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Wilms Tumor - Childhood - Introduction

Approved by the Cancer.Net Editorial Board, 02/2018

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. Use the menu to see other pages. Think of that menu as a roadmap for this complete guide.

About the kidneys

Every person has 2 kidneys, which are located above the waist on both sides of the spine. These reddish-brown, bean-shaped organs are located closer to the back of the body than to the front. The kidneys filter blood to remove impurities, excess minerals and salts, and extra water. They also produce hormones that help control blood pressure, red blood cell production, and other bodily functions.

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 3 or 4 years old, but some may grow out of control, 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 1 kidney, called unilateral. However, it can develop in both kidneys, called bilateral. Rarely, a Wilms tumor develops in 1 kidney first and then the other.

A Wilms tumor is often found only after it has grown to a size of about 8 ounces, which is about 4 times the weight of a healthy 3-year-old child’s kidney. About 1 out of 4 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 Introduction?

If you would more of an introduction, explore these related items. Please note that these links will take you to other sections on Cancer.Net:

  • ASCO Answers Fact Sheet: Read a 1-page fact sheet that offers an introduction to Wilms tumor. This fact sheet is available as a PDF, so it is easy to print out.

  • 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.

The next section in this guide is Statistics. It helps explain the number of children who are diagnosed with a Wilms tumor and general survival rates. Use the menu to choose a different section to read in this guide.

Wilms Tumor - Childhood - Statistics

Approved by the Cancer.Net Editorial Board, 02/2018

ON THIS PAGE: You will find information about the number of children who are diagnosed with a Wilms tumor each year. You will also read general information on surviving the disease. Remember, survival rates depend on several factors. Use the menu to see other pages.

In the United States, about 500 children are diagnosed with a Wilms tumor each year. The disease accounts for about 5% of all childhood cancers. Wilms tumor occurs most often in young children between the ages of 3 and 4. It is uncommon after age 6.

The 5-year survival rate tells you what percent of children live at least 5 years after the cancer is found. Percent means how many out of 100. The 5-year survival rate for children with Wilms tumor is 93%. However, the rate varies according to the stage of the disease.

Stage I, II, and III tumors with a favorable histology have a 4-year survival rate that ranges from 94% to 99%. Stage IV and V tumors have a 4-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.

It is important to remember that statistics on the survival rates for children with a Wilms tumor are an estimate. The estimate comes from annual data based on the number of children with this tumor in the United States. Also, experts measure the survival statistics every 4 or 5 years. So the estimate may not show the results of better diagnosis or treatment available for less than 4 or 5 years. People should talk with their child’s doctor if they have any questions about this information. Learn more about understanding statistics.

Statistics adapted from the American Cancer Society's (ACS) publication, Cancer Facts and Figures 2018, and the ACS website.

The next section in this guide is Medical Illustrations. It offers drawings of body parts often affected by a Wilms tumor. Use the menu to choose a different section to read in this guide.

Wilms Tumor - Childhood - Medical Illustrations

Approved by the Cancer.Net Editorial Board, 02/2018

ON THIS PAGE: You will find a drawing of a kidney. Use the menu to see other pages.

Every person has 2 kidneys, which are located above the waist on both sides of the spine. These reddish-brown, bean-shaped organs are each about the size of a small fist. They are located closer to the back of the body than to the front. The renal artery brings blood to the kidney and branches into the multi-sectioned medulla. Surrounding the medulla is the cortex. The renal pelvis is the broad opening at the top of the ureter. The renal pelvis connects the center of the kidney to the ureter, which connects to the bladder. Copyright 2004 American Society of Clinical Oncology. Robert Morreale/Visual Explanations, LLC.

The next section in this guide is Risk Factors. It explains what factors may increase the chance of developing a Wilms tumor. Use the menu to choose a different section to read in this guide.

Wilms Tumor - Childhood - Risk Factors

Approved by the Cancer.Net Editorial Board, 02/2018

ON THIS PAGE: You will find out more about the factors that increase the chance of developing Wilms tumor. Use the menu to see other pages.

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.

  • Genetic changes. 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.

  • WAGR syndrome. WAGR syndrome is a condition that causes a number of birth defects. Children with this syndrome have a 33% chance of developing Wilms tumor. WAGR stands for:

    • Wilms tumor

    • Aniridia (no iris in the eye)

    • Genitourinary abnormalities, which are changes to the reproductive and urinary organs, or gonadoblastoma, which is a rare tumor of the reproductive organs

    • Intellectual disability, once called mental retardation

  • Beckwith-Wiedemann syndrome. This condition 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.

  • Denys-Drash syndrome. This is a condition in which a boy’s sex organs do not develop correctly. Boys with this syndrome have a higher risk of developing Wilms tumor.

