Wilms Tumor Patient & Family Handbook

A Handbook for Families: Wilms Tumor

A Handbook for Families

Wilms Tumor

ONCOLOGY SERIES

A Handbook for Families

Wilms Tumor

ONCOLOGY SERIES

Wilms Tumor A HANDBOOK FOR FAMILIES

Edited by Deanna Hill, RN BSN CPHON®

Content Reviewers Steering Council

Parent Reviewers John Sullivan

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 Wilms tumor. 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 Wilms tumor, adjustments in treatment and drug therapy may be required. APHON makes no warranty, guarantee, or other representation, expressed or implied, concerning the validity or sufficiency of the treatments or related information contained in this handbook. 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.

n WHAT IS WILMS TUMOR? Wilms tumor is a cancer that develops from kidney cells. It is the most common childhood cancer of the kidneys. Wilms tumor can arise anywhere within the kidney. In rare cases, Wilms tumor can occur outside of the kidney. Not all of the kidney tumors found in children are Wilms tumors. Rhabdoid tumors and clear-cell sarcomas of the kidney are occasionally found in children and are more difficult to treat than Wilms tumor.

n WHAT ARE SOME OF THE SIGNS AND SYMPTOMS OF WILMS TUMOR? The most common sign of Wilms tumor is an enlarged or bloated belly in an otherwise healthy-looking child. Usually a caregiver notices the child’s enlarged belly or feels a mass in the belly while bathing or dressing the child. Some other general signs and symptoms are belly pain, tiredness, blood in the urine (usually found only by a urine test), and occasionally fever. One out of every four children diagnosed with Wilms tumor also has high blood pressure.

n WHAT CAUSES WILMS TUMOR? The cause of Wilms tumor isn’t known at this time. We do know that it is not contagious or genetic, so it cannot be passed from person to person. No behaviors or lifestyle habits, including those practiced during pregnancy, have been associated with the development of Wilms tumor. Cancer is not caused by anything the child ate or didn’t eat (such as vegetables or vitamins). n WHO GETS DIAGNOSED WITH WILMS TUMOR? Wilms tumor accounts for 5% of all childhood cancers. Approximately 500 cases of Wilms tumor are diagnosed each year in the United States. It is slightly more common among African-American children and females than among Caucasian children. Most children (78%) diagnosed with Wilms tumor are between 1 and 5 years of age. The most common age at diagnosis is 3–4 years. It is less common in older children and rare in adults. n IS WILMS TUMOR INHERITED? Cancer may be caused by abnormalities in the genes, but only some forms are inherited. Studies have shown that about 2% of all Wilms tumors are inherited (one or more family members with a history of the disease). It appears that children who have the disease in both kidneys and who are younger at the time of diagnosis are more likely to have a genetic predisposition to develop Wilms tumor. The presence of Wilms tumor in other family members or the presence of intellectual disabilities or other birth defects in the child is another indication that the child may be genetically predisposed to developing Wilms tumor. If your child’s doctor suspects that your child has an inherited type of tumor, examination of family members and genetic counseling are available.

n WHAT IS METASTASIS? Metastasis refers to the spread of cancer from its original location (primary site) to other parts of the body. Wilms tumors may metastasize, or spread, through the blood system or the lymphatic system (tissues and cells that help to fight infection). The most common site of metastasis is the lungs. Wilms tumors sometimes spread to the liver, other kidney, brain, or bones.

n WHAT IS STAGING? Staging is the process of determining the extent of the disease at the time of diagnosis. The method of treatment proposed for your child will depend upon the stage of the disease at the time of diagnosis. Staging for Wilms tumor is determined by the surgeon when the tumor is removed and by X rays and scans that look for metastatic disease.

The following staging system is recommended by the Renal Dis- ease Committee of the Children’s Oncology Group (COG). It is the major staging system currently used for Wilms tumor. Stage I : The tumor is only in the kidney, and the capsule around the kidney is not broken. There is no me- tastasis. The tumor can be removed by surgery. Stage II : The tumor extends outside the kidney and is pushing against the capsule that surrounds the kidney. The tumor has spread into the blood vessels outside the kidney. No spread can be detected at or beyond the edges of the tumor after surgery. Stage III : The tumor has extended beyond the capsule of the kidney but is only in the abdomen. Any of the following conditions may exist: • The tumor is found in the lymph nodes near the kidney. • The tumor has spilled into the peritoneum (the membrane that lines the walls of the abdomen or belly cavity and encloses organs inside the abdomen) before or during surgery, or by growth into the perito- neal surface. • Parts of the tumor are on the peritoneum. • Tumor cells are on the edges of the tumor after its removal and in the tissue surrounding the area where the tumor had been in the body. • The surgeon is not able to remove the entire tumor because it has spread into the surrounding area and has grown into important body structures that cannot be removed. Only a biopsy (medical test done on removed tissue) can be taken. Stage IV : The tumor has spread beyond the areas described in Stage III (for example, into the lungs, liver, bone, brain, or distant lymph nodes). Stage V : Both kidneys have a tumor. Each kidney is staged according to the above criteria, based on the extent of disease before and after surgery.

n WHAT TESTS AND PROCEDURES WILL MY CHILD NEED? To diagnose Wilms tumor and determine the extent of your child’s disease, a number of tests and procedures are necessary, including many of the following.

