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Wilms Tumor - Childhood - Overview

This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/2015

ON THIS PAGE: You will find some basic information about this disease and the parts of the body it may affect. This is the first page of Cancer.Net’s Guide to Wilms Tumor. To see other pages, use the menu on the side of your screen. Think of that menu as a roadmap to this full guide.

About the kidneys

Every person has two kidneys. They are bean-shaped organs located above the waist on both sides of the spine. They are closer to the back of the body than the front. The kidneys filter waste from the blood and make urine.

As the kidneys develop in an unborn baby, some of the early cells will become glomeruli, which are balls of blood vessels that filter water, salt, and waste out of the blood. Others will become nephrons, which are the tubes through which the water, salt, and waste pass.

About Wilms Tumor

If the early cells of the kidney do not develop into glomeruli or nephrons, clusters of the immature cells may form in the kidneys when the baby is born. Usually, these cells mature by the time a child is three or four years old, but some may grow uncontrollably, forming a mass of immature cells. This mass is called a Wilms tumor. It can also be called a nephroblastoma.

A Wilms tumor is always cancerous and is the most common type of kidney cancer diagnosed in children. A cancerous tumor is malignant, meaning it can grow and spread to other parts of the body. However, a Wilms tumor is very different from adult kidney cancer.

Wilms tumor usually occurs in only one kidney, called unilateral. However, it can develop in both kidneys, called bilateral. Rarely, a Wilms tumor develops in one kidney first and then the other.

A Wilms tumor is often found only after it has grown to a size of about eight ounces—about four times the weight of a healthy three-year-old child’s kidney. About one out of four children with Wilms tumor have evidence that the tumor has spread either to the lung or liver when it is first diagnosed.

Looking for More of an Overview?

If you would like additional introductory information, explore these related items. Please note these links will take you to other sections on Cancer.Net:

  • ASCO Answers Fact Sheet: Read a one-page fact sheet (available as a PDF) that offers an easy-to-print introduction to this type of tumor.

  • Cancer.Net Patient Education Video: View a short video led by an ASCO expert in childhood cancers that provides basic information and areas of research.

To continue reading this guide, use the menu on the side of your screen to select another section. The next section in this guide is Statistics, and it helps explain how many children are diagnosed with this type of tumor and general survival rates. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Wilms Tumor - Childhood - Statistics

This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/2015

ON THIS PAGE: You will find information about how many children are diagnosed with this type of tumor each year and some general survival information. Remember, survival rates depend on several factors. To see other pages, use the menu on the side of your screen.

In the United States, about 500 children are diagnosed with a Wilms tumor each year. It accounts for about 5% of all childhood cancers. Wilms tumor occurs most often in young children between the ages of three and four. It is uncommon after age six.

The five-year survival rate is the percentage of children who survive at least five years after the tumor is found. It varies according to the stage of the disease. The overall five-year survival rate for children with Wilms tumor is 90%. Stage I, II, and III tumors with a favorable histology have a four-year survival rate that ranges from 94% to 99%. Stage IV and V tumors have a four-year survival rate of 86% and 87% respectively. Survival rates for tumors with an anaplastic histology are lower in each category and range from 83% for children with a Stage I tumor to 38% for Stage IV and 55% for a Stage V tumor.

Survival statistics should be interpreted with caution. These estimates are based on data from hundreds of children with this type of tumor, so the actual risk for a particular individual may be different. It is not possible to tell a child how long he or she will live with a Wilms tumor. Because the survival statistics are measured in multi-year intervals, they may not represent advances made in the treatment or diagnosis of this cancer. Learn more about understanding statistics.

Statistics adapted from the American Cancer Society's (ACS) publication, Cancer Facts and Figures 2015, and the ACS website.

The next section in this guide is Medical Illustrations, and it offers drawings of body parts often affected by this disease. Or, use the menu on the left side of your screen to choose another section to continue reading this guide.

Wilms Tumor - Childhood - Medical Illustrations

This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/2015

ON THIS PAGE: You will find a basic drawing of the kidney. To see other pages, use the menu on the side of your screen.

Wilms Tumor Anatomy

Larger Image

To continue reading this guide, use the menu on the side of your screen to select another section. The next section in this guide is Risk Factors, and it explains what factors may increase the chance of developing this type of tumor. Or, use the menu on the side of your screen to choose another section to continue reading this guide. 

Wilms Tumor - Childhood - Risk Factors

This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/2015

ON THIS PAGE: You will find out more about the factors that increase the chance of developing this type of tumor. To see other pages, use the menu on the side of your screen.

A risk factor is anything that increases a person’s chance of developing a tumor. Although risk factors often influence the development of a Wilms tumor, most do not directly cause it. Some children with several risk factors never develop a tumor, while others with no known risk factors do.

Most often, Wilms tumor is not inherited, but there can be genetic reasons for the tumor’s development.

  • Children may have a mutated (changed), damaged, or missing gene. This change can also cause other birth defects. About 15% of children with a Wilms tumor were born with other health problems.

  • Children with WAGR syndrome, a condition that causes a number of birth defects, have a 33% chance of developing Wilms tumor. WAGR stands for Wilms tumor, aniridia (no iris in the eye), genitourinary abnormalities (changes to the reproductive and urinary organs) or gonadoblastoma (a rare tumor of the reproductive organs), and mental retardation.

  • Beckwith-Wiedemann syndrome is a condition that causes enlarged internal organs and limbs. Children with this syndrome have a higher risk of developing Wilms tumor, kidney cysts, and tumors of the liver (hepatoblastoma), pancreas, and adrenal glands.

  • Boys with Deny-Drash syndrome, a condition in which the male sex organs do not develop correctly, have a higher risk of developing Wilms tumor.