  • Family history. 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. It explains what body changes or medical problems a Wilms tumor can cause. Use the menu to choose a different section to read in this guide.

Wilms Tumor - Childhood - Symptoms and Signs

Approved by the Cancer.Net Editorial Board, 02/2018

ON THIS PAGE: You will find out more about body changes and other things that can signal a problem that may need medical care. Use the menu to see other pages.

Children with a Wilms tumor may experience the following symptoms or signs. Sometimes, children with a Wilms tumor do not have any of these changes. Or, the cause of a symptom may be a different medical condition that is not cancer.

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 presence of a 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 a 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 any changes your child experiences, 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 figure 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 be called palliative care or supportive care. Be sure to talk with your child’s health care team about the symptoms your child experiences, including any new symptoms or a change in symptoms.

The next section in this guide is Diagnosis. It explains what tests may be needed to learn more about the cause of the symptoms. Use the menu to choose a different section to read in this guide.

Wilms Tumor - Childhood - Diagnosis

Approved by the Cancer.Net Editorial Board, 02/2018

ON THIS PAGE: You will find a list of common tests, procedures, and scans that doctors use to find the cause of a medical problem. Use the menu to see other pages.

Doctors use many tests to find, or diagnose, a Wilms tumor. They also do tests to learn if the cancer has spread to another part of the body from where it started. If this happens, it is called metastasis. For example, imaging tests can show if the tumor has spread. Imaging tests show pictures of the inside of the body. Doctors may also do tests to learn which treatments could work best.

For most types of tumors, a biopsy is the only sure way for the doctor to know if one is present. In a biopsy, the doctor takes a small sample of tissue for testing in a laboratory. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis.

This list describes options for diagnosing a Wilms tumor. Not all tests listed below will be used for every child. Your child’s doctor may consider these factors when choosing a diagnostic test:

  • The type of cancer suspected

  • Your signs and symptoms

  • Your age and general health

  • The results of earlier medical tests

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 creates 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 takes pictures of the inside of the body using x-rays taken from different angles. A computer combines these images into a detailed, 3-dimensional image that shows any abnormalities or tumors. A CT scan can 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 person's vein or given as a pill or 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 Types of Treatment). 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 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 person's vein or given as a pill or 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 person'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) to determine whether tumor 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.

    People who have what looks like bilateral Wilms tumors (see Introduction) based on scans will usually not have a biopsy at the time of diagnosis. Instead, they usually have a biopsy after 6 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 use more chemotherapy to treat children with a Wilms tumor that has these changes (see Types of Treatment).

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. It explains the system doctors use to describe the extent of the disease. Use the menu to choose a different section to read in this guide.

Wilms Tumor - Childhood - Stages

Approved by the Cancer.Net Editorial Board, 02/2018

ON THIS PAGE: You will learn about how doctors describe a tumor’s growth or spread. This is called the stage. Use the menu to see other pages.

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 stage groupings

A Wilms tumor is staged based on the results of surgery, analysis of the tumor cells (see Diagnosis), and whether the cancer has spread.

Stage I: The tumor is found in 1 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 where 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 to 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 2 most important factors that predict the prognosis of a child with a Wilms tumor are:

  • The histology of the tumor. This is what the tumor cells look like under a microscope. 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. About 10% of Wilms tumors have an anaplastic histology. Tumors in people who are 12 to 16 years old are more likely to be anaplastic.

  • 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. The stages are the same for tumors, regardless of histology.

Information about the cancer’s stage will help the doctor recommend a specific treatment plan. The next section in this guide is Types of Treatment. Use the menu to choose a different section to read in this guide.

Wilms Tumor - Childhood - Types of Treatment

Approved by the Cancer.Net Editorial Board, 02/2018

ON THIS PAGE: You will learn about the different types of treatments doctors use to treat children with a Wilms tumor. Use the menu to see other pages.

In general, cancer in children is uncommon. This means it can be hard for doctors to plan treatments unless they know what has been most effective in other children. That is 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 the standard of care with newer approaches to treatments that may be more effective. The “standard of care” is the best treatments known. Clinical trials may test such approaches as a new drug, a new combination of existing treatments, or new doses of current therapies. The health and safety of all children who participate in clinical trials are closely monitored.

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.

Treatment overview

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 common types of treatments used for a Wilms tumor are listed below. Your child's care plan also includes treatment for symptoms and side effects, an important part of cancer care.

The main treatments for a Wilms tumor are surgery, chemotherapy, and radiation therapy. Treatment options and recommendations depend on several factors, including:

  • The type, stage, and histology of the tumor

  • Possible side effects

  • The child and family’s preferences

  • The child’s overall health

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 a Wilms tumor. It will usually be performed by either a pediatric surgeon with experience in 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 a 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 1 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 may also be used if preserving 1 functioning kidney is not possible. Dialysis is when a machine does the filtering work of the kidneys.