Ultrasound Ultrasound uses the echoes from high-frequency sound waves to produce a picture of internal organs or tumors. It can help find tumors in the belly. It also can reveal a lot about the health of the kidneys and bladder. It is used to measure the size of some organs, especially the liver and spleen. The ultrasound can show the blood flow through the veins and whether the blood flow is blocked by a clot. This test is

sometimes done when there is a clot in the arm or leg. Doppler is a type of ultrasound used to look at the way blood flows and the condition of the blood vessels, or to diagnose clots in blood vessels. It is a painless test and uses no radiation. The child lies on a table for a short time. A cold, clear jelly is smeared on the area being examined, and it wipes off easily after the test. A small, round handle is placed on the jelly and moved around to get a clear picture of the tissue or organ. If the ultrasound is of the belly, the child may not be permitted to eat or drink for several hours before the test. X Ray An X ray is a simple test done to look for something abnormal, such as a broken bone. Regular X rays, which may be referred to as plain films , are taken of the affected area and any other areas of concern. Efforts will be made to minimize your child’s exposure to the radiation of X rays. An X ray is one form of radiation that can be used at low levels to produce an image of the body on film. CT or CT Scan The CT (computerized or computed tomography) scan, which is sometimes called a CAT (computerized axial tomography) scan, creates detailed pictures of internal organs and tumors. It is a computer-assisted X ray. The CT scan machine is a table surrounded by a doughnut-shaped X-ray camera. Every second, that camera moves a very small distance around the patient while taking an X ray. Then 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 tumors, infections, blood clots, and areas of abnormal bleeding. Certain tumors are regularly checked by CT scan during and after treatment to determine the effectiveness or if there is a relapse. CT scans are painless. Some children require sedation to help them lie still during the scan. If the child is having a CT scan of their belly, they will be asked to drink a special dye that makes the pictures more easily seen. Some children may require a small amount of dye to be injected through an intravenous (IV) line as well. If your child receives the dye into their vein, they may have a warm, flushing feeling. Generally, there are no side effects from either type of dye; however, allergic reactions are possible. There is some radiation exposure with this test. Your child’s healthcare team will try to minimize how often it is done, but it is very helpful in diagnosing and following the stage and location of the cancer.

MRI MRI (magnetic resonance imaging) is a test that uses radio waves and magnetic fields instead of X rays. A computer helps translate the pattern of radio waves into very detailed pictures of parts of the body. MRI is sometimes more useful than other tests, like X rays or CT scans, because of the clearness of the images that are made. This is true with 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 the effectiveness of the treatment or to determine if there is a relapse. A person having an MRI lies on a table which is rolled into a machine that surrounds them on all sides, like a tube. There are open MRI machines available for people who are very anxious in a regular MRI machine. The test is painless. The machine makes quite a bit of noise, so your child might use ear plugs or a headset during the test. They must be able to lie completely still, and some children require sedation to do this. The test usually takes 1–2 hours. Often, a dye that will give better pictures will be injected into the vein. Because it uses magnets, MRI machines cannot have metal objects inside them. 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.

Blood Tests Blood tests are done to monitor your child’s blood cells, body salts, and chemistries. Blood tests are very helpful in diagnosing medical problems and monitoring for side effects of treatment. A specially trained nurse or technician uses a needle to enter a vein and collect a blood sample in tubes that are then sent to a laboratory where they are examined closely. 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 to serve as a baseline for your child. Blood tests will be used throughout treatment to monitor for possible side effects and your child’s response to treatment. Blood tests such as creatinine and blood urea nitrogen (BUN) monitor changes in kidney function. Blood tests called hepatic function panel and bilirubin detect changes in liver function. Alkaline phosphate and lactic dehydrogenase (LDH) levels may be high in the blood due to bone growth from a tumor. 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 to all cells of the body. The platelets stop bleeding by rushing to the site of an injury and forming a clot. A complete blood count , or CBC, is a blood test used to measure the amount and the health of these three types of cells. The CBC’s results is useful in diagnosing blood disorders that a child has inherited or developed. It also is used to diagnose cancers of the blood system, such as leukemia. A CBC may change due to things going on in the body, such as infections, nutritional problems, or as a response to some medications like chemotherapy. Blood Chemistry Tests A blood chemistry test measures the levels of electrolytes, like sodium and potassium, in the blood. These measurements can be very helpful by giving information about the health and function of the kidneys and liver. They also give the healthcare team information about a child’s nutritional status. Blood chemistry tests can diagnose diabetes (high blood sugar), tissue damage, and problems with metabolism.