  • A small number of children with Wilms tumor have a relative in their family with the disease.

The next section in this guide is Symptoms and Signs, and it explains what body changes or medical problems this type of tumor can cause. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Wilms Tumor - Childhood - Symptoms and Signs

This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/2015

ON THIS PAGE: You will find out more about body changes and other things that can signal a problem that may need medical care. To see other pages, use the menu on the side of your screen.

Children with a Wilms tumor may experience the following symptoms or signs. Sometimes, children with a Wilms tumor do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not a tumor. 

Usually, a Wilms tumor is found before it spreads to other parts of the body. A parent or relative may notice a large lump or feel a hard mass in the child’s belly, or notice that the belly is much larger than usual. In some cases, a rapid change to a larger diaper size alerts parents to the tumor. A few children have abdominal pain. However, most of the time the tumor grows without causing pain, and the child feels and appears healthy.

Frequently, children with Wilms tumor may experience the following symptoms:

  • Blood in the urine 

  • High blood pressure

  • Anemia, which is a low level of red blood cells

  • Fatigue

  • A fever that doesn’t go away

If you are concerned about one or more of the symptoms or signs on this list, please talk with your child’s doctor. The doctor will ask how long and how often your child has been experiencing the symptom(s), in addition to other questions. This is to help find out the cause of the problem, called a diagnosis.

If a Wilms tumor is diagnosed, relieving symptoms remains an important part of your child’s cancer care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with your child’s health care team about symptoms your child experiences, including any new symptoms or a change in symptoms.

The next section in this guide is Diagnosis, and it explains what tests may be needed to learn more about the cause of the symptoms. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Wilms Tumor - Childhood - Diagnosis

This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/2015

ON THIS PAGE: You will find a list of the common tests, procedures, and scans that doctors can use to find out what’s wrong and identify the cause of the problem. To see other pages, use the menu on the side of your screen.

Doctors use many tests to diagnose a tumor and find out if it has spread to another part of the body, called metastasis. Some tests may also determine which treatments may be the most effective. For most tumors, a biopsy is the only way to make a definitive diagnosis of cancer. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Imaging tests may be used to find out whether the tumor has spread.

This list describes options for diagnosing this type of tumor, and not all tests listed will be used for every child. Your child’s doctor may consider these factors when choosing a diagnostic test:

  • Age and medical condition

  • Type of tumor suspected

  • Signs and symptoms

  • Previous test results

In addition to a physical examination, the following tests may be used to diagnose a Wilms tumor:

  • Blood/urine tests. Doctors will take samples of your child’s blood and urine. The results of these tests will help the doctor learn more about the tumor and your child’s general health.

  • X-ray. An x-ray is a way to create a picture of the structures inside of the body, using a small amount of radiation.

  • Ultrasound. An ultrasound uses sound waves to create a picture of the internal organs. A transmitter that emits sound waves is moved over the body. A tumor generates different echoes of the sound waves than healthy tissue, so when the waves are bounced back to a computer and changed into images, the doctor can find a tumor inside the body. The procedure is painless.

  • Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. A CT scan can also be used to measure the tumor’s size. Sometimes, a special dye called a contrast medium is given before the scan to provide better detail on the image. This dye can be injected into a patient’s vein or given as a liquid to swallow.

    The first CT scan of the abdomen and pelvis will help show if the Wilms tumor has spread from the kidney into the blood vessels, particularly the inferior vena cava. The inferior vena cava is the major vein that carries blood from the legs, pelvis, and abdomen to the heart. If this is the case, the doctor will often recommend chemotherapy before surgery. (See the Treatment Options section.) A chest CT may be used to find out if the tumor has spread to the lungs.

  • Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. MRI can also be used to measure the tumor’s size. A special dye called a contrast medium is given before the scan to create a clearer picture. This dye can be injected into a patient’s vein or given as a liquid to swallow.

  • Bone x-ray and bone scan. Bone x-rays and bone scans look for cancer in the bones of children who have a rare kidney tumor called clear cell sarcoma. A bone scan uses a radioactive tracer to look at the inside of the bones. The tracer is injected into a patient’s vein. It collects in areas of the bone and is detected by a special camera. Healthy bone appears gray to the camera, and areas of injury, such as those caused by cancer, appear dark.

  • Surgery or biopsy. For children with a mass in the kidney that appears to be a stage I or stage II Wilms tumor, a surgeon usually performs an abdominal operation called a laparotomy. This surgery removes the affected kidney and tumor. If the tumor is too large to be removed, or if it has an abnormal appearance on CT or MRI scans, the surgeon will only perform a biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope.

    A pathologist then analyzes the sample(s) removed during surgery or the biopsy to determine whether cancer cells are present. If they are, the pathologist will also identify the cell type, which will help the doctor recommend an initial treatment. A pathologist is a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease.

    Patients who have what looks like bilateral Wilms tumors based on imaging studies will usually not have a biopsy at diagnosis. Instead, they usually have a biopsy after six weeks of chemotherapy.

  • Chromosome tests. The pathologist may test the tumor tissue removed during the biopsy for changes in chromosomes 1 and 16. A tumor with changes in both of these chromosomes will not respond as well to standard treatment. Current research studies for Wilms tumor use more chemotherapy to treat children with a tumor that has these changes. (See the Treatment Options section.)

After diagnostic tests are done, your child’s doctor will review all of the results with you. If the diagnosis is Wilms tumor, these results also help the doctor describe the tumor; this is called staging.

The next section in this guide is Stages, and it explains the system doctors use to describe the extent of the disease. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Wilms Tumor - Childhood - Stages

This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/2015

ON THIS PAGE: You will learn about how doctors describe a tumor’s growth or spread. This is called the stage. To see other pages, use the menu on the side of your screen.

