involved have been removed; there may be a very small amount of disease only seen with a microscope (microscopic disease) left after surgery. Clinical Group III: The tumor was only partially removed. Clinical Group IV: The cancer has spread from the primary site and is found in more than one other place in the body at the time of diagnosis.
Low Medium High Risk group assignment is determined by a combination of your child’s rhabdomyosarcoma stage, clinical group, and cytogenetic testing results.
HOW ARE RHABDOMYOSARCOMA AND SOFT TISSUE SARCOMA TREATED? Three types of treatment are commonly used to treat rhabdomyosarcoma and soft tissue sarcoma: surgery, chemotherapy, and radiation. The type chosen depends upon the type of tumor and the extent of the disease. Often, a combination of all three treatments is used, especially for rhabdomyosarcoma. The order of treatments depends on the disease’s signs and symptoms in your child. Surgery and radiation may be part of the initial therapy or may be delayed so that chemotherapy can shrink the tumor. Your child’s doctor will talk with you about the best treatment for your child, and decisions about treatment will be made by your family and the doctor. SURGERY Generally, the goal of surgery is to remove the entire tumor; this is called a total resection. Removing as much of the tumor as possible often can be done at the time of diagnosis. For some soft tissue sarcomas, surgery may be the only treatment required. Chemotherapy or radiation treatments may be given first to shrink very large tumors or tumors where surgical resection is not recommended due to potential impairment of body function or body image. Shrinking the tumor first can minimize the risk of surgical injury. A “second-look” surgery may be done after your child has undergone some treatment. This type of surgery can determine your child’s response to the treatment received so far and help the healthcare team plan any additional therapy.
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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” may 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 necessary 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. It 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 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 VAD for your child’s treatment. Each type of VAD requires slightly different care. A port requires almost no care at home because the port and catheter are under the skin, but a special needle is inserted through the skin into the port each time it is used. This is called “accessing the port.” Tubing can be attached to the needle if needed to give medication or IV fluids or to draw samples for testing. External VADs (such as a Broviac, Hickman, or PICC line) requires routine changes to the dressing (which covers the exit site of the line and keeps it in place to prevent infection) and to care for the skin around the tube. These external lines must be routinely flushed with a solution to keep them working properly. You will be taught how to care for the device at home. CHEMOTHERAPY Chemotherapy is a treatment of medicines that help kill cancer cells, shrink tumors, and prevent tumors from spreading. Several chemotherapy medications are effective in treating rhabdomyosarcoma and soft tissue sarcoma, but no single chemotherapy medicine can control this disease by itself. As a result, chemotherapy medications are usually given in combinations, through a vein. Treatment usually lasts several days and often is given in the hospital. Your child may receive some chemotherapy medicines that can be given in a clinic or at home. When chemotherapy is needed, close monitoring of the child is a priority. Your child’s healthcare team will explain the possible side effects of the specific chemotherapy treatment recommended for your child. RADIATION Radiation therapy is a special kind of X-ray treatment used in rhabdomyosarcoma and some soft tissue sarcomas. Radiation treatment is very precise and is given in strictly measured amounts by radiation therapy experts. If radiation is necessary for your child, the radiation oncologist will discuss with you exactly how the radiation will be given and how long the treatments will last. In general, children experience few side effects while they are receiving radiation therapy. Some children become more tired than usual or have decreased appetites. There can be delayed side effects, depending on which areas of the body are treated. The radiation oncologist will discuss possible side effects with you in detail. You may also choose to read the Radiation Therapy Handbook for Patients and Families, which is available on the APHON website. HOW LONG WILL MY CHILD’S THERAPY LAST? The length of treatment, or therapy, depends upon the type and stage of your child’s tumor at the time of diagnosis. Usually the treatment is given over several months, and it could last as long as a year. Follow-up blood tests and check-ups between chemotherapy treatments are usually done in the clinic or doctor’s office instead of at the hospital.