Other chemistries, such as alanine aminotransferase (ALT), aspartate aminotransferase (AST), and bilirubin, may be needed to assess liver function. Other blood tests including the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) may be used to measure response to treatment. Venous Access Device A venous access device (VAD), sometimes called a central venous catheter, is a temporary or long-term intravenous (IV) tube that can be used for the duration of your child’s therapy. 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 sec- onds. It can be used to administer medications, IV fluids, chemotherapy, blood products, and nutritional support and to draw blood for testing. It is inserted during surgery while your child is sedated or has been given general an- esthesia either before chemotherapy begins or within the first month of treatment. Not all children 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 tun- neled central venous catheter (Broviac/Hickman) that has a tube, a lumen (either double or single) which comes out of your child’s chest, or a PICC 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. The care for each of these types of catheters is slightly different. A port requires almost no care at home but a special needle (noncoring) will need to be inserted through the skin into the port each time it is used. Tubing can be attached to the needle if needed to give medication or IV fluids. An external central line requires routine dressing changes to prevent infection and to care for the skin around the tube. The external lines also are routinely flushed with a solution to keep them working properly. You will be taught how to care for the device at home. n HOW CAN WILMS TUMOR BE TREATED? Three types of therapy are commonly used to treat Wilms tumor: surgery, radiation, and chemotherapy. The type of therapy chosen depends upon the extent of the disease. Your child’s medical team will talk with you about the best treatment for your child. Surgery Surgery is the first-line treatment for all stages of Wilms tumor. Surgery can remove the bulk of the tumor and the affected kidney and is done at the time of diagnosis. In children whose tumor is too large, presurgery chemotherapy is given to shrink the tumor before removal. For children with Stage V Wilms tumor (both kidneys), the kidney with the largest tumor is removed. Chemotherapy Chemotherapy is medicine intended to kill tumor cells in the bloodstream or any tumor cells remaining elsewhere. Several chemotherapy medications are known to be effective in killing Wilms tumor cells, but no single chemotherapy medication can control this disease by itself. Most are given through a vein or CVC. Your child’s medical team will explain in detail the possible side effects of the specific chemotherapy medicines recommended for your child. Most children with Wilms tumor will get chemotherapy at some point during their treatment. Some children with very low risk might not need it.

Radiation Therapy Radiation therapy is a special kind of X-ray treatment. Radiation treatment is very precise and is given in specially measured amounts by radiation therapy experts. Radiation often is used together with chemotherapy. The purpose of radiation therapy is to kill cells that may have escaped from the tumor. Wilms tumor cells are often very sensitive and easily killed by radiation. Usually children with Stage I or Stage II disease do not receive radiation because surgery is the better option for them. If radiation is necessary for your child, the radiation- therapy team will discuss with you exactly how the radiation will be given and how long the treatments will last. In general, children experience very few side effects while they are getting radiation therapy. Some children may be more tired than usual or have decreased appetites. There can also be long-term side effects, which will be explained to you in detail.

n HOW LONG WILL MY CHILD’S THERAPY LAST? The length of treatment will depend on the stage of the tumor. Chemotherapy treatment generally lasts 5–7 months. It involves weekly visits to the clinic for chemotherapy, blood tests, or both.

n WHAT NEW METHODS OF TREATMENT ARE THERE? According to the American Cancer Society, over 90% of children survive Wilms tumor. Because most children with Wilms tumor are cured of their disease, researchers are looking at ways to fine-tune the treatment. Children with a good prognosis (favorable outcome) may require less treatment in the future. Children with unfavorable histology (the study of microscopic tissue structure) or with metastasis

may require more intensive treatment. Effective treatments are being developed for the 10%–15% of children who have a recurrence (repeated appearance) of Wilms tumor. New studies will help us see if current genetic markers are associated with the aggressiveness of the tumor or how the tumor responds to treatment.

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 to a new (research) treatment that is believed to be at least as good as, and possibly better than, the standard treatment. Clinical trials allow the medical team 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 cannot be known ahead of time, and unknown side effects may occur. On the other hand, children who participate in clinical trials may be among the first to benefit from new treatment approaches. Before making a decision about your child’s participation in a clinical trial, the potential risks and benefits should be discussed with your child’s medical team. More information about clinical trials is available at www.cancer.gov/research/areas/clinical-trials. You can also call 800.4CANCER (800.422.6237).

n WHAT ARE THE POTENTIAL LONG-TERM EFFECTS OF THERAPY? The major concern in children with Wilms tumor is long-term function of the remaining kidney. The Journal of Urology reported data from the National Wilms Tumor Study Group and the U.S. Renal Data Systems that less than 1% of the children with the disease in only one kidney developed kidney failure. Children with the disease in both kidneys, and those who had a syndrome associated with Wilms tumor, had a higher risk of developing kidney failure. Children who received radiation therapy for their disease are at risk for mild scoliosis (sideways curving of the spine), muscular hypoplasia (underdevelopment of the muscle), and fertility and pregnancy problems (e.g., increased numbers of spontaneous miscarriages, low birth-weight babies, premature babies, babies with congenital problems, and neonatal deaths). A small number of children treated for Wilms tumor may develop congestive heart failure due to one of the chemotherapy drugs. Also, a very small number of children have developed a second cancer due to the chemotherapy given. Your child’s treatment team can discuss these risks with you in more detail.

n 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. Ask questions when there is anything you are not sure about. It may help to write down your questions as you think of them. This will help you remember all of your questions when the healthcare team is there. Here are some 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 for recurrence/relapse? • Has the cancer spread beyond the primary site? • What should we do to prepare for treatment? • What is the timeframe for determining histology?

