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Wilms Tumor

What is cancer?
The body is made up of trillions of living cells. Normal body cells grow, divide, and die in an orderly fashion. During the early years of a person's life, normal cells divide faster to allow the person to grow. After the person becomes an adult, most cells divide only to replace worn-out or dying cells or to repair injuries. Cancer begins when cells in a part of the body start to grow out of control. There are many kinds of cancer, but they all start because of out-of-control growth of abnormal cells. Cancer cell growth is different from normal cell growth. Instead of dying, cancer cells continue to grow and form new, abnormal cells. Cancer cells can also invade (grow into) other tissues, something that normal cells cannot do. Growing out of control and invading other tissues are what makes a cell a cancer cell. Cells become cancer cells because of damage to DNA. DNA is in every cell and directs all its actions. In a normal cell, when DNA gets damaged the cell either repairs the damage or the cell dies. In cancer cells, the damaged DNA is not repaired, but the cell doesn't die like it should. Instead, this cell goes on making new cells that the body does not need. These new cells will all have the same damaged DNA as the first cell does. People can inherit damaged DNA, but most DNA damage is caused by mistakes that happen while the normal cell is reproducing or by something in our environment. Sometimes the cause of the DNA damage is something obvious, like cigarette smoking. But often no clear cause is found. In most cases the cancer cells form a tumor. Some cancers, like leukemia, rarely form tumors. Instead, these cancer cells involve the blood and blood-forming organs and circulate through other tissues where they grow. Cancer cells often travel to other parts of the body, where they begin to grow and form new tumors that replace normal tissue. This process is called metastasis. It happens when the cancer cells get into the bloodstream or lymph vessels of our body.

No matter where a cancer may spread, it is always named for the place where it started. For example, breast cancer that has spread to the liver is still called breast cancer, not liver cancer. Likewise, prostate cancer that has spread to the bone is metastatic prostate cancer, not bone cancer. Different types of cancer can behave very differently. For example, lung cancer and breast cancer are very different diseases. They grow at different rates and respond to different treatments. That is why people with cancer need treatment that is aimed at their particular kind of cancer. Not all tumors are cancerous. Tumors that aren't cancer are called benign. Benign tumors can cause problems -- they can grow very large and press on healthy organs and tissues. But they cannot grow into (invade) other tissues. Because they can't invade, they also can't spread to other parts of the body (metastasize). These tumors are almost never life threatening.

What are the differences between cancers in adults and children?
The types of cancers that develop in children are different from the types that develop in adults. Although there are exceptions, childhood cancers tend to respond better to chemotherapy. Children's bodies also tend to tolerate chemotherapy better than adults' bodies do. But, because chemotherapy can have some long-term side effects, children who survive their cancer need careful attention for the rest of their lives. Since the 1960s, most children and adolescents with cancer have been treated at specialized centers designed for them. Being treated in these centers offers the advantage of a team of specialists who know the differences between adult and childhood cancers, as well as the unique needs of children with cancers. This team usually includes pediatric oncologists, pathologists, surgeons, radiation oncologists, pediatric oncology nurses, and nurse practitioners. These centers also have psychologists, social workers, child life specialists, nutritionists, rehabilitation and physical therapists, and educators who can support and educate the entire family. Most children with cancer in the United States are treated at a center that is a member of the Children's Oncology Group (COG). All of these centers are associated with a university or children's hospital. As we have learned more about treating childhood cancer, it has become even more important that treatment be given by experienced experts.

What is Wilms tumor?
Wilms tumor (also called Wilms' tumor or nephroblastoma) is a type of cancer that starts in the kidneys. It is the most common type of kidney cancer in children. It is named after

Max Wilms, a German doctor who wrote one of the first medical articles about the disease in 1899.

About the kidneys
To understand Wilms tumor, it helps to know about the normal structure and function of the kidneys. The kidneys are 2 bean-shaped organs fixed to the back wall of the abdomen (see picture). Each kidney is about the size of a fist. One kidney is just to the left and the other just to the right of the backbone. The lower rib cage protects the kidneys.

The kidneys' main job is to filter blood and rid the body of excess water, salt, and waste products. The filtered products are changed into urine. Urine leaves the kidneys through long, slender tubes called ureters that connect to the bladder. Urine flows down the ureters into the bladder, and is stored there until the person urinates. The kidneys help control blood pressure. They also help make sure the body has enough red blood cells. They do this by making a hormone called erythropoietin, which tells the bone marrow to make more red blood cells.

which means they affect only one kidney. Mesoblastic nephroma This tumor usually appears in the first few months of life. The more anaplasia is found. Wilms tumors Wilms tumors may start anywhere in the kidneys. In most cases there is only one tumor. Most Wilms tumors are unilateral. but we actually need less than one complete kidney to do all of its basic functions. Tens of thousands of people in the United States are living normal. children may develop other types of kidney tumors.Our kidneys are important. Most tumors are found before they have spread (metastasized) to other organs. healthy lives with just one kidney. These tumors are usually curable with surgery. Wilms tumors often become quite large before they are noticed. More than 9 of 10 Wilms tumors have a favorable histology. This is called anaplasia. About 5% of children with Wilms tumors have bilateral disease (cancer in both kidneys). Types of Wilms tumor Wilms tumors are classified into 2 major types depending on how they look under a microscope (their histology): Favorable histology: Although the cells in the tumor don't look quite normal. Other types of kidney tumors in children About 9 of 10 kidney tumors that occur in children are Wilms tumors. but 5% to 10% of children with Wilms tumors have more than one tumor in the same kidney. . The chance of cure for children with these tumors is very good. the harder it is to cure children with these tumors. Unfavorable histology (anaplastic Wilms tumor): The look of the cancer cells varies widely. and the cells' nuclei (the central parts that contain the DNA) tend to be very large and distorted. they cannot be certain until a sample of the tumor is looked at under a microscope. Very rarely. there is no anaplasia (see next paragraph). The average weight of a newly found Wilms tumor is about 1 pound – many times larger than the kidney in which it developed. Even though doctors may think a child has a cancer such as Wilms tumor based on a physical exam or imaging tests.

which makes them hard to cure. Rhabdoid tumor of the kidney These tumors occur most often in infants. But these factors usually take many years to . The rest of this document refers only to Wilms tumor. Statistics related to survival for Wilms tumors are discussed in the section. This number has been fairly stable for many years. and tobacco use play a major role in many adult cancers. Wilms tumors tend to occur in young children. For example. Wilms tumors are slightly more common among African Americans and among girls. The average age at diagnosis is about 3 years. The outlook for these cancers depends largely on the extent (stage) of the cancer at the time it is found and on whether it can be completely removed with surgery. Treatment is usually similar to the intensive treatment used for Wilms tumors with unfavorable histology. and most have already spread by the time they are found. Treatment is usually similar to the intensive treatment used for Wilms tumors with unfavorable histology. Renal cell carcinoma This is the most common type of kidney cancer in adults. It is very rare in adults." What are the risk factors for Wilms tumor? A risk factor is anything that affects the chance of having a disease such as cancer. smoking is a risk factor for several types of cancer in adults. about 500 new cases of Wilms tumors are diagnosed in the United States. "Survival rates by stage and histology. About 5% of all cancers in children are Wilms tumors. although cases have been reported. They tend to spread to other parts of the body quickly. It becomes less common as children grow older and is uncommon after age 6. but it also accounts for a small number of kidney tumors in children. Lifestyle-related risk factors such as diet. What are the key statistics about Wilms tumor? Each year. physical activity. body weight. Different cancers have different risk factors.Clear cell sarcoma of kidney (CCSK) These tumors are much more likely to spread than Wilms tumors and are harder to cure.