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HOW DO I LEARN ABOUT CLINICAL TRIALS? Most of the advances in the treatment of childhood cancer have been made through a process known as clinical trials. In clinical trials, the best known (standard) treatment for a particular cancer is compared with a new (experimental) treatment that 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. A clinical trial may or may not be offered to your child as a treatment option. You should ask your doctor about potential clinical trial options if you are interested in your child participating in a clinical trial. Participation in clinical trials is voluntary. On one hand, because clinical trials involve research into new treatment plans, all risks cannot be known ahead of time, and unknown side effects may occur. On the other hand, 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 and benefits with your child’s healthcare team. Learn more about clinical trials from the free booklet “Taking Part in Cancer Treatment Research Studies” from the National Cancer Institute. Get the booklet and useful information about childhood cancer at cancer. gov/publications/patient-education/cancer-treatment-research-studies . You also can find information on clinical trials at cancer.gov/about-cancer/treatment/clinical-trials . 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 child’s healthcare team. Important questions you may 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?
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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 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, 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 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
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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 children go through treatment, 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 that 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 diagnosis and treatment.
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 a distressing side effect 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 steroid medications, such as prednisone or dexamethasone (Decadron®) , are part of the treatment, your child’s 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 so that dehydration is avoided. 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 having your child take any of these types of supplements. A dietitian may be member of your child’s healthcare team. Dietitians are trained in the calorie and nutritional needs of children. The dietitian can provide you with information regarding your child’s dietary needs and give you 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 nutrition arises, your child’s healthcare team will discuss with you the ways to manage and improve your child’s nutrition.
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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.
WHAT IS MY CHILD’S PROGNOSIS? Prognosis is a word that doctors use to describe what they believe the outcome will be for a patient, including their chances of recovery. Your child’s cancer prognosis is as unique as he or she is. There are many factors that affect survival outcomes. A child’s age at diagnosis, the extent of disease, genetic factors, infections, nutrition, delays in the timing of treatment, and how well your child’s body can tolerate chemotherapy and radiation all play an important role in the child’s long-term outcome. Because general statements about survival rates are just numbers, please talk to your medical team about your child’s individual prognosis and plan of care.
ONLINE RESOURCES CureSearch: www.curesearch.org
National Cancer Institute’s Childhood Rhabdomyosarcoma Treatment (PDQ®)–Patient Version: https://www.cancer.gov/types/soft-tissue-sarcoma/patient/rhabdomyosarcoma-treatment-pdq National Cancer Institute’s Childhood Soft Tissue Sarcoma Treatment (PDQ®)–Patient Version: https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq
National Cancer Institute’s Taking Part in Cancer Treatment Research Studies: cancer.gov/publications/patient-education/cancer-treatment-research-studies National Cancer Institute’s Clinical Trials Information for Patients and Caregivers: cancer.gov/about-cancer/treatment/clinical-trials
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Rabdomiosarcoma y sarcoma de tejido bland o
Manual para pacientes y familiares • Serie Oncología
Rabdomiosarcoma y sarcoma de tejido bland o Manual para pacientes y familiares
Autores Kelly Laschinger, MSN RN CPNP CPHON®
Revisora por parte de los padres Krisann Stenz
Revisores Steering Council
Colaboradores anteriores Margaret Bottcher, MN RN CPNP CPON® Violet Shen, MD
David Tishler, MD Ann Watts, RN CNP
La Association of Paediatric Hematology/Oncology Nurses (APHON) publica este manual únicamente con fines educativos. El material ha sido desarrollado por fuentes consideradas confiables. El material no pretende representar el único tratamiento aceptable o seguro del Rabdomiosarcoma y del sarcoma de tejido blando. 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 Rabdomiosarcoma y del sarcoma de tejido blando, 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 proveedores de atención médica. APHON proporciona este manual únicamente 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.