Staging is a way of describing where the tumor is located, if or where it has spread, and whether it is affecting other parts of the body. Doctors use diagnostic tests to find out the tumor's stage, so staging may not be complete until all of the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a child's prognosis, which is the chance of recovery.

There are different stage descriptions for different types of tumors. Wilms tumor is staged based on the results of surgery, analysis of the tumor cells (see the Diagnosis section), and whether the tumor has spread.

Wilms tumor stage groupings

Stage I. The tumor is found in one kidney and can be completely removed with surgery.

Stage II. Cancer is found in the kidney and in the fat, soft tissue, or blood vessels near the kidney. It may have spread to the renal sinus. The renal sinus is the part of the kidney through which blood and fluid enter and exit the organ. The tumor can be completely removed with surgery.

Stage III. Cancer is found in areas near the kidney and cannot be completely removed with surgery. The tumor may have spread to nearby organs and blood vessels or throughout the abdomen and to nearby lymph nodes. Lymph nodes are tiny, bean-shaped organs that help fight infection. Stage III cancer has not spread outside the abdomen.

Stage IV. Cancer has spread to other more distant organs, such as the lungs, liver, bones, and brain, or lymph nodes outside the abdomen.

Stage V. Cancer cells are in both kidneys at the same time. The tumor in each kidney is staged separately.

Refractory. A refractory tumor is a tumor that does not shrink during or after treatment.

Recurrent. A recurrent tumor is a tumor that has come back after treatment. The tumor may come back in the area where it first started or in another part of the body. If the tumor does return, there will be another round of tests to learn about the extent of the recurrence. These tests and scans are often similar to those done at the time of the original diagnosis.

Source: National Wilms Tumor Study Group Staging System.

Prognostic factors

The two most important factors that predict the prognosis of a child with a Wilms tumor are:

  • What the tumor cells look like under a microscope, called the histology of the tumor. A favorable histology is usually linked to a better prognosis. Tumors with an anaplastic histology are less likely to be successfully treated with chemotherapy and/or radiation therapy and require more aggressive treatment.

  • The stage of the tumor. In general, a low stage cancer is more likely to be successfully treated than a high stage cancer. This is particularly true for Wilms tumors with an anaplastic histology.

Information about the tumor’s stage will help the doctor recommend a specific treatment plan. The next section in this guide is Treatment Options. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Wilms Tumor - Childhood - Treatment Options

This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/2015

ON THIS PAGE: You will learn about the different ways doctors use to treat children with this type of tumor. To see other pages, use the menu on the side of your screen.

In general, cancer in children is uncommon, so it can be hard for doctors to plan treatments unless they know what has been most effective in other children. That’s why more than 60% of children with cancer are treated as part of a clinical trial.

Wilms tumor is a success story for improving the prognosis of children with cancer using clinical trials. Clinical trials are research studies that compare standard treatments (the best known treatments available) with newer approaches to treatments that may be more effective. Clinical trials may test such approaches as a new drug, a new combination of standard treatments, or new doses of current therapies. Studying new treatments involves careful monitoring using scientific methods, and all participants are followed closely to track their health and progress.

To take advantage of these newer treatments, children with cancer should be treated at a specialized cancer center. Doctors at these centers have extensive experience in treating children with cancer and have access to the latest research. A doctor who specializes in treating children with cancer is called a pediatric oncologist. If a pediatric cancer center is not nearby, general cancer centers sometimes have pediatric specialists who are able to be part of your child’s care.

In many cases, a team of doctors works with a child and the family to provide care; this is called a multidisciplinary team. Pediatric cancer centers often have extra support services for children and their families, such as child life specialists, dietitians, physical and occupational therapists, social workers, and counselors. Special activities and programs to help your child and family cope may also be available.

Descriptions of the most common treatment options for Wilms tumor are listed below. The main treatments for Wilms tumor are surgery, chemotherapy, and radiation therapy. Treatment options and recommendations depend on several factors, including the type and stage of cancer, possible side effects, and the child and family’s preferences and overall health. Your child’s care plan may also include treatment for symptoms and side effects, an important part of cancer care.

Take time to learn about all of your child's treatment options and be sure to ask questions about things that are unclear. Also, talk about the goals of each treatment with your child's doctor and what you and your child can expect while receiving the treatment. Learn more about making treatment decisions.

Surgery

Surgery is the removal of the tumor and some surrounding healthy tissue during an operation. Surgery is a common treatment for all stages of Wilms tumor. It will usually be performed by either a pediatric surgeon with experience removing cancer in children or a pediatric urologist. A pediatric urologist is a doctor who specializes in treating urinary tract problems in children.

The types of surgery used to treat Wilms tumor include:

  • Radical nephrectomy. A radical nephrectomy is the removal of the whole kidney and some surrounding tissue, as well as nearby lymph nodes. The surgical removal of the lymph nodes is called a lymph node dissection.

  • Partial nephrectomy. A partial nephrectomy is the removal of the tumor and some of the surrounding healthy tissue, leaving as much of the kidney as possible.

When one kidney is removed, the other kidney takes over the full job of filtering wastes from the body. A partial nephrectomy is performed when the other kidney is damaged, also contains a tumor, or has already been removed. Dialysis, a mechanized filtering process, may also be used if preserving one functioning kidney is not possible.

Talk with the health care team about the possible side effects of your child’s specific surgery and how they can be reduced or relieved. Learn more about the basics of surgery.

Chemotherapy

Chemotherapy is the use of drugs to destroy cancer cells, usually by stopping the cancer cells’ ability to grow and divide. Chemotherapy for Wilms tumor is given by a pediatric oncologist.

Systemic chemotherapy gets into the bloodstream to reach cancer cells throughout the body. Chemotherapy for Wilms tumor is usually given through an intravenous (IV) tube placed into a vein using a needle.