Syndromes are groups of symptoms. and they are thought to have little or no effect on the risk of childhood cancers.influence cancer risk. Most birth defects linked to Wilms tumors occur in syndromes. with the average age being about 3 years. It is caused by the loss (deletion) of part of chromosome 11. the risk of Wilms tumor is slightly higher in African-American children than in white children and is lowest among Asian-American children. Scientists think that these children inherit chromosomes with an abnormal or missing gene from a parent that increases their risk of developing Wilms tumor. Those linked to Wilms tumor include: WAGR syndrome Children with this syndrome have about a 50% chance of having a Wilms tumor. either during a mother's pregnancy or after a child's birth. Race In the United States. It is less common in older children. Family history of Wilms tumor Between 1% and 3% of children with Wilms tumors have one or more relatives with the same cancer. Children with these familial cases of Wilms tumors are slightly more likely than children with sporadic cases (no relatives with Wilms tumor) to have tumors in both kidneys. Gender The risk of having a Wilms tumor is slightly higher in girls than in boys. WAGR stands for: . But most familial cases affect only one kidney. and it is rare in adults. About 1 child in 10 with Wilms tumor also has birth defects. malformations. The reason for this is not known. where the WT1 gene is normally found. including Wilms tumors. So far research has not found any strong links between Wilms tumor and environmental factors. Age Wilms tumors are most common in young children. Most Wilms tumors have no clear cause. Certain inherited syndromes/birth defects There is a strong link between Wilms tumors and certain kinds of birth defects. signs. or other abnormalities that occur together in the same person.

or ovaries) • mental Retardation Beckwith-Wiedemann syndrome Children with this syndrome tend to be big for their age. Wilms tumor has been linked to other syndromes. including: • Perlman syndrome • Sotos syndrome • Simpson-Golabi-Behmel syndrome • Bloom syndrome Certain birth defects Wilms tumor is also more common in children with certain birth defects: • Aniridia (complete or partial lack of the iris [colored area] of the eyes) • Hemihypertrophy (an oversized arm and/or leg on one side of the body) • Cryptorchidism (failure of the testicles to descend into the scrotum) in boys • Hypospadias (defect in boys where the urinary opening is on the underside of the penis) . as well as other medical problems. doctors often advise removing the kidneys soon after this syndrome is diagnosed. which may cause them to be mistaken for girls. testicles. In this syndrome the kidneys become diseased and stop working when the child is very young. Wilms tumors usually develop in the diseased kidneys. clitoris. penis. They also have larger than normal internal organs and often have an enlarged tongue. In boys. Because the risk of Wilms tumors is very high. They may have an oversized arm and/or leg on one side of the body (hemihypertrophy).• Wilms tumor • Aniridia (complete or partial lack of the iris [colored area] of the eyes) • Genitourinary tract abnormalities (defects of the kidneys. Other syndromes Less often. testicles. scrotum. urinary tract. Denys-Drash syndrome This rare syndrome has been linked to changes (mutations) in the WT1 gene. The reproductive organs do not develop normally. and scrotum do not develop. the penis. They have about a 5% to 10% risk of having Wilms tumors.

Certain genes that speed up cell division are called oncogenes. they seem to be the result of gene changes that occur early in a child's life. Normal human cells grow and function based mainly on the information contained in each cell's chromosomes – long molecules of DNA in each cell. as well as how this process can go wrong. That is why other abnormalities. Instead. leading to a Wilms tumor. Cancers can be caused by DNA changes that turn on oncogenes or turn off tumor suppressor genes. like some of those described in the section. "What are the risk factors for Wilms tumor?" are sometimes found together with Wilms tumors. But most Wilms tumors do not seem to be caused by inherited gene mutations. In many cases. and die. are called tumor suppressor genes. which are tumor suppressor genes found on chromosome 11. but they have made great progress in understanding how normal kidneys develop. more than one gene change is probably involved. These gene changes can be inherited from a parent (as is sometimes the case with childhood cancers) or may happen during a person's lifetime as cells in the body divide to form 2 new cells. divide. Some genes control when our cells grow. Some of the cells that are supposed to develop into mature kidney cells remain embryonal or fetal (early) kidney cells. these cells mature by the time the child is 3 to 4 years old. DNA is the chemical in each of our cells that makes up our genes – the instructions for how our cells function. the tissue may somehow begin to grow out of control. If this does not happen. Changes in these genes lead to overgrowth of certain tissues. Usually. Changes in certain genes increase the chance that some kidney cells will remain in an embryonal form and turn into a Wilms tumor. which is found on the X chromosome. Researchers do not yet know exactly why some children have Wilms tumors. It's not clear exactly what causes it to be altered. a small number of Wilms tumors are caused by inherited changes (mutations) in or loss of the WT1 or WT2 genes. perhaps even before birth. For example. there must be changes in other genes that have not yet been found. The kidneys develop very early as a fetus grows in the womb. The result is a mass of primitive-looking cells that may develop into a Wilms tumor. Others that slow down cell division. . Because the genes described above are not altered in all cases of Wilms tumors. In a small number of Wilms tumors there is a change in a gene known as WTX. Clusters of these early kidney cells sometimes remain after the baby is born. Changes (mutations) in certain genes in cells may lead to problems as the kidneys develop. or cause cells to die at the right time. most children with this type of cancer do not have any known birth defects or inherited gene changes.Do we know what causes Wilms tumor? Although there is a clear link between Wilms tumors and certain birth defect syndromes and genetic changes.

it is not practical to do an ultrasound exam as a screening test (a test used for early diagnosis in people with no signs or symptoms of disease) in children who are not at increased risk. at this time there are no known ways to prevent most cancers in children. But because Wilms tumors are so rare. "How are Wilms tumors diagnosed?"). and certain inherited conditions) cannot be changed. If you do. such as in children with Denys-Drash syndrome who are almost certain to develop Wilms tumors.Researchers now understand some of the gene changes that may occur in Wilms tumors. but it's still not clear what might cause these changes. Can Wilms tumor be found early? Wilms tumors are usually detected when they start to cause symptoms such as swelling in the abdomen. so at this time there is no way to protect against most of these cancers. Most doctors recommend physical exams by a specialist and ultrasound exams on a regular basis (for example. There are no known lifestyle-related or environmental causes of Wilms tumors. but most Wilms tumors are not the result of known inherited syndromes. This doesn't seem to be caused by anything that a mother could avoid. Tell your health care provider if you have relatives who have had Wilms tumor. They can be detected earlier in some children with tests such as an abdominal ultrasound (a test that uses sound waves and a computer to make images of internal organs – see the section. Some gene changes may be inherited. On the other hand. The only known risk factors for Wilms tumors (age. race. In some very rare cases. the children in your family may need to have regular ultrasound exams. without having an external cause. doctors may recommend removing the kidneys at a very young age (with a donor kidney transplant later on). but by this point they have often grown quite large. Other gene changes may just be random events that sometimes happen inside a cell. Experts think the cancer comes from cells that were in the fetus but failed to develop into mature kidney cells. so it is important to remember that there is nothing these children or their parents could have done to prevent these cancers. every 3 months until the child reaches 7 or 8 years old) to find any kidney tumors when they are still small and have not yet spread to other organs. There are no known lifestyle-related or environmental causes of Wilms tumors. There are no blood tests or other tests that are useful in screening otherwise healthy children for Wilms tumors. gender. . screening for Wilms tumor is very important for children who have syndromes or birth defects known to be linked to this disease. Can Wilms tumor be prevented? Although the risk of many adult cancers can be reduced with certain lifestyle changes (such as maintaining a healthy weight or quitting smoking).

Imaging tests If the doctor thinks your child might have a kidney tumor. check with your child's doctor so that the cause can be evaluated and treated. the doctor will want to get a complete medical history to learn more about the symptoms and how long they have been there. These tests use sound waves. nausea. Still. The doctor will examine your child to look for possible signs of Wilms tumor or other health problems. which is another possible sign of this cancer. magnetic fields. Children may look healthy and play normally. he or she will probably order imaging tests. which parents may notice while bathing or dressing the child. The main focus will likely be on the abdomen and on any increase in blood pressure. But even if the doctor thinks a child has Wilms tumor because of the results of a physical exam or imaging tests. loss of appetite. It is usually not painful. or radioactive substances to create pictures of the inside of the body. if symptoms suggest that a tumor may be present. and if so. if it is likely to be a Wilms tumor • To learn how far the tumor may have spread. Some children with Wilms tumor may also have fever. both within the kidney and to other parts of the body • To help guide surgery or radiation therapy • To look at the area after treatment to help determine if it has been effective . including: • To help find out if there is a tumor in the kidney(s). but it may cause belly pain in some cases. Many of these signs and symptoms are more likely to be caused by something other than a kidney tumor. The first sign is usually swelling or a hard mass in the abdomen (belly). or blood in the urine. if needed. Imaging tests are done for a number of reasons. the diagnosis needs to be confirmed by looking at a sample of the tumor under a microscope. constipation. The doctor may also ask if there's a family history of cancer or birth defects. especially in the genitals or urinary system.How are Wilms tumors diagnosed? Wilms tumors are usually brought to a doctor's attention because of signs or symptoms a child is having. x-rays. Signs and symptoms of Wilms tumor Wilms tumors can be hard to find early because they can grow quite large without causing any symptoms. Medical history and physical exam If your child has signs or symptoms that suggest a tumor may be present. It feels firm and is often large enough to be felt on both sides of the belly.