Use this space to write down some additional questions:

n ARE MY FEELINGS NORMAL? 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 them in the beginning. However, information will be repeated and absorbed. Many families feel somehow responsible for their child’s disease. Feelings of guilt are common because they could not protect their child from illness or about 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 this cancer is not known. Symptoms are often the same as many childhood illnesses and diagnosis often takes some time. Feelings of sadness, anger, and helplessness about your child’s diagnosis are also 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.

n HOW CAN I HELP MY CHILD? Children often think that something they did caused their cancer; it will help if you reinforce 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 to keep your relationship honest and maintain closeness. Your child will need to share their feelings with someone that they trust. Sometimes, children choose to share feelings with someone other than a parent because they are afraid that they might upset the parent. Don’t be afraid to ask your child about their feelings—it may be what your child is waiting for. Also, don’t be afraid to share information with your child about what is happening and why. The things children sometimes imagine on their own are often more frightening than what is actually happening. In spite of your child’s disease, keep in mind that they are still a child first. All children need love, attention, the opportunity to learn and try new skills, and limits. As your child goes through cancer treatment keep in mind that they still have all the needs of a growing child. Do not avoid talking to your child about therapy, using explanations your child will understand. Children tolerate treatment better if they understand it and if they are allowed to help make decisions about their care when appropriate. Keeping information from your children will force them to use their imaginations and draw their own conclusions about their situations. These conclusions can be much more frightening than the reality of the situation. As a parent it is difficult to watch your child go through cancer therapy. Your child may sometimes seem sicker than before the therapy. Your feelings about what your child is going through during treatment must be balanced with the knowledge that treatment provides the chance of curing the disease and having your child live a full and meaningful life. Accepting the changes in your child that the cancer and the treatment may cause is often difficult, but keep in mind that many of them, like hair loss, are often temporary. Always remember that despite outward changes your child is still the same person on the inside.

n IS MY CHILD’S DIET IMPORTANT DURING THERAPY? Yes. Research has shown that well-nourished children tolerate therapy 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 avoid junk foods and foods that are high in salt in favor of more nutritious foods.

If your child is on steroid medications such as prednisone or Decadron® (dexamethasone), their appetite will likely be increased. While on these medicines, your child may be constantly hungry and may 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; these side effects are temporary. Your child’s hydration status is also an important part of their well-being. Offer your child fluids to drink frequently throughout the day. If your child is not drinking enough fluids throughout the day they may become dehydrated. Signs of dehydration include decreased urine output, dizziness, and a dry mouth.

Multivitamins, herbs, and other diet supplements should be avoided unless approved by your child’s healthcare team. There can be interactions between them and your child’s chemotherapy. Please ask your healthcare team if you are considering any of these types of therapy. A dietitian may be part of your child’s healthcare team. They are trained in the calorie and nutritional needs of children. The dietician can provide you with education regarding your child’s dietary needs and ideas about how you can 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. If there is a concern about nutritional problems, your child’s healthcare team will discuss with you ways to manage and improve your child’s nutrition. n CAN MY CHILD ATTEND SCHOOL DURING THERAPY? Your child’s ability to go to school during therapy will depend on the intensity of the treatment and your child’s response to 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 their schoolwork, even during these times. Federal laws ensure that your child can receive the same education as their peers. Talk with your child’s healthcare team and school about these laws. Then work with them to arrange for a tutor or another way to help your child stay on track with schoolwork. Discuss your child’s ability to attend school with the healthcare team. 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. SUGGESTED READING CureSearch for Children’s Cancer. (2011). Kidney cancer in children (Wilms tumor): Just diagnosed information. Retrieved from http://www.curesearch.org/Wilms-Tumor-in-Children-Just-Diagnosed-Information. National Cancer Institute. (2018). Wilms tumor and other childhood kidney tumors. Retrieved from http://www. cancer.gov/cancertopics/pdq/treatment/wilms/Patient. REFERENCES Baggot, C., Fochtman, D., Foley, G., & Kelly, K. (2011). Nursing Care of Children and Adolescents with Cancer and Blood Disorders . Glenview, IL: APHON. American Cancer Society. (n.d.). Wilms Tumor. Retrieved December 16, 2020, from http://www.cancer.org/ cancer/wilms-tumor/about.html