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¿QUÉ SON EL RABDOMIOSARCOMA Y EL SARCOMA DE TEJIDOS BLANDOS? El rabdomiosarcoma , también llamado rabdo, es un cáncer que se desarrolla a partir de las células musculares (rabdomioblastos). Puede presentarse en los músculos de diferentes partes del cuerpo, más comúnmente en la cabeza y el cuello, brazos, piernas, vejiga, glándula prostática y vagina. Otros sitios menos comunes son el tórax, el abdomen y el área genital y anal. El rabdomiosarcoma representa más de la mitad de todos los sarcomas de tejidos blandos diagnosticados en niños, lo que lo convierte en el tumor de tejidos blandos más común en los niños. El tipo de rabdomiosarcoma se determina observando una parte del tumor bajo el microscopio. Los tipos más comunes son el rabdomiosarcoma embrionario y el alveolar. El Sarcoma de tejidos blandos se refiere a un cáncer que surge de tejidos blandos que no son músculos, como el tejido conectivo, tejido fibroso, vasos sanguíneos, nervios y tejido de membrana articular. Los tejidos blandos conectan, sostienen y rodean otras partes y órganos del cuerpo. Este sarcoma es raro en bebés y niños. ¿CUÁLES SON ALGUNOS SIGNOS Y SÍNTOMAS DEL RABDOMIOSARCOMA Y DEL SARCOMA DE TEJIDOS BLANDOS? Los signos y síntomas del rabdomiosarcoma y del sarcoma de tejidos blandos dependen de la ubicación del tumor y su efecto sobre los órganos y tejidos circundantes. Por ejemplo, si el tumor está en la cabeza o el cuello, puede haber hinchazón alrededor del ojo (proptosis), debilidad del nervio facial, oído con secreciones, dolor de cabeza, dolor facial, un bulto en el cuello o signos de sinusitis. Si está ubicado en el brazo o la pierna, puede haber un área sensible o agrandada en o alrededor del músculo. Con los tumores en la vejiga hay sangre en la orina (hematuria) o dificultad para orinar. Los tumores paratesticulares causan agrandamiento indoloro del escroto o del área de la ingle y los vaginales causan flujo o sangrado vaginal.
¿QUÉ CAUSA EL RABDOMIOSARCOMA Y EL SARCOMA DE TEJIDOS BLANDOS?
Por el momento, no se conocen las causas del rabdomiosarcoma ni el sarcoma de tejidos blandos pediátricos. Sin embargo, sabemos que los tumores no son contagiosos, o sea que no pueden pasarse de una persona a otra. No se han asociado comportamientos ni hábitos de vida, incluidos los de la madre durante el embarazo, con el desarrollo de estos tumores. El cáncer no es causado por la presencia o ausencia de algo (como vegetales o vitaminas) en la dieta de los niños. ¿EL RABDOMIOSARCOMA Y EL SARCOMA DE TEJIDOS BLANDOS SON HEREDITARIOS? A veces, pero muy rara vez, el rabdomiosarcoma se asocia con un síndrome que se ha transmitido de padres a hijos (un síndrome familiar hereditario), como el síndrome de Li-Fraumeni, la neurofibromatosis tipo I, el síndrome de Beckwith-Wiedemann, el síndrome cardiofaciocutáneo y el síndrome de Costello. Si se sospecha que un síndrome familiar es la causa del cáncer, se enviará a tu hijo(a) a un especialista en genética para una evaluación adicional que puede incluir análisis de sangre.