allowing doctors to collect the images much quicker than with standard CT. The echoes are converted by a computer into a black and white image on a screen. Before the scan. a small. does not involve radiation. This is often the first imaging test done if the doctor suspects your child has a Wilms tumor because it is easy to have. It is moved across the skin over the kidney so that it can be viewed from different angles. During the test. microphone-like instrument called a transducer is placed on the skin (which is first lubricated with a gel). For this test.Ultrasound (ultrasonography) Ultrasound uses sound waves to create images of internal organs. a ringshaped machine that completely surrounds the table. The slices it images are thinner. Your child will need to lie still on a table while they are being done. Unlike a regular x-ray. like a regular x-ray. which gives more detailed pictures. CAT) scan The CT scan is an x-ray test that produces detailed cross-sectional images of parts of your child's body. This helps in planning the surgery. The echo patterns made by most kidney tumors look different from those of normal kidney tissue. Some people are allergic and get hives. The transducer gives off sound waves and picks up the echoes as they bounce off the tissues in the kidney. Different echo patterns also can distinguish some types of cancerous and non-cancerous kidney tumors from one another. In addition. . This type of CT scan uses a faster machine. the table slides in and out of the scanner. Some people feel a bit confined by the ring they have to lie in while the pictures are being taken. and lets the doctor see the entire abdomen. Your child may need an IV line for the dye. especially in the face). a CT scan creates detailed images of the soft tissues in the body. Instead of taking one picture. Rarely. This lowers the chance of images blurring as a result of body movement. The scanner part of the machine rotates around the body continuously. Computed tomography (CT. Be sure to tell the doctor if your child has any allergies or has ever had a reaction to any contrast material used for x-rays. It also lowers the dose of radiation received during the test. The contrast may cause some flushing (a feeling of warmth. more serious reactions like trouble breathing or low blood pressure can occur. Some doctors may give younger children medicine to help keep them calm or even asleep during the test. your child may be asked to drink a contrast solution and/or get an intravenous (IV) injection of a contrast dye that better outlines abnormal areas in the body. ultrasound is very useful when looking for tumor thrombus (tumor growing into the main veins around the kidney). A computer then combines these pictures into images of slices of the part of the body being studied. CT scans take longer than regular x-rays. Spiral CT (also known as helical CT) is now used in many medical centers. a CT scanner takes many pictures as it rotates around your child while he or she lies on a table.

They may be done if there's a chance that the cancer is in a major vein (the inferior vena cava) in the abdomen. A computer translates the pattern into a very detailed image of parts of the body. Bone scan For this test. Some places provide headphones or earplugs to help block this out. or even a bone biopsy might be needed. Younger children may be given medicine to help keep them calm or even asleep during the test. . so sedation is sometimes needed. Doctors don't usually order this test unless they think your child has a type of Wilms tumor that is likely to spread. Your child then lies on a table for about 30 minutes while a special camera detects the radioactivity and creates a picture of the skeleton. The MRI machine makes loud buzzing and clicking noises that your child may find disturbing. Magnetic resonance imaging (MRI) scan Like CT scans. A contrast material called gadolinium may be injected into a vein before the scan to better see details. but other bone diseases can also cause the same pattern. Areas of active bone changes appear as "hot spots" on the skeleton – that is. as well as to have a baseline view of the lungs to compare with other x-rays that might be done in the future. a small amount of low-level radioactive material is injected into a vein (intravenously. MRI scans show more detailed images than CT and ultrasound. which is confining and can be distressing. MRI scans take longer than CT scans – often up to an hour. MRI scans provide detailed images of soft tissues in the body. The energy from the radio waves is absorbed and then released in a pattern formed by the type of body tissue and by certain diseases. To distinguish between these conditions. These areas may suggest the presence of cancer. or IV). they attract the radioactivity. They may also be used to look for possible spread of cancer to the brain or spinal cord if doctors are concerned the cancer may have spread there. a chest x-ray will be done to look for any spread of Wilms tumor to the lungs. The substance settles in areas of damaged bone throughout the entire skeleton over the course of a couple of hours. Bone scans can help find cancer that has spread to bones.CT scanning is one of the most useful tests to look for a mass inside the kidney. It is also helpful in checking whether a cancer has spread to organs and tissues beyond the kidney. Chest x-ray In most cases. The contrast material usually does not cause allergic reactions. But MRI scans use radio waves and strong magnets instead of x-rays (and therefore don't expose your child to radiation). other imaging tests such as plain x-rays or MRI scans. Your child may have to lie inside a narrow tube. Newer. such as the lungs. more open MRI machines may be another option for some children.

These may include tests to count the number of white blood cells. The biopsy may be done either surgically or with a long. The National Wilms Tumor Study Group staging system is used to describe the extent of spread of Wilms tumors. . The urine may also be tested for substances called catecholamines. as was described in the section "What is Wilms tumor?" In most cases. etc. and blood platelets.Lab tests A urine sample may be tested (urinalysis) to look for blood and other substances in the urine. How is Wilms tumor staged? Staging is the process of finding out how far a cancer has spread. This system describes Wilms tumor stages using Roman numerals I through V (1 through 5). The cells in Wilms tumors have a characteristic appearance when looked at this way. the sample to confirm the diagnosis is obtained during a biopsy as a separate procedure before surgery to treat the tumor. on the cancer's stage. This is done to make sure your child doesn't have another kind of tumor called neuroblastoma. Your child's treatment and prognosis (outlook) depend. if it has been done. and tests to measure certain chemicals and salts in the blood that give clues about how well the liver and kidneys are working. This is also when doctors determine the histology of the Wilms tumor (favorable or unfavorable). (Neuroblastomas often start in the adrenal gland.). imaging tests can give doctors enough information to decide if a child probably has a Wilms tumor. Staging is based on the results of the physical exam and imaging tests (ultrasound. But the actual diagnosis of Wilms tumor is made when a sample of the tumor is removed and looked at under a microscope. hollow needle. as well as on the results of surgery. to a large extent. which lies just above the kidney. Less often. but they may be done to check a child's general health (especially before surgery) and to look for side effects during treatment such as chemotherapy. red blood cells. the sample is obtained during surgery to treat the tumor (see the Surgery section in "How is Wilms tumor treated?"). and therefore surgery should be done. which were described in "How is Wilms tumor diagnosed?". Kidney biopsy/surgery In most cases.) Blood tests are not used to find Wilms tumors. CT scans. National Wilms Tumor Study Group (NWTSG) staging system A staging system is a standard way for the cancer care team to summarize their findings of how extensive the tumor is.

. the tumor was in the kidney and in the nearby adrenal gland. • The cancer has invaded nearby vital structures so the surgeon could not completely remove it. Stage II The tumor grew beyond the kidney. such as those inside the chest. About 20% to 25% of all Wilms tumors are stage II. The cancer remaining after surgery is limited to the abdomen (belly). One or more of the following features may be present: • The cancer has spread to lymph nodes in the abdomen or pelvis but not to more distant lymph nodes.Stage I The tumor was contained within one kidney and was completely removed by surgery. • A biopsy of the tumor was done before it was removed. but it was completely removed surgically without any apparent cancer left behind. Stage IV The cancer has spread through the blood to organs away from the kidneys such as the lungs. which was removed separately. liver. indicating that some of the cancer still remains after surgery. or bone. The tumor was not biopsied before surgery. Stage III This stage refers to Wilms tumors that may not have been completely removed. • Deposits of tumor (tumor implants) are found along the lining of the abdominal cavity. The tissue layer surrounding the kidney (the renal capsule) was not broken during surgery. About 20% to 25% of all Wilms tumors are stage III. The cancer had not grown into blood vessels in or next to the kidney. brain. either into nearby fatty tissue or into blood vessels in or near the kidney. • The cancer "spilled" into the abdominal cavity before or during surgery. • The tumor was removed in more than one piece – for example. About 40% to 45% of all Wilms tumors are stage I. or to lymph nodes far away from the kidneys. • The cancer cells are present at the edge of the sample removed by surgery. The tumor was not biopsied before surgery. About 10% of all Wilms tumors are stage IV.