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

Manual para la familia

Tumor de Wilms

SERIE ONCOLOGÍA

Manual para la familia

Tumor de Wilms

SERIE ONCOLOGÍA

Tumor de Wilms MANUAL PARA LA FAMILIA

Editado por Deanna Hill, RN BSN CPHON®

Revisor de contenido Steering Council

Revisor por parte de los padres John Sullivan

Este manual ha sido publicado por la Association of Pediatric Hematology/Oncology Nurses (APHON) con fines educativos únicamente. El contenido ha sido desarrollado por fuentes confiables y no pretende ser el único tratamiento aceptable o seguro para tratar el tumor de Wilms. Es posible que bajo ciertas circunstancias o condiciones se requiera un tratamiento adicional o diferente. A medida que las nuevas investigaciones y la experiencia clínica amplían las fuentes de información disponibles sobre el tratamiento del tumor de Wilms, es posible que sea necesario ajustar el tratamiento y la farmacoterapia. APHON no garantiza ni asegura, ni hace ninguna otra declaración, expresa o implícita, con respecto a la validez o suficiencia de los tratamientos o a la información relacionada contenida en este manual APHON proporciona este manual para uso educativo. Los usuarios pueden copiar y distribuir este material únicamente en su forma no adaptada, para uso no comercial y con atribución a APHON.

n ¿QUÉ ES EL TUMOR DE WILMS? El tumor de Wilms es un cáncer que se desarrolla a partir de células renales. Es el cáncer de riñón infantil más común y puede aparecer en cualquier parte del riñón. En casos raros, puede ocurrir fuera del riñón. No todos los tumores renales infantiles son tumores de Wilms. En ocasiones se encuentran tumores rabdoides y sarcomas de células claras del riñón en niños y son más difíciles de tratar que el tumor de Wilms.

n ¿CUÁLES SON ALGUNOS SIGNOS Y SÍNTOMAS DEL TUMOR DE WILMS?

El signo más común es un abdomen agrandado o hinchado en un niño de apariencia sana. Usualmente, es la persona a cargo del niño la que nota el agrandamiento del abdomen o siente un bulto mientras lo baña o viste. Otros signos y síntomas generales son dolor de estómago, cansancio, sangre en la orina (detectada en un análisis de orina) y, ocasionalmente, fiebre. Uno de cada cuatro niños diagnosticados con tumor de Wilms también tiene presión arterial alta.

n ¿QUÉ CAUSA EL TUMOR DE WILMS? Actualmente se desconoce la causa del tumor de Wilms. Se sabe que no es ni contagioso ni genético, por lo que no se puede transmitir de persona a persona. No se han asociado comportamientos o hábitos de estilo de vida, incluidos los practicados durante el embarazo, con el desarrollo del tumor de Wilms. El cáncer no es causado por nada que el niño haya comido o dejado de comer (como verduras o vitaminas). n ¿QUIÉN ES DIAGNOSTICADO CON TUMOR DE WILMS? El tumor de Wilms representa 5% de todos los cánceres infantiles. Cada año se diagnostican aproximadamente 500 casos en los Estados Unidos. Es un poco más común entre niños y mujeres afroamericanos que entre niños caucásicos. La mayoría de los niños (78%) diagnosticados tiene entre 1 y 5 años de edad. La edad más común en el momento del diagnóstico es de 3 o 4 años. Es menos común en niños mayores y es raro en adultos. n ¿EL TUMOR DE WILMS SE HEREDA? El cáncer puede ser causado por anomalías en los genes, pero sólo algunas formas se heredan. Estudios han demostrado que un 2% de los tumores de Wilms se heredan (uno o más miembros de la familia con antecedentes de la enfermedad). Al parecer, los niños con la enfermedad en ambos riñones, y que son más jóvenes al ser diagnosticados, tienen una predisposición genética a padecer este tumor. La presencia de un tumor de Wilms en otros miembros de la familia, una discapacidad intelectual u otros defectos de nacimiento también son indicios de una predisposición genética. Si el médico sospecha que tu hijo(a) tiene un tipo de tumor hereditario, se pueden realizar pruebas a los miembros de la familia y recibir asesoramiento genético.

n ¿QUÉ ES LA METÁSTASIS? Metástasis se refiere a la propagación del cáncer desde su ubicación original (sitio primario) a otras partes del cuerpo. El tumor de Wilms puede hacer metástasis, o diseminarse, a través del sistema sanguíneo o linfático (tejidos y células que ayudan a combatir infecciones). El sitio más común son los pulmones, pero a veces se pasa al hígado, al otro riñón, al cerebro o los huesos.

n ¿QUÉ ES LA ESTADIFICACIÓN? La estadificación es el proceso que determina la extensión de una enfermedad en el momento del diagnóstico. El tratamiento de tu hijo(a) dependerá de la etapa en la que se encuentre el tumor de Wilms en el momento del diagnóstico. El cirujano la determinará al extraer el tumor y por medio de radiografías y exámenes que buscan una metástasis.