A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a set period of time. A patient may receive one drug at a time or combinations of different drugs at the same time. Common drugs for Wilms tumor with a favorable histology include dactinomycin (Cosmegen), doxorubicin (Adriamycin), and vincristine (Vincasar PFS, Oncovin). Other drugs being used to treat Wilms tumor include cyclophosphamide (Cytoxan, Neosar), etoposide (VePesid, Toposar), and irinotecan (Camptosar).

Chemotherapy may be given after surgery to eliminate any remaining cancer cells, called adjuvant chemotherapy. Sometimes, chemotherapy is given before surgery to shrink a tumor that is too large to remove or a tumor that is growing into blood vessels or other organs, making it too risky to remove.

The side effects of chemotherapy depend on the individual and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, hair loss, loss of appetite, and diarrhea. These side effects usually go away once treatment is finished.

Learn more about the basics of chemotherapy and preparing for treatment. The medications used to treat cancer are continually being evaluated. Talking with your child’s doctor is often the best way to learn about the medications prescribed for your child, their purpose, and their potential side effects or interactions with other medications. Learn more about your child’s prescriptions by using searchable drug databases.

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to destroy cancer cells. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist.

The most common type of radiation treatment is called external-beam radiation therapy, which is radiation therapy given from a machine outside the body. When radiation treatment is given using implants, it is called internal radiation therapy or brachytherapy. A radiation therapy regimen (schedule) usually consists of a specific number of treatments given over a set period of time.

Radiation therapy may be used to treat a large tumor or a tumor that has spread to other parts of the body. It may also be used to shrink a tumor before surgery or destroy cancer cells that remain after surgery. Radiation therapy is usually part of the treatment plan for children with stage III or IV Wilms tumor with a favorable histology and for all children who have tumors with an anaplastic histology (see the Stages section for more information).

Side effects from radiation therapy depend on the part of the body treated. For example, side effects from radiation therapy to the abdomen can include fatigue, mild skin reactions, upset stomach, and loose bowel movements. Most side effects go away soon after treatment is finished. Learn more about the basics of radiation therapy.

Getting care for symptoms and side effects

Cancer and its treatment often cause side effects. In addition to treatment to slow, stop, or eliminate the cancer, an important part of cancer care is relieving a child’s symptoms and side effects. This approach is called palliative care, and it includes supporting your child with his or her physical, emotional, and social needs.

Palliative care is any treatment that focuses on reducing symptoms, improving quality of life, and supporting patients and their families. Any person, regardless of age or type and stage of cancer, may receive palliative care. It works best when palliative care is started as early as needed in the cancer treatment process.

Children often receive treatment for the cancer and treatment to ease side effects at the same time. In fact, patients who receive both often have less severe symptoms, better quality of life, and report they are more satisfied with treatment.

Palliative treatments vary widely and often include medication, nutritional changes, relaxation techniques, emotional support, and other therapies. Your child may also receive palliative treatments similar to those meant to eliminate the cancer, such as chemotherapy, surgery, or radiation therapy. Talk with your child's doctor about the goals of each treatment in his/her treatment plan.

Before treatment begins, talk with your child’s health care team about the possible side effects of his/her specific treatment plan and palliative care options. And during and after treatment, be sure to tell your child’s doctor or another health care team member if your child is experiencing a problem so it can be addressed as quickly as possible. Learn more about palliative care.   

Remission and the chance of recurrence

A remission is when cancer cannot be detected in the body and there are no symptoms. This may also be called having “no evidence of disease” or NED. 

A remission may be temporary or permanent. This uncertainty causes many people to worry that the tumor will come back. While many remissions are permanent, it’s important to talk with your child’s doctor about the possibility of your child’s tumor returning. Understanding your child’s risk of recurrence and the treatment options may help you and your child feel more prepared if the cancer does return. Learn more about coping with the fear of recurrence

If the tumor does return after the original treatment, it is called a recurrent tumor. It may come back in the same place (called a local recurrence), nearby (regional recurrence), or in another place (distant recurrence).

When this occurs, a cycle of testing will begin again to learn as much as possible about the recurrence. After testing is done, your child’s doctor will talk with you about treatment options. The treatment plan for recurrent Wilms tumor depends on four factors:

  • Where the cancer has returned in the body

  • The type of treatment your child received for the original tumor

  • How long it has been since the original cancer was treated

  • Whether the new growth has a favorable or anaplastic histology

Often the treatment plan will include the treatments described above, such as surgery, radiation therapy, and chemotherapy, but they may be used in a different combination or given at a different pace. Your child’s doctor may also suggest clinical trials that are studying new ways to treat this type of recurrent tumor, including new medications. Whichever treatment plan you choose, palliative care will be important for relieving symptoms and side effects.

When a tumor recurs, children and their families often experience emotions such as disbelief or fear. Families are encouraged to talk with the health care team about these feelings and ask about support services to help them cope. Learn more about dealing with cancer recurrence.

If treatment fails

Although treatment is successful for the majority of children with a Wilms tumor, sometimes it is not. If a child’s cancer cannot be cured or controlled, this is called advanced or terminal cancer. This diagnosis is stressful, and advanced cancer may be difficult to discuss. However, it is important to have open and honest conversations with your child’s doctor and health care team to express your family’s feelings, preferences, and concerns. The health care team is there to help, and many team members have special skills, experience, and knowledge to support patients and their families.

Parents or guardians are encouraged to think about where their child would be most comfortable: at home, in a home-like setting elsewhere, in the hospital, or in a hospice environment. Hospice care is a type of palliative care for people who are expected to live less than six months. It is designed to provide the best possible quality of life for people who are near the end of life. Nursing care and special equipment can make staying at home a workable alternative for many families. Some children may be happier if they can arrange to attend school part-time or keep up other activities and social connections. Your child’s health care team can help you decide on an appropriate level of activity. Making sure a child is physically comfortable and free from pain is extremely important as part of end-of-life care. Learn more about caring for a terminally ill child and advanced cancer care planning.