survival rates are only rough estimates. such as how well the tumor responds to treatment. The 2 most important factors in determining a child's outlook are the stage and histology of the tumor. (The histology refers to how it appears under the microscope – see. which included most of the children treated in the United States in the last few decades. Survival rates are often based on previous outcomes of large numbers of children who had the disease. Some parents may want to know the survival statistics for children in similar situations. In order to get 4-year survival rates. Even when taking other factors into account. or may even not want to know them. The survival rates below are based on the results of the National Wilms Tumor Studies. About 5% of all Wilms tumors are stage V. while others may not find the numbers helpful. many children live much longer than 4 years (and many are cured). doctors have to look at children who were treated at least 4 years ago. The 4-year survival rate refers to the percentage of children who live at least 4 years after their cancer is diagnosed. Survival rates for Wilms tumor by stage and histology Survival rates are often used by doctors as a standard way of discussing a person's prognosis (outlook). Wilms Tumor 4-year Survival Rates Tumor Stage I II III Favorable Histology 99% 98% 94% Unfavorable Histology (Anaplastic Wilms Tumor) 83% 81% 72% . but they cannot predict what will happen in any particular child's case. as he or she is familiar with the aspects of your child's particular situation.Stage V Tumors are found in both kidneys at diagnosis. Of course. Improvements in treatment since then may result in a more favorable outlook for children now being diagnosed with Wilms tumors. so they might not be accurate. Knowing the stage and histology of a child's Wilms tumor are important in estimating their outlook. Your child's doctor can tell you if the numbers below may apply. But other factors may also affect a child's outlook. "What is Wilms tumor?") Some of these numbers are based on only a small number of cases.

Your doctor may have reasons for suggesting a treatment plan different from these general treatment options. General comments about treatment Overall. together with your doctor. nurses. This is followed by a description of the most common approaches used for these tumors based on the tumor stage (extent) and histology (appearance under a microscope). about 9 of 10 children with Wilms tumor are cured. The next few sections describe the types of treatments used for Wilms tumors. as well as their own professional experience. A great deal of progress has been made in treating this disease with surgery. rehabilitation specialists. Most doctors recommend a team approach that includes the child's pediatrician as well as specialists at a child's cancer center. Today. . which conducts clinical trials of new treatments for most children with Wilms tumor. Don't hesitate to ask him or her questions about your treatment options. Because Wilms tumors are rare. and chemotherapy. These views are based on their interpretation of studies published in medical journals. psychologists. The goal of these studies is to find ways to cure as many children as possible while limiting side effects by giving as little treatment as is necessary. It is intended to help you and your family make informed decisions. radiation therapy. For Wilms tumors. including nurse practitioners. most children with this cancer are treated in a clinical trial to try and improve on what doctors believe is the best treatment.IV V 86% 87% 38% 55% How is Wilms tumor treated? This information represents the views of the doctors and nurses serving on the American Cancer Society's Cancer Information Database Editorial Board. few doctors outside of those in children's cancer centers have much experience in treating them. Much of this progress in the United States has been because of the work of the National Wilms Tumor Study Group (now part of the Children's Oncology Group). and other health professionals. the doctors on this team often include: • A pediatric surgeon or pediatric urologist (doctor who treats urinary system problems in children [and genital problems in boys]) • A pediatric oncologist (doctor who uses chemotherapy and other medicines to treat childhood cancers) • A pediatric radiation oncologist (doctor who uses radiation therapy to treat cancer in children) Many other specialists may be involved in your child's care as well. The treatment information in this document is not official policy of the Society and is not intended as medical advice to replace the expertise and judgment of your cancer care team. social workers.

a procedure in which a machine filters waste products out of the blood . the surgeon will try to save some normal kidney tissue. During this procedure. doctors might use chemotherapy. and the attached adrenal gland (a hormone-making gland that sits on top of the kidney). Another option may be to do partial nephrectomies in both kidneys. Sometimes. Radical nephrectomy: This is the most common operation for Wilms tumor that is only in one kidney. or a combination of the 2 to shrink the tumor(s) before trying surgery. Depending on the situation. or it may be in both kidneys. Most children can live very well with only one kidney. the surgeon makes an incision in the middle of the abdomen and removes the cancer along with the whole kidney. Sometimes the tumor may be hard to remove. the ureter (tube that carries urine from the kidney to the bladder). Using surgery first is the preferred treatment approach for most Wilms tumors in the United States. different operations may be used. radiation therapy. If you get one. Treatment for Wilms tumor usually includes surgery. if possible. and sometimes radiation therapy. The child will then need to have dialysis. doctors prefer to give a short course of chemotherapy before the surgery. Take your time and think about all of your options. both kidneys may need to be removed completely. it may have spread into nearby blood vessels or other vital structures. If any cancer cells remain after the first operation. Partial nephrectomy (nephron-sparing surgery): In the small number of children who have Wilms tumor in both kidneys. it's important to get it fairly quickly because Wilms tumors are usually very large and tend to grow fast. You can often get a second opinion if you have questions about the recommended plan (or if you just want to confirm this is the best option). as it provides the best chance of making sure all of the tumor is removed. There seems to be no difference in the results from these 2 approaches. the fatty tissue around the kidney. which removes just the tumor and a margin of normal kidney around it. In these children.After your child's Wilms tumor is found and its stage and histology are determined. Removing the tumor The main goal of surgery is to remove the Wilms tumor. radiation therapy or more surgery may be needed. Surgery for Wilms tumors Surgery is the main treatment for Wilms tumor. The surgeon may remove the kidney containing the most tumor with a radical nephrectomy. It should be done by a surgeon who specializes in operating on children and has experience in treating these cancers. In Europe. In the other kidney the surgeon may do a partial nephrectomy. your cancer care team will suggest a treatment plan. It may be very large. chemotherapy. The first goal of treatment is to remove the primary (main) tumor even if the cancer has spread to distant parts of the body.

or other nearby organs is important in determining its stage and further treatment options. This tube may be called a venous access line. Knowing if a Wilms tumor has spread to the lymph nodes. The end of the tube will be just under the skin or sticking out of the chest area or upper arm. and any suspicious areas biopsied (samples taken to be looked at under a microscope). Complications during surgery such as bleeding. Surgery. central venous catheter. This can be left in place for several months to take blood samples for tests and to give intravenous (IV) chemotherapy drugs and blood transfusions. Almost all children will have some pain for a while after the operation. the other kidney. Assessing the extent of the disease During the operation to remove the tumor (radical or partial nephrectomy). if the child is to have chemotherapy. although this can usually be helped with medicines if needed. or just a port. without needing to puncture the skin in the arm each time. In these cases. but they can happen.) Lymph node removal is known as a regional lymphadenectomy. doctors must balance between making sure the tumors are removed completely and removing only as much tissue as is needed. Possible risks and side effects of surgery Surgery to remove a Wilms tumor is a serious operation. . or reactions to anesthesia are rare. The other kidney and nearby organs such as the liver may also be looked at closely. a surgeon will insert a small plastic tube called a catheter into a large blood vessel – usually under the collar bone. Other problems after surgery are uncommon but can include internal bleeding. For more information on surgery as a treatment for cancer. Placing a central venous access line or device (port) Often. Once the child is healthy enough. infections. a kidney transplant may be an option if a donor kidney becomes available. If there are tumors in both kidneys.several times a week. These drugs enter the bloodstream and reach all areas of the body. Chemotherapy for Wilms tumors Chemotherapy (often called chemo) uses anti-cancer drugs that are given into a vein or by mouth (in pill form). (Lymph nodes are bean-shaped collections of immune cells that normally fight infection. venous access device. Cancer often spreads to lymph nodes. see the separate American Cancer Society document. injuries to major blood vessels or other organs. or problems with food moving through the intestines. lymph nodes near the kidney will also be removed to look for cancer cells in them. Members of the cancer care team will teach you how to care for your child's venous access device. another concern is the loss of kidney function. and surgeons are very careful to try to limit any problems either during or after surgery.

chemotherapy is given before surgery and continued afterward.which makes this treatment useful for cancer that has spread or may have spread to organs beyond the kidney. Different combinations of drugs and dosages are used. Possible side effects of chemotherapy Chemotherapy drugs work by attacking cells that are dividing quickly. other drugs such as doxorubicin (Adriamycin). chemotherapy is usually given after surgery. and let him or her know if your child has side effects so they can be managed effectively. the drugs are given once a week for at least several months. But other cells in the body. They are usually given by a nurse in the doctor's office or in the outpatient section of the hospital. children with Wilms tumors stay in the hospital while they are being given chemotherapy. . and the hair follicles. The side effects of chemotherapy depend on the type of drugs. In most cases. and the length of treatment. the type and amount of chemotherapy depend on the stage of the cancer. the lining of the mouth and intestines. cyclophosphamide. but usually this is not necessary. In both cases. or tumors that recur after treatment. For example. These cells are also likely to be affected by chemotherapy. and/or etoposide may also be used. which can lead to side effects. depending on the type and stage of the Wilms tumor and the child's age. For advanced stage. In the United States. The drugs used most often to treat Wilms tumor are actinomycin D (dactinomycin) and vincristine. which is why they often work against cancer cells. also divide quickly. In Europe. Possible short-term side effects can include: • Hair loss • Mouth sores • Loss of appetite • Nausea and vomiting • Diarrhea or constipation • Increased chance of infections (from low white blood cell counts) • Easy bruising or bleeding (from low blood platelet counts) • Fatigue (from low red blood cell counts) Your child's doctor and treating team will watch closely for any side effects that develop. unfavorable histology. In some cases. ifosfamide. the amount taken. carboplatin. Be sure to ask your child's doctor or nurse about medicines to help reduce side effects. such as those in the bone marrow. There are often ways to lessen these side effects. drugs can be given to help prevent or reduce nausea and vomiting. These drugs are injected into a vein or into a venous access device. Sometimes it may be needed before surgery to shrink a tumor to make the operation possible.