El siguiente sistema de estadificación es recomendado por el Re- nal Disease Committee del Children’s Oncology Group (COG). Es el principal sistema de estadificación que se utiliza actualmente para el tumor de Wilms. Etapa I: el tumor sólo se encuentra en el riñón y la cápsula alrededor del riñón no está rota. No hay me- tástasis. El tumor se puede extirpar mediante cirugía. Etapa II: el tumor se extiende fuera del riñón y presiona la cápsula que lo rodea. El tumor se ha diseminado a los vasos sanguíneos fuera del riñón. No se detecta diseminación en, o más allá de, los bordes del tumor después de la cirugía. Etapa III: el tumor se ha extendido más allá de la cápsula del riñón, pero sólo está en el abdomen. Puede existir cualquiera de las siguientes condiciones: • El tumor se encuentra en los ganglios linfáticos cerca del riñón. • El tumor se ha extendido al peritoneo (la membrana que recubre las paredes del abdomen y los órganos dentro de la cavidad abdominal) antes o durante la cirugía, o por su crecimiento en la superficie perito- neal. • Partes del tumor se encuentran en el peritoneo. • Las células tumorales se encuentran en los bordes del tumor después de su extirpación y en el tejido que rodea el área donde el tumor se encontraba. • El cirujano no puede extirpar todo el tumor porque se ha diseminado al área circundante y ha invadido estructuras corporales importantes que no se pueden extirpar. Sólo es posible hacer una biopsia (exa- men médico que se realiza en el tejido extraído). Etapa IV: el tumor se ha extendido más allá de las áreas descritas en la etapa III (por ejemplo, hacia los pulmones, el hígado, los huesos, el cerebro o los ganglios linfáticos distantes). Etapa V: ambos riñones tienen un tumor. Cada riñón se clasifica de acuerdo a los criterios anteriores, según la extensión de la enfermedad antes y después de la cirugía.

n ¿QUÉ PRUEBAS Y PROCEDIMIENTOS NECESITARÁ MI HIJO(A)? Para diagnosticar el tumor de Wilms y determinar la extensión de la enfermedad de tu hijo(a), se necesitan hacer varias pruebas y procedimientos, incluidos los siguientes.

Ultrasonido Esta prueba usa los ecos de ondas sonoras de alta frecuencia para producir una imagen de los órganos internos o tumores. Ayuda a encontrar tumores en el abdomen; revela mucho sobre la salud de los riñones y la vejiga. Se usa para medir el tamaño de algunos órganos, especialmente del hígado y el bazo. Puede mostrar el flujo sanguíneo a través de las venas y si éste está bloqueado por un coágulo. A

veces se realiza cuando hay un coágulo en el brazo o la pierna. Un doppler es un tipo de ecografía usada para observar el flujo de la sangre y el estado de los vasos sanguíneos, o detectar coágulos en los vasos sanguíneos. Es una prueba indolora y no utiliza radiación. El niño se acuesta y se le unta un gel transparente y frío en el área a examinar; el gel se limpia fácilmente al terminar. Una especie de manija pequeña y redonda se mueve sobre el gel para obtener una imagen clara del tejido u órgano. Si la ecografía es del abdomen, el niño no podrá comer ni beber durante varias horas antes de la prueba. Rayos X La radiografía es una prueba sencilla que se realiza para buscar algo anormal, como un hueso roto. Se toman radiografías regulares, o radiografías simples , del área afectada o de cualquier otra área de interés. Se hará lo posible por minimizar la exposición de tu hijo(a) a la radiación de los rayos X, que es la forma de radiación con niveles más bajos que se puede utilizar para producir una imagen del cuerpo en una película. TC o tomografía computarizada La TC (tomografía computarizada), a veces llamada CAT (tomografía axial computarizada), crea imágenes detalladas de los órganos internos y los tumores. Es una radiografía asistida por computadora. La máquina es una camilla rodeada por una cámara de rayos X en forma de rosquilla o dona. Cada segundo, la cámara avanza una distancia muy pequeña alrededor del paciente y toma una radiografía. La computadora reúne estos rayos X y da una imagen tridimensional transversal desde todos los ángulos. Las TC son útiles para diagnosticar tumores, infecciones, coágulos de sangre y áreas con sangrado anormal. Ciertos tumores se controlan periódicamente mediante una TC durante y después del tratamiento para determinar su eficacia o si hay una recaída. La tomografía computarizada es indolora. Algunos niños tienen que ser sedados para que puedan estar quietos durante la prueba. Si la TC es del abdomen, al niño se le dará a beber un tinte especial que hace que las imágenes sean más nítidas. A veces se inyecta una pequeña cantidad de tinte por vía intravenosa (IV), si este es el caso, tu hijo(a) puede sentir calor y presentar enrojecimiento en la zona. Generalmente, no hay efectos secundarios con ninguno de los dos tipos de tintes; sin embargo, puede haber reacciones alérgicas. Con la TC, el niño se expone a una pequeña cantidad de radiación. Se hará lo posible por minimizar la frecuencia con la que se realiza esta prueba. La tomografía computarizasa es muy útil para diagnosticar y seguir el estadio y la ubicación del cáncer.