The death of a child is an enormous tragedy, and families may need support to help them cope with the loss. Pediatric cancer centers often have professional staff and support groups to help with the process of grieving. Learn more on grieving the loss of a child.

The next section in this guide is About Clinical Trials, and it offers more information about research studies that are focused on finding better ways to care for children with a Wilms tumor. Or, use the menu on the side of your screen to choose another section to continue reading this guide.  

Wilms Tumor - Childhood - About Clinical Trials

This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/2015

ON THIS PAGE: You will learn more about clinical trials, which are the main way that new medical approaches are tested to see how well they work. To see other pages, use the menu on the side of your screen.

What are clinical trials?

Doctors and scientists are always looking for better ways to care for children with a Wilms tumor. To make scientific advances, doctors create research studies involving volunteers, called clinical trials. Wilms tumor is one of the best examples of the impact of clinical trials on finding the best treatment approaches to improve outcomes and avoid side effects.

Many clinical trials are focused on new treatments, evaluating whether a new treatment is safe, effective, and possibly better than the current (standard) treatment. These types of studies evaluate new drugs, different combinations of existing treatments, new approaches to radiation therapy or surgery, and new methods of treatment. Children who participate in clinical trials are often among the first to receive new treatments before they are widely available. However, there is no guarantee that the new treatment will be safe, effective, or better than a standard treatment.

There are also clinical trials that study new ways to ease symptoms and side effects during treatment and managing the late effects that may occur after treatment. Talk with your child’s doctor about clinical trials regarding side effects.

Deciding to join a clinical trial

Parents and children decide to participate in clinical trials for many reasons. For some, a clinical trial is the best treatment option available. Because standard treatments are not perfect, people are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other parents and children volunteer for clinical trials because they know that these studies are the only way to make progress in treating Wilms tumor. Even if your child does not benefit directly from the clinical trial, his or her participation may benefit future children with a Wilms tumor.

Sometimes people have concerns that, in a clinical trial, their child may receive no treatment by being given a placebo or a “sugar pill.” The use of placebos in cancer clinical trials in this way is rare overall and not done at all in childhood cancer research. Find out more about placebos in cancer clinical trials.

Patient safety and informed consent

To join a clinical trial, parents and children must participate in a process known as informed consent. During informed consent, the doctor should list all of the child’s options so both parents and children understand how the new treatment differs from the standard treatment. The doctor must also list all of the risks of the new treatment, which may or may not be different from the risks of standard treatment. Finally, the doctor must explain what will be required of each parent and child in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment.

Patients who participate in a clinical trial may stop participating at any time for any personal or medical reason. This may include that the new treatment is not working or there are serious side effects. Clinical trials are also closely monitored by experts who watch for any problems with each study. It is important that parents of patients participating in a clinical trial talk with their child’s doctor and researchers about who will be providing their treatment and care during the clinical trial, after the clinical trial ends, and/or if the patient chooses to leave the clinical trial before it ends. 

Finding a clinical trial

Research through clinical trials is ongoing for all types of cancer. For specific topics being studied for Wilms tumor, learn more in the Latest Research section. In particular, the Children’s Oncology Group conducts clinical trials for most types of childhood cancer, including Wilms tumor.

Cancer.Net offers a lot of information about cancer clinical trials in other areas of the website, including a complete section on clinical trials and places to search for clinical trials for a specific type of cancer.

In addition, this website offers free access to a video-based educational program about cancer clinical trials, located outside of this guide.

The next section in this guide is Latest Research, and it explains areas of scientific research currently going on for this type of tumor. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Wilms Tumor - Childhood - Latest Research

This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/2015

ON THIS PAGE: You will read about the scientific research being done now to learn more about this type of tumor and how to treat it. To see other pages, use the menu on the side of your screen.

Doctors are working to learn more about Wilms tumor, ways to prevent it, how to best treat it, and how to provide the best care to people diagnosed with this disease. The following areas of research may include new options for patients through clinical trials. Always talk with your child’s doctor about the diagnostic and treatment options that are best for your family.

  • Bilateral Wilms tumors. The Children’s Oncology Group is studying how to treat bilateral Wilms tumors while preserving as much kidney function as possible.

  • Tumor markers. A tumor marker, also called a biomarker, is a substance found in a person's blood, urine, or body tissue that indicates a possible disease. Researchers have found that Wilms tumors with a favorable histology and changes in chromosome 1q, also called WT-1, are more likely to come back after treatment. In the future, this may be used to identify patients who are at an increased risk for recurrence.

  • Tumor genetics. Another Wilms tumor gene has been identified on the X chromosome, called WTX. This gene plays a role in normal kidney development, but it is not active in approximately one-third of children with Wilms tumor. Studies on the importance of this gene are ongoing.

  • Targeted therapy. Targeted therapy is a treatment that targets the cancer’s specific genes, proteins, or the tissue environment that contributes to cancer growth and survival. This type of treatment blocks the growth and spread of cancer cells while limiting damage to healthy cells.

    The Children's Oncology Group is currently conducting a phase II study of an antibody-drug conjugate called IMGN901 (lorvotuzumab mertansine) for the treatment of recurrent or refractory Wilms tumors. This treatment is designed to deliver the drug directly to the cancer cells. Very early studies have shown promising results.

  • New treatment approaches for tumors that have spread to the lungs. Researchers are trying to figure out if a favorable histology Wilms tumor that has spread to the lungs should be treated with radiation therapy if the lung tumors go away after six weeks of chemotherapy. Researchers are also using a different chemotherapy, as well as radiation therapy to the lungs, to treat lung tumors that do not go away after six weeks.