But this small increase in risk has to be weighed against the importance of chemotherapy in treating Wilms tumor. For more information on chemotherapy in general. there is a chance it could damage his or her heart. Doctors try to reduce this risk as much as possible by not giving more than the recommended doses of doxorubicin and by checking the heart with a test called an echocardiogram (an ultrasound of the heart) during treatment. kidney. Your child's doctor will carefully watch the doses used and will check your child's heart function with imaging tests. and blood platelets. The complete blood count (CBC) includes counts of white blood cells (WBCs). Some drugs used in chemotherapy can damage the kidneys and liver. See the section. which helps protect the bladder. For example. • Cyclophosphamide can damage the bladder. The risk of this happening goes up as the total amount of the drug that is given goes up. Some chemotherapy drugs can increase the risk of developing a second type of cancer (such as leukemia) years after the Wilms tumor is cured. red blood cells (RBCs). • Doxorubicin may cause heart damage. Some patients may notice weakness. tingling or numbness. so blood counts will be watched closely during and after chemotherapy. This can be avoided or minimized by giving the drugs with plenty of fluids and with a drug called mesna. . Lab tests to check for side effects of chemotherapy Before giving chemotherapy. If not. if your child is given doxorubicin (Adriamycin). "What happens after treatment for Wilms tumor?" for more on the possible long-term effects of treatment. chemotherapy may need to be delayed or the doses reduced. and bone marrow are functioning well. particularly in the hands and feet. Understanding Chemotherapy: A Guide for Patients and Families. some drugs can have specific side effects: • Vincristine can damage nerve cells. Chemotherapy can lower the numbers of these blood cells. see the separate American Cancer Society document. Blood chemistry tests measure certain blood chemicals that tell doctors how well the liver and the kidneys are working.Along with the effects listed above. Long-term side effects of chemotherapy Possible long-term effects of treatment are one of the major challenges facing children after cancer treatment. your child's doctor will check blood test results to be sure the liver.

known as external beam radiation therapy. Some younger children may be given medicine to make them drowsy before each treatment. Radiation to the abdomen (belly) can cause nausea or diarrhea. It is much like getting an x-ray. with most of the time being spent making sure the radiation is aimed correctly. Radiation therapy can also interfere with the growth of normal body tissues (such as bones) that get radiation. The total dose of radiation is divided into daily fractions given over many days. Many major hospitals and cancer centers now use IMRT. your child lies on a special table while a machine delivers the radiation from a precise angle. Each session lasts about 15 to 30 minutes. Possible side effects of radiation therapy The side effects of radiation therapy depend on the dose of radiation and where it is aimed. Your child may be fitted with a plastic mold resembling a body cast to keep him or her in the same position so that the radiation can be aimed more accurately. especially in younger children. focuses radiation from outside the body on the cancer. but the beams converge at the tumor to give a higher dose of radiation there. . Effects on skin areas that receive radiation can range from mild sunburn-like changes and hair loss to more severe skin reactions. and V) and for some earlier stage tumors with unfavorable histology. Several radiation beams are then shaped and aimed at the tumor from different directions. Three-dimensional conformal radiation therapy (3D-CRT) 3D-CRT uses the results of imaging tests such as MRI and special computers to precisely map the location of the tumor. IV. Each beam alone is fairly weak. The type of radiation used for Wilms tumors. doctors try to limit the area of radiation to avoid any growth problems. Intensity modulated radiation therapy (IMRT) IMRT is an advanced form of 3D therapy. In addition to shaping the beams and aiming them at the tumor from several angles. which makes it less likely to damage normal tissues. For each session. The actual treatment time each day is much shorter.Radiation therapy for Wilms tumor Radiation therapy uses high-energy rays or particles to kill cancer cells. The treatment is not painful. In general. although the dose of radiation is much higher. Some newer radiation techniques allow doctors to focus the radiation more precisely. This may allow the doctor to deliver a higher dose to the tumor. Radiation therapy is usually part of treatment only for more advanced Wilms tumors (stages III. the intensity (strength) of the beams can be adjusted to limit the dose reaching the nearby normal tissues.

who can understand more. Complementary and alternative therapies for Wilms tumor When your child has cancer you are likely to hear about ways to treat his or her cancer or relieve symptoms that your doctor hasn't mentioned. You can get a lot more information on clinical trials in our document called Clinical Trials: What You Need to Know. parents should continue to have their child watched closely by doctors so that they can treat any problems quickly if they come up. they are not right for every child. If your child does qualify for a clinical trial. Older children. You can read it on our Web site or call our toll-free number (1-800-227-2345) and have it sent to you. You may have heard about clinical trials being done for this type of cancer. see the separate American Cancer Society document.cancer. These studies are done to get a closer look at promising new treatments or procedures. As a result. One of the most important decisions you will make is deciding which treatment is best. usually many years after the radiation is Clinical trials for Wilms tumor You may have had to make a lot of decisions since you've been told your child has cancer. You can reach this service at 1-800-303-5691 or on our Web site at Clinical trials are one way to get state-of-the-art cancer care for your child. Still. They are the only way for doctors to learn better methods to treat cancer. Clinical trials are carefully controlled research studies that are done with patients who volunteer for them. radiation therapy slightly increases the risk of developing a second cancer in the areas that get radiation. Or maybe someone on your health care team has mentioned a clinical trial to you.cancer. For more information on radiation therapy. Everyone from friends and family to . you should start by asking your doctor if your clinic or hospital conducts clinical trials. This doesn't happen often with Wilms tumor because the amount of radiation therapy is low. usually must also agree to take part in the clinical trial before the parents' consent is accepted.Radiation can damage DNA. Your child will have to meet certain requirements to take part in any clinical trial. you will have to decide whether or not to enter (enroll) the child into it. You can also call our clinical trials matching service for a list of clinical trials that may meet your child's needs. See the section. If you would like your child to take part in a clinical trial. Still. You can also get a list of current clinical trials by calling the National Cancer Institute Cancer Information Service toll free at 1-800-4-CANCER (1-800-422-6237) or by visiting the NCI clinical trials Web site www. Understanding Radiation Therapy: A Guide for Patients and Families. "What happens after treatment for Wilms tumor?" for more on the possible long-term effects of treatment.

These treatments have not been proven safe and effective in clinical trials. to name a few. Finding out more It is easy to see why parents who have children with cancer think about alternative methods. Alternative treatments: Alternative treatments may be offered as cancer cures. Some methods that are used along with regular treatment are: art therapy or play therapy to reduce stress. or peppermint tea to relieve nausea. We use complementary to refer to treatments that are used along with your regular medical care. You want to do all you can to help fight the cancer. or other methods such as acupuncture or massage. here are 3 important steps you can take: • Look for "red flags" that suggest fraud. But the biggest danger in most cases is that your child may lose the chance to be helped by standard medical treatment. and a few have even been found harmful. or they may no longer be working. • Contact us at 1-800-227-2345 to learn more about complementary and alternative methods in general and to find out about the specific methods you are looking at. As you consider your child's options. Some of these methods may pose danger. Does the method promise to cure all or most cancers? Are you told not to use regular medical treatments? Is the treatment a "secret" that requires you to take your child to certain providers or to another country? • Talk to your child's doctor or nurse about any method you are thinking about. Delays or interruptions in medical treatments may give the cancer more time to grow and make it less likely that treatment will help. Alternative treatments are used instead of a doctor's medical treatment. and special diets. and the idea of a treatment with few or no side effects sounds great. Some have been proven not to be helpful. Complementary methods: Most complementary treatment methods are not offered as cures for cancer. What exactly are complementary and alternative therapies? Not everyone uses these terms the same way. while others have not been tested. or have life-threatening side effects.Internet groups and Web sites offer ideas for what might help. But the truth is that most of these alternative methods have not been tested and proven to work in treating cancer. they are used to help the person with cancer feel better. . These methods can include vitamins. Sometimes medical treatments like chemotherapy can be hard to take. so it can be confusing. acupuncture to help relieve pain. Some complementary methods are known to help. Mainly. herbs. and they are used to refer to many different methods.