MRI o resonancia magnética La resonancia magnética (MRI) es una prueba que usa ondas de radio y campos magnéticos en lugar de rayos X. Una computadora ayuda a traducir el patrón de ondas de radio a imágenes muy detalladas de partes del cuerpo. En ocasiones, la MRI es más útil que otras pruebas, como los rayos X o las tomografías computarizadas, debido a la claridad de las imágenes que se obtienen; por ejemplo, cuando se trata de áreas del cerebro y la médula espinal, vasos sanguíneos, huesos y algunos otros órganos del cuerpo. Ciertos tumores se controlan periódicamente mediante una MRI durante y después del tratamiento para determinar su eficacia o si hay una recaída. El niño se acuesta sobre una camilla que se introduce en una máquina en forma de tubo que rodea la camilla por todos lados. Hay máquinas abiertas para las personas que se sienten muy ansiosas dentro de una máquina normal. La prueba es indolora. La máquina hace mucho ruido, por lo que tu hijo(a) podría usar tapones para los oídos o auriculares durante la prueba. Se debe estar totalmente quieto y algunos niños necesitan ser sedados. La prueba dura entre 1 y 2 horas. Se puede inyectar en la vena un tinte que da mejores imágenes. Debido a que utiliza imanes, las máquinas de MRI no pueden tener objetos metálicos en su interior. Tu hijo(a) deberá quitarse todos los objetos metálicos como joyería y ropa con broches o cierres metálicos. La resonancia magnética no expone a tu hijo(a) a ninguna forma de radiación.

Análisis de sangre Estos análisis se realizan para controlar las células sanguíneas, las sales corporales y la química de tu hijo(a). Son útiles para diagnosticar problemas médicos y controlar los efectos secundarios del tratamiento. Persdonal especializado introduce una aguja en una vena y recolecta una muestra de sangre en tubos que luego se envían a un laboratorio donde se examina. A veces, un pinchazo en el dedo es suficiente. Por lo general, estas pruebas se realizan en el momento del diagnóstico para descartar otras enfermedades y servir como referencia. Se hacen análisis de sangre durante todo el tratamiento para controlar los efectos secundarios y la respuesta al tratamiento. Los análisis de sangre como la creatinina y el nitrógeno ureico en sangre (BUN) controlan los cambios en la función renal. Los llamados panel de función hepática y bilirrubina detectan cambios en la función hepática. Los niveles de fosfato alcalino y deshidrogenasa láctica (LDH) pueden estar altos en la sangre debido al crecimiento óseo de un tumor. Hemograma completo (CBC)

La sangre se compone de plasma (la parte líquida de la sangre) y células que flotan en el plasma. Hay tres tipos de glóbulos: blancos, rojos y plaquetas. Los glóbulos blancos combaten las infecciones como parte del sistema inmunológico; los rojos transportan oxígeno a todas las células del cuerpo y las plaquetas detienen el sangrado al formar un coágulo en la lesión. Un hemograma completo , o CBC, mide la cantidad y la salud de estos tres tipos de células. Los resultados sirven para diagnosticar trastornos sanguíneos que un niño ha heredado o desarrollado. También se usa para diagnosticar cánceres del sistema sanguíneo, como la leucemia. El CBC puede cambiar debido a cosas que suceden en el cuerpo, como infecciones, problemas nutricionales o como respuesta a algunos medicamentos como la quimioterapia. Pruebas de química sanguínea Una prueba de química sanguínea mide los niveles de electrolitos, como sodio y potasio, en la sangre. Estas medidas pueden ser muy útiles al brindar información sobre la salud y el funcionamiento de los riñones y el hígado. También dan información sobre el estado nutricional del niño. Las pruebas de química sanguínea pueden diagnosticar diabetes (azúcar alta en sangre), daño tisular y problemas con el metabolismo.