  • New treatment methods. Doctors are looking at the effect of using only surgery as a treatment for patients with a small stage I tumor. After surgery, the patients are watched closely for a recurrence. 

  • Palliative care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current Wilms tumor treatments in order to improve children’s comfort and quality of life.

Looking for More About the Latest Research?

If you would like additional information about the latest areas of research regarding Wilms tumor, explore these related items that will take you outside of this guide:

  • To find clinical trials specific to your diagnosis, talk with your child’s doctor or search online clinical trial databases now.

  • Review research announced at the 2015 ASCO Annual Meeting.

  • Visit ASCO’s CancerProgress.Net website to learn more about the historical pace of research for childhood cancer, called pediatric cancer on this website. Please note this link takes you to a separate ASCO website.

  • Visit the website of the Conquer Cancer Foundation to find out how to help support research for every cancer type. Please note this link takes you to a separate ASCO website. 

The next section in this guide is Coping with Side Effects, and it offers some guidance on how to cope with the physical, emotional, and social changes that a tumor and its treatment can bring. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Wilms Tumor - Childhood - Coping with Side Effects

This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/2015

ON THIS PAGE: You will find out more about steps to take to help your child cope with physical, social, and emotional side effects. This page includes several links outside of this guide to other sections of this website. To see other pages, use the menu on the side of your screen.

Fear of treatment side effects is common after a diagnosis of a Wilms tumor, but it may help to know that preventing and controlling side effects is a major focus of your child’s health care team. This is called palliative care, and it is an important part of the overall treatment plan, regardless of the stage of disease.

There are possible side effects for every cancer treatment, but patients don’t experience the same side effects when given the same treatments for many reasons. That can make it hard to predict exactly how your child will feel during treatment. 

Common side effects from each treatment option for Wilms tumor are described in detail within the Treatment Options section. Learn more about the most common side effects of a tumor and different treatments, along with ways to prevent or control them. Side effects depend on a variety of factors, including the tumor’s stage, the length and dosage of treatment(s), and your child’s overall health.

Talking with the health care team about side effects

Before treatment begins, talk with your child’s doctor about possible side effects of each type of treatment your child will be receiving. Ask which side effects are most likely to happen, when they are likely to occur, and what can be done to prevent or relieve them.

And, ask about the level of caregiving your child may need during treatment and recovery, as family members and friends often play an important role in the care of a person with Wilms tumor. Learn more about caregiving.

In addition to physical side effects, there may be emotional and social effects as well. Patients and their families are encouraged to share their feelings with a member of the health care team who can help with coping strategies, including concerns about managing the cost of your child’s cancer care

During and after treatment, be sure to tell a member of the health care team about the side effects your child experiences, even if you feel they are not serious. Sometimes, side effects can last beyond the treatment period, called a long-term side effect. A side effect that occurs months or years after treatment is called a late effect. Treatment of all types of side effects is an important part of survivorship care. Learn more by reading the Late Effects of Treatment or the Follow-up Care section of this guide section or talking with your child’s doctor.

The next section in this guide is Late Effects of Treatment, and it describes some of the side effects that can occur long after treatment has finished. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Wilms Tumor - Childhood - Late Effects of Treatment

This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/2015

ON THIS PAGE: You will find out more about side effects that can occur long after treatment for this type of tumor has finished. To see other pages, use the menu on the side of your screen.

Although many children with Wilms tumor are treated successfully, they often have an increased risk of developing other diseases or conditions later in life, as chemotherapy and radiation therapy can cause permanent damage to healthy parts of the body. Therefore, it is important that children who have received treatment for a Wilms tumor are monitored closely for possible long-term or late effects of the treatment. These may include:

  • Kidney failure. Because children treated for Wilms tumor usually have only one kidney, they have a slightly higher risk of kidney failure and will need to be monitored for the rest of their lives. They also need to keep well hydrated to help promote good kidney health. There are some medications such as codeine and ibuprofen that should only be taken in small amounts to avoid kidney damage.

  • Bowel obstruction. Children treated with certain types of surgery are at risk for developing a bowel obstruction throughout their lives from scar tissue. A bowel obstruction will cause abdominal pain and vomiting.

  • Heart problems. Children who received doxorubicin have a higher risk of developing heart abnormalities, even years after treatment has ended. Children treated with doxorubicin and radiation therapy to the lungs have an even higher risk of developing heart problems. An echocardiogram may be used to monitor the effects of doxorubicin on the heart.

  • Lung problems. Children who received radiation therapy to their lungs for a metastatic Wilms tumor are at risk for developing lung problems after treatment has ended. Pulmonary function tests can be done to see if there are any changes to the lungs.

  • Skeletal abnormalities. If radiation therapy was given, scoliosis (curvature of the spine) and underdevelopment of nearby soft tissue may occur. This risk depends on the location and dose of the radiation therapy.

  • Secondary cancer. Wilms tumor survivors have a small risk of developing another type of cancer within 15 years after the Wilms tumor was first diagnosed. The most important risk factors are whether treatment included radiation therapy and doxorubicin. For example, young girls who have radiation therapy to treat a Wilms tumor that has spread to the lungs have an increased risk of developing breast cancer.

  • Pregnancy concerns. Females who had abdominal radiation therapy for a Wilms tumor should be considered high-risk during pregnancy and monitored closely by their doctors. Chemotherapy alone does not increase the risk of miscarriage; however, women who received radiation therapy aimed at the side of the body between the rib and hip, depending on the dose, are more likely to have early labor, an unusual position of the baby’s head during birth, lower birth weight, and premature delivery (less than 36 weeks) based on National Wilms Tumor Study records. Congenital abnormalities are also more common.