For children older than 2 and for those of any age with larger tumors or unfavorable histology. doctors prefer to use surgery as the first treatment in most cases. Children younger than 2 years with small tumors (weighing less than 550 grams) with favorable histology may not need further treatment. but surgery can remove all visible signs of cancer. Stage II These tumors have grown outside the kidney into nearby tissues. Because of this. chemotherapy (actinomycin D and vincristine) is very likely to be effective at this point. Standard treatment starts with surgery to remove the kidney containing the tumor (an operation called radical nephrectomy). and then give chemotherapy (and possibly radiation therapy) afterward. The goal of these studies is to cure as many children as possible while limiting side effects by giving as little treatment as is necessary. Most children with this type of cancer are treated in clinical trials developed by the Children's Oncology Group. With good information and the support of your child's health care team.The choice is yours You always have a say in how your child is treated. In Europe. such as chemotherapy. followed by chemotherapy with actinomycin D and vincristine. In the United States. learn all you can about the method and talk to your child's doctor about it. This is done by comparing the current best treatment with one the doctors think might be better. If the cancer does come back. The chemotherapy is given for 18 weeks. which is given for 18 weeks. experts prefer to begin the chemotherapy before surgery. . Children who have unfavorable histology tumors may also get radiation therapy. surgery is usually followed by chemotherapy with actinomycin D (dactinomycin) and vincristine. Favorable histology: Standard treatment is surgery (radical nephrectomy). If you want to use a non-standard treatment. Stage I (favorable or unfavorable histology) These tumors are still only in the kidney. Treatment by type and stage of Wilms tumor Below are some of the most common treatment approaches for patients with Wilms tumors. you may be able to safely use the methods that can help your child while avoiding those that could be harmful. Treatment for Wilms tumor is based mainly on the stage of the cancer and whether its histology (appearance under the microscope) is favorable or unfavorable. The results seem to be the same. treatment may differ slightly from those described below in some cases. But they need to be watched closely because the chance the cancer will come back is slightly higher than if they had chemotherapy.

In some instances the tumor may be very large or may have grown into nearby blood vessels or other structures so that it cannot be removed safely. . Chemotherapy starts again after surgery. and cyclophosphamide along with mesna (a drug that protects the bladder from the effects of cyclophosphamide). and vincristine) is given for about 6 months. vincristine. then radiation therapy is given to shrink the tumor. which is given for about 6 months. Unfavorable histology with diffuse (widespread) anaplasia: These children also get surgery (radical nephrectomy) and radiation to the abdomen for several days. chemotherapy (doxorubicin. This is followed by chemotherapy. Unfavorable histology with focal (only a little) anaplasia: Treatment begins with surgery (radical nephrectomy) followed by radiation therapy to the abdomen for several days.Unfavorable histology with focal (only a little) anaplasia: Treatment begins with surgery (radical nephrectomy). If there is no response or not enough response to chemotherapy. This is followed by chemotherapy. If there is no response or not enough response to chemotherapy. This is followed by more intense chemotherapy using the drugs vincristine. Chemotherapy lasts about 6 months. actinomycin D. radiation therapy is given to the abdomen for 6 or 7 days. When this is finished. In these patients a small tissue sample is taken from the tumor for testing to be sure that it is a Wilms tumor and then chemotherapy is started. it is given after surgery. When the child recovers. then radiation therapy is given to shrink the tumor. If radiation was not given before surgery. vincristine. Usually the tumor will shrink enough within several weeks so that a radical nephrectomy can be done. Usually the tumor will shrink within several weeks so that a radical nephrectomy can be done. In these cases a small tissue sample is taken from the tumor for testing to be sure that it is a Wilms tumor and then chemotherapy is started. usually with the drugs vincristine. and doxorubicin) for about 6 months. Unfavorable histology with diffuse (widespread) anaplasia: Treatment begins with surgery (radical nephrectomy) followed by radiation therapy to the abdomen for several days. doxorubicin. In rare instances the tumor may be very large or may have grown into nearby blood vessels or other structures so that it cannot be removed safely. and cyclophosphamide along with mesna (a drug that protects the bladder from the effects of cyclophosphamide). usually with 3 drugs (actinomycin D. and doxorubicin) for about 6 months. Stage III Favorable histology: Treatment is usually surgery (radical nephrectomy) followed by radiation therapy to the abdomen over several days. If radiation was not given before surgery. Chemotherapy will be started again after surgery. etoposide. etoposide. it is given after surgery. doxorubicin. This is followed by chemotherapy with 3 drugs (actinomycin D.

This would be followed by more chemotherapy (and radiation therapy if it wasn't given already). and cyclophosphamide along with mesna given for about 6 months. radiation therapy. If the cancer has spread to the lungs. chemotherapy. and doxorubicin) for about 6 months. If there is no evidence of any cancer after a year or two. In cases where the tumor is too large or has grown too extensively to be safely treated with surgery. Unfavorable histology with diffuse (widespread) anaplasia: Treatment begins with surgery (radical nephrectomy) followed by radiation therapy to the abdomen. Recurrent Wilms tumor The prognosis and treatment for children with Wilms tumor that recurs (comes back after treatment) depends on their prior treatment and the cancer's histology (favorable or . If the cancer is still present. and/or surgery to try to remove the tumors but not the entire kidneys. Surgery may be an option at this point. Low doses of radiation will also be given to both lungs if there is spread to the lungs. After about 6 weeks of chemotherapy. This is followed by chemotherapy with the drugs vincristine. The tumors may be removed at this point if enough normal kidney tissue can be left behind. The drugs used will depend on the extent and histology of the tumors. vincristine. a procedure where a special machine filters waste products out of the blood several times a week. low doses of radiation will also be given to that area. etoposide.Stage IV These tumors have already spread to distant parts of the body at the time of diagnosis. Usually biopsies (tissue samples) of tumors in both kidneys and of lymph nodes are taken first. and radiation therapy at some point. For stage IV cancers that have spread to the liver. If not enough functioning kidney tissue is left after surgery. Chemotherapy is then given to try to shrink the tumors. Favorable histology and unfavorable histology with focal (only a little) anaplasia: Standard treatment is surgery (radical nephrectomy). The entire abdomen will be treated if there is still some cancer in the abdomen. a donor kidney transplant may be done. This is followed by chemotherapy with 3 drugs (actinomycin D. doxorubicin. treatment may include more chemotherapy. The entire abdomen will be treated if there is still some cancer in the abdomen. a child may need to be placed on dialysis. followed by radiation therapy to the abdomen. Stage V Treatment for children with tumors in both kidneys is unique for each child. surgery may be an option to remove any liver tumors that still remain after chemotherapy and radiation therapy. a second operation may be done. although it typically involves surgery. chemotherapy and/or radiation therapy may be used first to shrink the tumor.

Detailed guidelines intended for use by cancer care professionals are also available on www. You should ask any questions that are on your mind. and chemotherapy. consider these questions: • What kind of kidney cancer does my child have? Is it a Wilms tumor? • Is the histology of the tumor favorable or unfavorable? • What is the stage of my child's cancer. such as the ICE regimen (ifosfamide. often with drugs different from those used during first treatment. The outlook is generally better for recurrent Wilms tumor with the following features: • Favorable histology • Initial stage of I or II • Initial chemotherapy with vincristine and actinomycin D only • No previous radiation therapy • Recurrence at least 12 months after initial diagnosis The usual treatment for these children is surgery to remove the recurrent cancer (if possible). carboplatin. More treatment information for Wilms tumor For more details on treatment options – including some that may not be addressed in this document – the National Cancer Institute (NCI) is a good source of information.cancer. For instance.unfavorable). The NCI provides treatment guidelines via its telephone information center (1-800-4CANCER) and its Web site (www. and what does that mean? • Are there other tests that need to be done before we can decide on treatment? • How much experience do you have treating this type of cancer? • What other doctors will we need to see? .gov). radiation therapy.cancer. Recurrent Wilms tumors that do not have the features listed above are much harder to Very high-dose chemotherapy followed by a stem cell transplant (sometimes called a bone marrow transplant) may also be an option in this situation. What should you ask your child's doctor about Wilms tumor? It is important to have frank. no matter how small they may seem. These children are usually treated with aggressive chemotherapy. and etoposide) or others being studied in clinical trials. open discussions with your child's cancer care team.