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¿QUIÉN CONTRAE RABDOMIOSARCOMA O SARCOMA DE TEJIDOS BLANDOS? El rabdomiosarcoma representa alrededor del 4% de todos los cánceres infantiles diagnosticados antes de los 15 años. Unos 350 casos de rabdomiosarcoma se diagnostican cada año en los Estados Unidos. La mayoría de los niños tienen menos de 9 años en el momento del diagnóstico. Los tumores de vejiga y vagina ocurren con más frecuencia en bebés y niños pequeños; es más probable que los tumores de tronco, brazos y piernas afecten a niños mayores y adolescentes. El rabdomiosarcoma es un poco más común en niños que en niñas. El sarcoma de tejidos blandos es raro en niños y representa el 3% de todos los cánceres infantiles, pero puede ocurrir a cualquier edad. Puede asociarse con otras condiciones hereditarias o con la exposición a la radiación. ¿QUÉ PRUEBAS Y PROCEDIMIENTOS NECESITARÁ MI HIJO(A)? Se pueden usar varias pruebas y procedimientos para diagnosticar rabdomiosarcoma o sarcoma de tejidos blandos y para determinar el alcance de la enfermedad de tu hijo(a). BIOPSIA TUMORAL La biopsia de una tumor es la extirpación de una pequeña parte de tejido para observarla con un microscopio. Esta prueba es necesaria para confirmar el diagnóstico y decidir cuál es el mejor tratamiento. La biopsia generalmente la realiza un cirujano que extirpa un ganglio linfático agrandado o una pequeña parte de un tumor. En algunos centros médicos, se realiza en el departamento de radiología, donde se guía una aguja hacia el ganglio linfático o el tumor para obtener la muestra de la biopsia ( biopsia por aspiración con aguja fina ). En otros casos, se realiza una incisión quirúrgica. Los pacientes son sedados para las biopsias; el nivel de comodidad y ansiedad de tu hijo(a) siempre se tomará en cuenta al planificar este procedimiento. PRUEBAS CITOGENÉTICAS Las pruebas citogenéticas son parte importante del proceso de diagnóstico. Con tu permiso, el equipo de atención médica realizará pruebas citogenéticas en la muestra del tumor obtenida durante la biopsia. Estas pruebas incluyen la evaluación de los genes o cromosomas que componen una célula tumoral para buscar anomalías genéticas específicas, incluida la fusión de los genes PAX3-FOXO1 y PAX7-FOXO1, para determinar el tratamiento más efectivo e identificar si hay medicamentos específicos para atacar el tumor. Se están realizando investigaciones para mejorar la clasificación del rabdomiosarcoma mediante pruebas moleculares. ASPIRACIÓN Y BIOPSIA DE MÉDULA ÓSEA La aspiración y biopsia de médula ósea es un procedimiento que se realiza para extraer una pequeña muestra de médula ósea, que es la que produce las células sanguíneas. Esta prueba se realiza para determinar si alguna célula cancerosa se ha propagado a la médula ósea. Se inserta una aguja en la médula dentro del hueso y se recolecta parte de la médula ósea (la biopsia) y del líquido de la médula ósea (el aspirado). En general, se obtienen muestras de médula ósea de ambos huesos de la cadera. Los pacientes pueden experimentar dolor durante el procedimiento, por lo que los niños son sedados. Si tu hijo(a) se siente incómodo/a después de la prueba, se le puede administrar un analgésico suave, como acetaminofén (Tylenol®).
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ANÁLISIS DE SANGRE Los análisis de sangre ayudan a diagnosticar problemas médicos y a controlar los efectos secundarios del tratamiento. Una enfermera o técnico capacitado usa una aguja para extraer sangre de una vena. Las muestras se recolectan en tubos y se envían a un laboratorio para ser examinadas. A veces, un pinchazo en el dedo da suficiente sangre para la prueba. Las pruebas se realizan en el momento del diagnóstico para descartar otras enfermedades y servir como referencia. Los análisis de sangre se usan durante todo el tratamiento para controlar los posibles efectos secundarios y la respuesta al tratamiento. HEMOGRAMA COMPLETO (CBC)