The next section in this guide is Follow-up Care, and it explains the importance of check-ups after treatment has finished. Or, use the menu on the side of your screen to choose another section to continue reading this guide. 

Wilms Tumor - Childhood - Follow-Up Care

This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/2015

ON THIS PAGE: You will read about your child’s medical care after treatment for Wilms tumor is finished and why this follow-up care is important. To see other pages, use the menu on the side of your screen.

Care for children diagnosed with a Wilms tumor doesn’t end when active treatment has finished. Your child’s health care team will continue to check to make sure the tumor has not returned, manage any side effects, and monitor your child’s overall health. This is called follow-up care. All children treated for cancer, including Wilms tumor, should have life-long, follow-up care. Learn more about the importance of follow-up care.

Follow-up care for a Wilms tumor includes regular visits to the doctor for physical examinations, blood and urine tests, and imaging tests, such as CT scans, ultrasounds, and x-rays. At first, these visits and tests will be more frequent, such as every three months. The time between checkups will increase until five years after treatment when your child will be scheduled for a follow-up visit once a year.

Watching for recurrence

One goal of follow-up care is to check for a recurrence. A tumor recurs because small areas of cancer cells may remain undetected in the body. Over time, these cells may increase in number until they show up on test results or cause signs or symptoms.

During follow-up care, a doctor familiar with your child’s medical history can give you personalized information about the risk of recurrence. The doctor will also ask specific questions about your child’s health. Some children may have blood tests or imaging tests as part of regular follow-up care, but testing recommendations depend on several factors, including the type and stage of tumor originally diagnosed and the types of treatment given.

Managing long-term and late side effects of childhood cancer

The longer the time since treatment ended, the less likely the tumor will return. At this point, monitoring for late effects becomes an important part of follow-up visits. Late effects can occur almost anywhere in the body and include physical problems, such as heart and lung problems and second cancers, and emotional and cognitive (memory, thinking, and attention) problems, such as anxiety, depression, and learning difficulties.

Based on the type of treatment your child received, the doctor will recommend the examinations and tests needed to check for late effects. Usually, patients will have blood tests to make sure no changes have occurred in their kidney function. This is especially important for patients who have one kidney or had a partial nephrectomy. Follow-up care should also address your child’s quality of life, including any developmental or emotional concerns.

The Children's Oncology Group (COG) has studied the physical and psychological effects that childhood cancer survivors face. Based on these studies, COG has created recommendations for long-term follow-up care for childhood, adolescent, and young adult cancer survivors that can be found on a separate website: www.survivorshipguidelines.org.

Keeping a child’s personal health record

You are encouraged to organize and keep a personal record of your child’s medical information. The doctor will help you create this. That way, as your child enters adulthood, he or she has a clear, written history of the diagnosis, the treatment given, and the doctor’s recommendations about the schedule for follow-up care. ASCO offers forms to help create a treatment summary to keep track of the cancer treatment your child received and develop a survivorship care plan once treatment is completed.

Some children continue to see their oncologist, while others transition back to the general care of their family doctor or another health care professional. This decision depends on several factors, including the type and stage of cancer, side effects, health insurance rules, and your family’s personal preferences. Talk with your health care team about your child’s ongoing medical care and any concerns you have about his or her future health.

If a doctor who was not directly involved in your child’s cancer care will lead the follow-up care, be sure to share the cancer treatment summary and survivorship care plan forms with him or her, as well as all future health care providers. Details about the specific cancer treatment given are very valuable to the health care professionals who will care for your child throughout his or her lifetime.

The next section in this guide is Survivorship, and it describes how to cope with challenges in everyday life after a cancer diagnosis. Or, use the menu on the side of your screen to choose another section to continue reading this guide.  

Wilms Tumor - Childhood - Survivorship

This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/2015

ON THIS PAGE: You will read about how to cope with challenges in everyday life after your child’s cancer diagnosis. To see other pages, use the menu on the side of your screen.

What is survivorship?

The word survivorship means different things to different people, but it often describes the process of living with, through, and beyond cancer. In some ways, survivorship is one of the most complex aspects of the cancer experience because it is different for every patient and his or her family.

After active cancer treatment ends, children and their families may experience a mixture of strong feelings, including joy, concern, relief, guilt, and fear. Some people say they appreciate life more after a cancer diagnosis. Other families stay very anxious about their child’s health and become uncertain of how to cope with everyday life.

One source of stress may occur when frequent visits to the health care team end following treatment. Often, relationships built with the cancer care team provide a sense of security during treatment, and children and their families miss this source of support. This may be especially true as new worries and challenges surface over time, such as any late effects of treatment, educational issues, emotional challenges, sexual development and fertility concerns, and/or financial issues.

Every family faces different concerns and challenges. With any challenge, a good first step is being able to recognize each fear and talk about it. Effective coping requires:

  • Understanding the challenge your family is facing,

  • Thinking through solutions,

  • Asking for and allowing the support of others, and

  • Feeling comfortable with the course of action your family chooses.

It may be helpful for your child to join an in-person support group or online community of childhood cancer survivors. Support groups also exist for parents of children diagnosed with cancer. This allows you to talk with people who have had similar first-hand experiences. Other options for finding support include talking with a friend or member of the health care team, individual counseling, or asking for assistance at the learning resource center of the center where your child received treatment.

Changing role of caregivers

A caregiver plays a very important role in supporting a child diagnosed with cancer, providing physical, emotional, and practical care on a daily or as-needed basis. Many caregivers become focused on providing this support, especially if the treatment period lasts for many months or longer.

However, as treatment is completed, the caregiver's role often changes. Eventually, the need for caregiving related to a child’s cancer diagnosis will become much less or come to an end as your child gets older. Family counselors at pediatric cancer centers can help with this transition. You can also learn more about adjusting to life after caregiving in this article.