Follow-up tests Your child's health care team will set up a follow-up schedule. It is certainly normal to want to put the tumor and its treatment behind you. which will include physical exams and imaging tests (such as ultrasound and CT scans) to look for the growth or return of the tumor. or any problems related to treatment. be sure to write down any others you might have. you might want to ask about getting a second opinion. and to get back to a life that doesn't revolve around cancer.• What treatment options do we have? • What do you recommend and why? • What are the short-term risks or side effects to the treatments you suggest? • What are the likely long-term effects of treatment? Could it affect my child's fertility? • What should we do to be ready for treatment? • How long will treatment last? What will it involve? Where will it be done? • How will treatment affect our daily activities? • Based on what you've learned about my child's cancer. Since most patients have had a kidney removed. and concerns about progression or recurrence of the tumor. For instance. where you may be able to get in touch with other families who have been through this. You might also want to ask about nearby or online support groups. or you may want more information about recovery times so you can plan your child's school and activity schedule. blood and urine tests will be done to check how well the .and long-term effects of the tumor and its treatment. What happens after treatment for Wilms tumor? After treatment for Wilms tumor. what is the outlook for cure? • What are the chances of the cancer coming back with these treatment plans? • What would we do if the treatment doesn't work or if the cancer recurs? • What clinical trials are suitable options for my child? Are these studies run by the Children's Oncology Group or a nationally prominent children's cancer center? • What type of follow-up will my child need after treatment? Along with these sample questions. But it's important to realize that followup care is a central part of this process that offers your child the best chance for long-term recovery. the main concerns for most families are the short.

so that the cause can be determined and treated. the more likely it is they can be treated effectively. These late effects may include: • Reduced kidney function • Heart or lung problems after receiving certain chemotherapy drugs or radiation therapy to these parts of the body • Slowed or decreased growth and development • Changes in sexual development and ability to have children • Development of second cancers later in life (rare) To help increase awareness of late effects and improve follow-up care of childhood cancer survivors throughout their lives. If the tumor recurs. so does the care and follow-up after treatment. To learn more. Ask your child's health care team about possible long-term complications and make sure there is a plan in place to watch for these problems and treat them. and the age of the child when being treated. and see the separate .remaining kidney is working. Doctor visits will be more frequent at first. if needed. The earlier any problems can be recognized. The recommended schedule for follow-up exams and tests depends on the initial stage and appearance (favorable or unfavorable) of the cancer. and any problems that the child may have had during treatment. or if it does not respond to treatment. Doctors have learned that the treatments may affect children's health later in life. your child's doctors will discuss with you the other treatment options available (see "How is Wilms tumor treated?"). written for doctors and other health care professionals. the doctor may also order imaging tests to check the function of your child's heart. Just as the treatment of childhood cancer requires a very specialized approach. but the time between visits may be extended as time goes on. it is important to report any new symptoms to your child's doctor right away. such as the specific treatments the child received. many children treated for Wilms tumor are now surviving into adulthood. Long-term effects of cancer treatment Because of major advances in treatment. so watching for health effects as they get older has become more of a concern in recent years. describe in detail the suggested long-term follow-up care based on the treatments the child has received. During this time. the Children's Oncology Group (COG) has developed long-term follow-up guidelines for survivors of childhood cancers. if needed. The risk of late effects depends on a number of factors. the type of treatment. the doses of treatment. Your child' doctor can give you an idea of what to look for. ask your child's doctors about the COG survivor guidelines. If your child received doxorubicin (Adriamycin) as part of the chemotherapy regimen. These guidelines.

If you can get a list of these from the pediatric oncologist. this might be related to how aggressive certain tumors are likely to be. Biology of Wilms tumors Research is continuing to unravel the mystery of how changes in certain genes cause Wilms tumors. and other health professionals whose hard work has already saved the lives of thousands of children with Wilms tumor. • If there was surgery. • If there was chemotherapy. a list of the final doses of each drug your child received. COG is a large group of doctors. What's new in Wilms tumor research and treatment? Much of the research going on in the area of Wilms tumor is coordinated by the Children's Oncology Group (COG). Doctors and other members of the health care team can also often recommend special support programs and services to help children after cancer treatment. Still. a copy of the operative report(s). There are certain pieces of information that your child's doctors should have. • If there were hospitalizations. they may also have some problems with normal functioning and school work. a summary of the type and dose of radiation and when and where it was given. Keeping good medical records As much as you may want to put the experience behind you once treatment is completed. your child will grow up and be on his or her own. even into adulthood. Childhood Cancer: Late Effects of Cancer Treatment. Certain drugs may have specific long-term side effects. • If there was radiation therapy. Because different genes seem to be involved. nurses. Most children with Wilms tumors are very young at the time of diagnosis. it might help any new doctors your child has. . Eventually. it is also very important to keep good records of your child's medical care during this time. whose main goal is to improve the treatment and quality of life of children with Wilms tumor and other types of cancer. a copy of the discharge summaries doctors prepare when patients are sent home. including: • A copy of the pathology report(s) from any biopsies or surgeries. scientists. These can often be overcome with support and encouragement. Depending on their age. It is important that you or your child is able to give other doctors the details of the cancer diagnosis and treatment. some children may have emotional or psychological issues that need to be addressed during and after treatment.American Cancer Society document.

they have begun to look for ways to determine which children might be spared from more intensive treatment. most research on targeted drugs so far has been done on cells growing in lab dishes or in animals. Researchers are also studying the molecular changes that seem to cause Wilms tumor cells to grow and spread.As doctors have learned how to treat Wilms tumors more effectively. and because Wilms tumors with unfavorable histology are uncommon. researchers hope to test these new drugs in clinical trials. studies have shown that Wilms tumors with certain changes on chromosomes 1 or 16 seem to be more likely to recur after initial treatment. Many studies are looking to improve upon current chemotherapy treatments. Because Wilms tumors with favorable histology are usually cured with surgery and chemotherapy. They are also looking to for ways to identify children who might need more aggressive treatment to be cured. Newer chemotherapy drugs such as topotecan and irinotecan are now being tested. studies are looking at whether young children with very favorable outlooks need any treatment other than surgery. They often have different (and less severe) side effects. Targeted drugs work differently from standard chemotherapy drugs. Eventually. Targeted therapies have already become standard treatments for some kinds of cancers of adults. Other studies are looking at bone marrow or peripheral blood stem cell transplants. Doctors are now studying whether children with such tumors might benefit from more intensive treatment. so that they may someday have a role in treating children with unfavorable histology Wilms tumors. recently. Earlier studies identified treatments that were very effective in curing Wilms tumors with the most favorable histology. For example. Current clinical trials are studying ways to treat these cancers successfully while reducing side effects as much as possible. recent studies from Europe suggest that in some cases chemotherapy may not need to be continued as long as previously thought. For example. This approach might help treat tumors that are not responding to standard treatments or that would otherwise have a poor prognosis. As researchers have learned more about the gene changes in Wilms tumor cells. . The outlook for patients with Wilms tumors with unfavorable histology is not as good. which let doctors give higher doses of chemotherapy than the body normally could tolerate. For example. they have started to develop newer drugs that specifically target these changes. This may lead to treatments that are specifically targeted to correct or overcome these changes. and the Children's Oncology Group is continuing to study more intensive and more effective treatments for these children. Treatment of Wilms tumors Clinical trials continue to study ways to improve treatment for children with Wilms tumors.