La sangre se compone de plasma (la parte líquida de la sangre) y de células que flotan en el plasma. Hay tres tipos de glóbulos: glóbulos blancos, glóbulos rojos y plaquetas. Los glóbulos blancos combaten las infecciones como parte del sistema inmunológico del cuerpo. Los glóbulos rojos transportan oxígeno por todo el cuerpo. Las plaquetas detienen el sangrado al ir al sitio de una lesión para formar un coágulo. Un conteo sanguíneo completo, o CBC, es un análisis de sangre que mide la cantidad y la salud de estos tres tipos de células. Es útil en el diagnóstico de trastornos de la sangre, así como en el de cánceres del sistema sanguíneo, como la leucemia. Los resultados de tu hijo(a) pueden cambiar cada vez debido a otras causas, como infecciones o problemas nutricionales, o a una respuesta a medicamentos como la quimioterapia. QUÍMICA SANGUÍNEA Una prueba de química sanguínea mide el equilibrio de líquidos, electrolitos y azúcar en la sangre. Estas mediciones dan información sobre la salud y el funcionamiento de los riñones y el hígado y sobre el metabolismo del cuerpo. También ayudan a diagnosticar ciertas enfermedades, como la diabetes. Indican cómo responde una enfermedad al tratamiento y monitorean los posibles efectos secundarios. Los análisis de química sanguínea se hacen para: • Medir la creatinina y el nitrógeno ureico en sangre (BUN) para controlar los cambios en la función renal • Detectar cambios en la función hepática midiendo la bilirrubina • Medir la tasa de sedimentación de eritrocitos (ESR) y los niveles de proteína C reactiva (CRP), que a veces son elevados por ciertos tipos de cáncer HEMOCULTIVO Se extrae sangre para un hemocultivo si se sospecha que hay una infección bacteriana en la sangre de tu hijo(a). El personal del laboratorio observa la sangre por un tiempo para ver si crecen bacterias. El hemocultivo puede confirmar una infección bacteriana y dar información importante sobre el tipo de bacteria involucrada para ayudar al equipo médico a determinar cómo tratarla. PUNCIÓN LUMBAR La punción lumbar se usa para obtener una muestra de líquido cefalorraquídeo (CSF), que es el líquido que rodea y protege nuestro cerebro y nuestra médula espinal. El cuerpo produce continuamente CSF, por lo que la cantidad obtenida se reemplaza rápidamente. Esta prueba sirve para saber si alguna célula cancerosa pasó al cerebro o a la médula espinal o si hay una infección en el sistema nervioso central. En la punción lumbar, también conocida como punción espinal, se inserta una aguja en la espalda, entre los espacios de los huesos de la columna (vértebras) para obtener una muestra de CSF. La aguja entra en la columna vertebral por debajo de la médula espinal para evitar lesionar la médula. Esto puede ser incómodo, por lo que se seda a los niños para que estén tranquilos. Si se debe hacer una biopsia de médula ósea, ésta se hará al mismo tiempo que la punción lumbar, para limitar la exposición de tu hijo(a) a la sedación.
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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. Para una MRI, el paciente se acuesta sobre una mesa, y una máquina en forma de tubo se desliza rodeándolo por todos lados. El procedimiento es indoloro, pero la máquina hace ruidos fuertes. Tu hijo(a) puede usar tapones para los oídos o auriculares durante la prueba. Algunos niños requieren sedación para estar completamente quietos. La MRI suele tomar de 1 a 2 horas. A veces, se inyecta un tinte en una vena para mejorar las imágenes. Como se usan potentes imanes, no puede haber objetos metálicos en el interior de la máquina, por lo que se le pedirá a tu hijo(a) que se quite todos los objetos metálicos, como joyería y ropa con broches o cremalleras de metal. La MRI no expone a tu hijo(a) a ninguna forma de radiación. TOMOGRAFÍA COMPUTARIZADA (CT) La tomografía computarizada (CT) es una radiografía asistida por computadora que crea imágenes de los órganos internos y los tumores. Solía llamarse exploración CAT (tomografía axial computarizada). La máquina de CT es una cámara de rayos X con forma de dona que se mueve lentamente alrededor de una mesa en la que se acuesta el paciente. Cada segundo, la cámara avanza alrededor del paciente tomando una radiografía. Estos rayos X se juntan para formar una imagen 3D transversal desde todos los ángulos. La CT es muy útil para diagnosticar y seguir el estadio y la ubicación del cáncer, así como para diagnosticar tumores, infecciones, coágulos de sangre y áreas de sangrado anormal. Algunos tumores se examinan periódicamente mediante una CT durante y después del tratamiento para ver qué tan efectivo ha sido y si ha ocurrido 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
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