Es posible que se necesiten otras químicas, como la alanina aminotransferasa (ALT), la aspartato aminotransferasa (AST) y la bilirrubina, para evaluar la función hepática. Otros análisis, como la velocidad de sedimentación globular (ESR) y la proteína C reactiva (CRP), miden la respuesta al tratamiento. Dispositivo de acceso venoso Un dispositivo de acceso venoso (DAV), a veces llamado catéter venoso central, es un tubo intravenoso (IV) tem- poral o de largo plazo que se puede usar durante la terapia de tu hijo(a). Se coloca en una vena grande del pecho o del brazo. Estos catéteres largos y flexibles se insertan en el corazón, o cerca del mismo, para administrar los tratamientos necesarios en segundos, como medicamentos, líquidos intravenosos, quimioterapia, productos sanguíneos y apoyo nutricional, así como para extraer sangre para análisis. El DAV se inserta durante la cirugía mientras tu hijo(a) está sedado(a), o con anestesia general, antes de comenzar la quimioterapia o durante el primer mes de tratamiento. No todos los niños necesitan un VAD, esto lo determinará el equipo de atención médica junto contigo. El VAD se retira cuando ya no es necesario. Existen diferentes tipos de VAD: un puerto implantado que se inserta debajo de la piel cerca de la clavícula; un catéter venoso central tunelizado (Broviac / Hickman) que tiene un tubo; un lumen (ya sea doble o simple) que sale del pecho; o una línea PICC (línea central que se inserta a través de una vena en el brazo). El equipo de aten- ción médica analizará cada tipo y te ayudará a elegir el mejor dispositivo para el tratamiento de tu hijo(a). El cuidado de cada uno de estos tipos de catéteres es ligeramente diferente. Un puerto casi no requiere cuidados en el hogar, pero se necesita insertar una aguja especial (sin perforaciones) a través de la piel hasta el puerto cada vez que se use. Se puede conectar un tubo a la aguja si es necesario para administrar medicamentos o líquidos por vía intravenosa. Una vía central externa requiere cambiar los vendajes constantemente para prevenir infeccio- nes y cuidar la piel alrededor de la sonda. Las líneas externas también se deben lavar de manera rutinaria con una solución para que sigan funcionando correctamente. Se te enseñará cómo cuidar el dispositivo en casa. n ¿CÓMO SE PUEDE TRATAR EL TUMOR DE WILMS? Normalmente se utilizan tres tipos de terapia para tratar el tumor de Wilms: cirugía, radiación y quimioterapia. El tipo de terapia elegida depende de la extensión de la enfermedad. El equipo médico de tu hijo(a) te hablará sobre el mejor tratamiento para tu hijo(a). Cirugía La cirugía es el tratamiento de primera línea para todas las etapas del tumor de Wilms. La cirugía puede extirpar la mayor parte del tumor y el riñón afectado y se realiza en el momento del diagnóstico. En niños cuyo tumor es demasiado grande, se da quimioterapia previa a la cirugía para reducir el tamaño del tumor antes de extirparlo. En los niños con un tumor de Wilms en estadio V (ambos riñones), se extirpa el riñón con el tumor más grande. Quimioterapia La quimioterapia son medicamentos que matan las células tumorales en el torrente sanguíneo, o cualquier célula tumoral que haya quedado en otra parte. Hay varios medicamentos de quimioterapia eficaces para matar las células del tumor de Wilms, pero ninguno controla la enfermedad por sí solo. La mayoría se administra a través de una vena o CVC. El equipo médico te explicará los posibles efectos secundarios de los medicamentos específicos recomendados para tu hijo(a). La mayoría de los niños con tumor de Wilms recibirán quimioterapia en algún momento de su tratamiento, pero es posible que los niños con muy bajo riesgo no la necesiten.

Radioterapia La radioterapia es un tipo especial de tratamiento con rayos X. El tratamiento con radiación es muy preciso y es administrado en cantidades especialmente medidas por expertos. A menudo, la radiación se usa junto con la quimioterapia. El propósito de la radioterapia es destruir las células que pueden haber escapado del tumor. Frecuentemente, las células del tumor de Wilms son muy sensibles y se destruyen fácilmente con la radiación. Por lo general, los niños con la enfermedad en estadio I o II no reciben radiación porque la cirugía es su mejor opción. Si tu hijo(a) necesita la radiación, el equipo de radioterapia te informará cómo exactamente se administrará y cuánto durarán los tratamientos. En general, los niños experimentan muy pocos efectos secundarios mientras reciben radioterapia; algunos pueden estar más cansados de lo habitual o tener menos apetito. También puede haber efectos secundarios a largo plazo, éstos se te explicarán en detalle.

n ¿CUÁNTO DURARÁ LA TERAPIA DE MI HIJO(A? La duración del tratamiento dependerá del estadio del tumor. El tratamiento de quimioterapia generalmente dura de 5 a 7 meses y requiere de visitas semanales a la clínica para quimioterapia, análisis de sangre o ambos.

n ¿QUÉ NUEVOS MÉTODOS DE TRATAMIENTO EXISTEN? Según la American Cancer Society, más del 90% de los niños sobreviven al tumor de Wilms, y ya que la mayoría se cura, los investigadores están buscando formas de ajustar el tratamiento. Los niños con buen pronóstico (resultado favorable) pueden requerir menos tratamiento en el futuro. Los niños con una histología (estudio de la estructura microscópica del tejido) desfavorable o con metástasis, pueden requerir un tratamiento más intensivo. Se están desarrollando tratamientos efectivos para el 10% -15% de los niños que tienen una recurrencia (aparición repetida) del tumor de Wilms. Nuevos estudios nos ayudarán a ver si los marcadores genéticos actuales están asociados con la agresividad del tumor o cómo el tumor responde al tratamiento. La mayoría de los avances en el tratamiento del cáncer infantil se han obtenido mediante un proceso conocido como ensayos clínicos. En los ensayos clínicos, el tratamiento más conocido (estándar) para un cáncer específico se compara con un tratamiento nuevo (experimental) que se cree que es al menos tan bueno como el estándar. Los ensayos clínicos permiten a los médicos determinar si los nuevos tratamientos son seguros y eficaces. La participación en ensayos clínicos es voluntaria. Estos ensayos implican la investigación de nuevos planes de tratamiento y no se pueden conocer todos los riesgos con anticipación ni los posibles efectos secundarios. Por otro lado, los niños que participan en ensayos clínicos pueden estar entre los primeros en beneficiarse de nuevos tratamientos. Antes de tomar una decisión sobre la participación de tu hijo(a) en un ensayo clínico, analiza los riesgos y los posibles beneficios con su equipo de tratamiento. Más información sobre ensayos clínicos en www.cancer.gov/research/areas/clinical-trials. También puedes llamar al 800.4CANCER (800.422.6237).

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