Healthy living after cancer

Survivorship often serves as a strong motivator to make positive lifestyle changes, often for the family as a whole.

Children who have had cancer can enhance the quality of their future by following established guidelines for good health into and through adulthood, including not smoking, maintaining a healthy weight, eating well, managing stress, and participating in regular physical activity. Talk with the doctor about developing a plan that is best for your child’s needs. Learn more about making healthy lifestyle choices.

In addition, it is important that your child has recommended medical check-ups and tests (see Follow-up Care) to take care of his or her health. Cancer rehabilitation may be recommended, and this could mean any of a wide range of services such as physical therapy, family or individual counseling, nutritional planning, and/or educational assistance. The goal of rehabilitation is to help survivors and their families regain control over many aspects of their lives and remain as independent and productive as possible.

Talk with your child’s doctor to develop a survivorship care plan that is best for your child’s needs.

Looking for More Survivorship Resources?

For more information about cancer survivorship, explore these related items. Please note these links will take you to other sections of Cancer.Net:

  • Survivorship Resources: Cancer.Net offers a lot of information and resources to help survivors cope, including specific sections for children, teens, and young adults. There is also a main section on survivorship for people of all ages.

  • ASCO Answers Cancer Survivorship Guide: This 44-page booklet (available as a PDF) can help with the transition into life after treatment. It includes blank treatment summary and survivorship care plan forms.

  • Cancer.Net Patient Education Video: View a short video led by an ASCO expert that provides information about childhood cancer survivorship.

The next section offers Questions to Ask the Doctor to help start conversations with your child’s cancer care team. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Wilms Tumor - Childhood - Questions to Ask the Doctor

This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/2015

ON THIS PAGE: You will find some questions to ask your child's doctor or other members of your child's health care team, to help you and your child better understand his/her diagnosis, treatment plan, and overall care. To see other pages, use the menu on the side of your screen.

Talking often with the doctor is important to make informed decisions about your child’s health care. These suggested questions are a starting point to help you learn more about your child’s cancer care and treatment. You are also encouraged to ask additional questions that are important to your family. You may want to print this list and bring it to your child's next appointment, or download Cancer.Net’s free mobile app for an e-list and other interactive tools to manage your child’s care.

Questions to ask after getting a diagnosis

  • Can you explain my child’s pathology report (laboratory test results) and radiology reports (imaging test results) to me?

  • Can you give me copies of these reports so I can have a record of them?

  • What is the histology of the tumor? What does this mean?

  • What is the stage of the tumor? What does this mean?

Questions to ask about choosing a treatment and managing side effects

  • What are my child’s treatment options?

  • What clinical trials are open to my child? Where are they located, and how can we find out more about them?

  • What treatment plan do you recommend? Why?

  • What is the goal of each treatment? Is it to eliminate the cancer, help my child feel better, or both?

  • What are the possible side effects of each treatment option, both in the short term and the long term?

  • Who will be part of my child’s health care team, and what does each member do?

  • Who will be coordinating my child’s overall treatment?

  • How long will my child receive treatment?

  • How will this treatment affect my child’s daily life? Will he or she be able to go to school and perform his or her usual activities?

  • What should I do if my child has abdominal pain with vomiting?

  • Will this treatment change how my child goes through puberty?

  • Will this treatment affect my child’s dental care?

  • Can my child play sports and, if so, which ones? Are there any precautions we need to take when he or she participates in sports?

  • Could this treatment affect my child’s ability to become pregnant or have children in the future? If so, should we talk with a fertility specialist before cancer treatment begins?

  • If my child has children in the future, what is their risk of developing a Wilms tumor?

  • If I’m worried about managing the costs related to my child’s medical care, who can help me with these concerns?

  • What support services are available to my child? To my family?

  • Whom should I call for questions or problems?

  • Is there anything else I should be asking?

Questions to ask about having surgery

  • What type of surgery will my child have?

  • How long will the operation take?

  • How long will my child need to be in the hospital?

  • Can you describe what the recovery from surgery will be like?

  • What are the possible long-term effects of having this surgery?

Questions to ask about having radiation therapy or chemotherapy

  • What type of treatment is recommended?

  • What is the goal of this treatment?

  • How long will it take to give this treatment?

  • What side effects can my child expect to experience during treatment?

  • What are the possible long-term effects of having this treatment?

  • What can be done to relieve or manage these side effects?

Questions to ask about planning follow-up care

  • What is the risk of the tumor returning? Are there signs and symptoms we should watch for?

  • What long-term side effects or late effects are possible based on the cancer treatment my child received?

  • What follow-up tests will my child need, and how often will he or she need them?

  • How do I get a treatment summary and survivorship care plan to keep in my child’s personal records?

  • Who will be coordinating my child’s follow-up care?

  • What survivorship support services are available to my child? To my family?

The next section in this guide is Additional Resources, and it offers some more resources on this website beyond this guide that may be helpful to you. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Wilms Tumor - Childhood - Additional Resources

This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/2015

ON THIS PAGE: You will find some helpful links to other areas of Cancer.Net that provide information about cancer care and treatment. This is the final page of Cancer.Net’s Guide to Wilms Tumor. To go back and review other pages, use the menu on the side of your screen.

Cancer.Net includes many other sections about the medical and emotional aspects of cancer, both for the child diagnosed and their family members and friends. This website is meant to be a resource for you and your loved ones from the time of diagnosis, through treatment, and beyond.

Beyond this guide, here are a few links to help you explore other parts of Cancer.Net:

This is the end of the Cancer.Net’s Guide to Wilms Tumor. Use the menu on the side of your screen to select another section, to continue reading this guide.