These materials may be ordered from our toll-free number.. Happened to Me (children's booklet) When Your Brother or Sister Has Cancer (children's booklet) When Your Child's Treatment Ends: A Guide for Families (booklet) Books The following books are available from the American Cancer Society. Because. Someone I Love Has Cancer (kids' activity book) Caregiving: A Step-By-Step Resource for Caring for the Person with Cancer at Home . Call us at 1-800227-2345 to ask about costs or to place your order. 1-800-227-2345..Additional resources for Wilms tumor More information from your American Cancer Society The following related information may also be helpful to you. After Diagnosis: A Guide for Patients and Families (also available in Spanish) Childhood Cancer: Late Effects of Cancer Treatment Children Diagnosed with Cancer: Dealing with Diagnosis (also available in Spanish) Children Diagnosed with Cancer: Financial and Insurance Issues Children Diagnosed with Cancer: Returning to School Children Diagnosed with Cancer: Understanding the Health Care System (also available in Spanish) Clinical Trials: What You Need to Know (also available in Spanish) Family Medical Leave Act (FMLA) Fertility and Cancer: What Are My Options? Nutrition for Children with Cancer (also available in Spanish) Pediatric Cancer Centers (also available in Spanish) Surgery (also available in Spanish) Understanding Chemotherapy: A Guide for Patients and Families (also available in Spanish) Understanding Radiation: A Guide for Patients and Families (also available in Spanish) What Happened to You.

gov National Children's Cancer Society. by William L.candlelighters. National Cancer Institute. Cancer Kids Web site: www. Other publications* For adults 100 Questions & Answers About Your Child's Cancer. Care for Children and Adolescents with Cancer: Questions and Answers. Toll-free number: 1-800-532-6459 (1-800-5-FAMILY) Web site: www. Carroll and Jessica or call CureSearch (National Childhood Cancer Foundation and Children's Oncology Group) Toll-free number: 1-800-458-6223 Web site: www. University of Wisconsin Press. .org *Inclusion on this list does not imply endorsement by the American Cancer Society.Jacob Has Cancer: His Friends Want to Help (coloring book for a child with a friend who has cancer) Let My Colors Out (picture book for young children) National organizations and Web sites* In addition to the American Cancer Society. Cancer & Self-Help: Bridging the Troubled Waters of Childhood Illness. Inc.curesearch.starlight. Chester and Barbara National Cancer Institute Toll-free number: 1-800-422-6237 (1-800-4-CANCER) Web site: www. by Mark A. 1995. Starlight Children's Foundation Toll-free number: 1-310-479-1212 Web site: www.cancer. other sources of patient information and support include: American Childhood Cancer Organization (formerly Candlelighters) Toll-free number: 1-800-366-2223 Web site: www. Available at: www.cancer. Jones and Bartlett Publishers.children-cancer.

American Psychological Association.candlelighters. G. The Amazing Hannah. Young People with Cancer: A Handbook for Parents. Paperstar Book. Available at: www. 2002. 2005. 1999. by Nancy Keene. by Jeanne Munn Bracken and Pruden Pruden. Patient Centered Guides. Surviving Childhood Cancer: A Guide for Families. 2002.). . New Harbinger Publications. or call 1-800-332-8615. Available at: www. When Someone You Love Is Being Treated for Cancer. O'Reilly & Associates. Childhood Cancer Survivors: A Practical Guide to Your Future (2nd Ed. For ages 6 to 12. (Also available in Spanish. edited by Nancy Keene. by Harold Kushner. 2002. Look at Everything I Can Do! by Amy Klett.cancer. Goodheart. Living with Childhood Cancer: A Practical Guide to Help Families Cope. Perseus Publishing. Wendy Hobbie. 1982. by Fred Rogers. 2000. by Grant Steen and Joseph Mirro (editors).org. For older teens.). O'Reilly and Associates. Published by Candlelighters Childhood Cancer Foundation. 2003. National Cancer Institute. Childhood Cancer: A Handbook from St Jude Children's Research Your Child in the Hospital: A Practical Guide for Parents (2nd Ed. Candlelighters Childhood Cancer Foundation. Educating the Child With Cancer: A Guide for Parents and Teachers. by Leigh A. Can be ordered from www. 2000. Oxford University Press. 1998. or call 1800-332-8615. Going to the Hospital. When Bad Things Happen to Good People. For ages 1 to 6.) Chemo. by Kathy Ruccione. Childhood Cancer Survivors: A Practical Guide to Your Future. 2003. Children with Cancer: A Comprehensive Reference Guide for Parents (2nd Edition).) Books for teens and children Although these books are intended for children. by Margo Joan Fromer. and Kathy Ruccione. Candlelighters Childhood Cancer Foundation. N. Craziness and Comfort: My Book about Childhood Cancer. younger kids are helped more when an adult reads with and helps the child reflect about what different parts of the book mean to the child. O'Reilly and Associates. Nancy Keene. For ages 4 to 8. 1997. National Cancer Institute. and Wendy Hobbie. by Nancy Keene. (Also available in Spanish. Woznick and Carol D. by Honna Janes-Hodder and Nancy Keene.Childhood Cancer: A Parent's Guide to Solid Tumor Cancers. 2006. Hall.cancer. by Nancy Keene.

For ages 4 to 8. For ages 6 to 12. eds. Magination Press. 1990. by Karen Crowe. Philadelphia Pa: Lippincott Williams & Wilkins.Life Isn't Always a Day at the Beach: A Book for All Children Whose Lives Are Affected by Cancer by Pam Ganz. For ages 8 to 12. J Clin Oncol. Principles and Practice of Pediatric Oncology. Dome JS. Spunt SL. For brothers and sisters (ages 4 to 8) of a child with cancer. Santana V. Workbook for ages 6 to 10. Why. Poplack DG. Dome JS. When Someone Has a Very Serious Illness: Children Can Learn to Cope with Loss and Change.) What About Me? When Brothers and Sisters Get Sick. 2008:2096–2101. eds. Niederhuber JE. For teens. by Charles M. References: Wilms tumor detailed guide American Cancer Society. 1999. by Jason Gaes. 1991. No matter who you are. for information and support. In: Pizzo PA. (Also available in Spanish. Woodland Press. My Book for Kids with Cansur [sic]. et al. Butterfly Press. Cancer Facts & Figures 2012. For ages 6 to 12. High-Five Publishing. Schultz. Treatment of anaplastic histology Wilms' tumor: results from the fifth National Wilms' Tumor Study. Dome JS. Hershey. Pediatric solid tumors: Wilms' tumor. et al. McKenna WG. Published by Fujsawa Healthcare. Ga: American Cancer Society. . Ballantine Publishing Group. Stupology…I Want to Go Home! by Marilyn K. Magination Press.5:37–46. 2003. Perlman EJ. Viking Penguin. For ages 4 to 8.24:2352–2358. Living Well With My Serious Illness. by Allan Peterkin and Frances Middendorf. by Marge Heegaard. by Joyce C. Management of Wilms' tumour: current practice and future goals. Abeloff's Clinical Oncology. 2004. Dome JS. Cotton CA. Armitage JO. Renal tumors. day or night. 1996. Mills. Ritchey ML. Fairview Press. 2006. Call us at 1-800-227-2345 or visit www. 2003.cancer. by Marge Heegaard. Charlie Brown. Perlman EJ. Contact us anytime. Oncology. 1999. Me and My Marrow. Little Tree: A Story for Children with Serious Medical Problems. 2012. Kastan MB. Pa: Elsevier. 4th ed. Philadelphia. we can help. 5th ed. Why? A Story About What Happens When a Friend Is Very Ill. For ages 8 to 12. 1992. Rodriguez-Galindo C. Kalapurakal JA. 2006:905–932. et al. Lancet Oncol. *Inclusion on this list does not imply endorsement by the American Cancer Society. Atlanta. In: Abeloff MD.

Last Medical Review: 1/31/2011 Last Revised: 1/20/2012 2011 Copyright American Cancer Society . Current therapy for Wilms' tumor. eds. Anderson JR. Accessed at www. Shamberger RC. Nuchtern JG. Kim WJ.10:815–826. 2007. An X chromosome gene.Metzger ML. Rosenberg SA. Dome JS. and Rosenberg's Cancer: Principles and Practice of Oncology. DeVita. 2008:2050–2053. et al. WTX. Rivera MN. Philadelphia. et al. Wells J. 8th ed. Hellman.251:555-558. Pa: Lippincott Williams & Wilkins. Wilms Tumor and Other Childhood Kidney Tumors Treatment. National Cancer Institute Physician Data Query (PDQ). is commonly inactivated in Wilms tumor. Solid tumors of childhood: Wilms' tumor. In: DeVita VT. Ann Surg. Breslow NE.315:642– on October 4. Russell HV. Oncologist. Long-term outcomes with very low risk Wilms tumor treated with surgery alone in National Wilms Tumor Study-5. 2005.cancer. Lawrence TS. 2010. Pappo AS. Science. et al. 2010. 2010.