Before surgery, talk with your health care team about the possible side effects from the specific surgery you will have. Learn more about the basics of surgery.

Chemotherapy

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

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

A chemotherapy regimen, or schedule, usually consists of a specific number of cycles given over a set period of time. A person may receive 1 drug at a time or combinations of different drugs given at the same time. A Wilms tumor with a favorable histology is often treated with dactinomycin (Cosmegen), doxorubicin (Adriamycin), and/or vincristine (Vincasar PFS, Oncovin). Other drugs used to treat Wilms tumors include cyclophosphamide (Cytoxan, Neosar), etoposide (Toposar, VePesid), and irinotecan (Camptosar).

Chemotherapy may be given after surgery to eliminate any remaining cancer cells. When this is done, it is 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 after 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 a tumor 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, or 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 a stage III or IV Wilms tumor with a favorable histology and for all children who have a tumor with an anaplastic histology (see Stages for more information).

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

Care for symptoms and side effects

Cancer and its treatment often cause side effects. In addition to treatments intended 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 or supportive 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 people with cancer 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 treatment process.

Children often receive treatment for the tumor at the same time that they receive treatment to ease side effects at the same time. In fact, people who receive both at the same time 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 tumor, such as chemotherapy, surgery, or radiation therapy. Talk with your child's doctor about the goals of each treatment in his or her treatment plan.

Before treatment begins, talk with your child’s health care team about the possible side effects of the specific treatment plan and palliative care options. 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 is 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 Wilms 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 new cycle of testing will begin again to learn as much as possible about the recurrence. After this testing is done, your child’s doctor will talk with you about the treatment options. The treatment plan for recurrent Wilms tumor depends on 4 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 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 Wilms 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 does not work

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 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 people with cancer and their families.

Hospice care is designed to provide the best possible quality of life for people who are expected to live less than 6 months. Parents and guardians are encouraged to talk with the health care team about hospice options, which include hospice care at home, a special hospice center, or other health care locations. Nursing care and special equipment can make staying at home a workable option 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. It offers more information about research studies that are focused on finding better ways to care for children with a Wilms tumor. Use the menu to choose a different section to read in this guide.

Wilms Tumor - Childhood - About Clinical Trials

Approved by the Cancer.Net Editorial Board, 02/2018

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. Use the menu to see other pages.

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. There has been much progress made in treating this type of cancer in the past 2 decades. In fact, Wilms tumor is one of the best examples of the impact that clinical trials have on finding the best treatment approaches to improve outcomes and avoid side effects.

Many clinical trials focus on new treatments. Researchers want to learn if a new treatment is safe, effective, and possibly better than the treatment doctors use now. 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 can be some of the first to get a treatment before it is available to the public. However, there are some risks with a clinical trial, including possible side effects and that the new treatment may not work. People are encouraged to talk with their health care team about the pros and cons of joining a specific study.

Some clinical trials study new ways to relieve symptoms and side effects during treatment. Others study ways to manage the late effects that may happen a long time after treatment. Talk with your doctor about clinical trials for symptoms and side effects. There are also clinical trials studying ways to prevent cancer.

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 a way to contribute to the progress in treating Wilms tumors. Even if your child does not benefit directly from the clinical trial, his or her participation may benefit future children with a Wilms tumor.

Insurance coverage of clinical trials costs differs by location and by study. In some programs, some of the people's expenses from participating in the clinical trial are reimbursed. In others, they are not. It is important to talk with the research team and your insurance company first to learn if and how your child's treatment in a clinical trial will be covered. Learn more about health insurance coverage of clinical trials.

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:

  • Describe all of the treatment options so the parents and children understand how the new treatment differs from the standard treatment.

  • List all of the risks of the new treatment, which may or may not be different from the risks of standard treatment.

  • 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.

Clinical trials also have certain rules called “eligibility criteria” that help structure the research and keep people safe. You and the research team will carefully review these criteria together.

People 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 people participating in a clinical trial talk with their child’s doctor and researchers about who will be providing treatment and care during the clinical trial, after the clinical trial ends, and/or if they choose 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.

PRE-ACT, Preparatory Education About Clinical Trials

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. It explains areas of scientific research currently going on for Wilms tumor. Use the menu to choose a different section to read in this guide.

Wilms Tumor - Childhood - Latest Research

Approved by the Cancer.Net Editorial Board, 02/2018

ON THIS PAGE: You will read about the scientific research being done now to learn more about Wilms tumor and how to treat it. Use the menu to see other pages.

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 people through clinical trials. Always talk with your child’s doctor about the best diagnostic and treatment options for your child.

  • Bilateral Wilms tumors. The Children’s Oncology Group recently studied 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 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 about one-third of children with a 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.

  • New treatment approaches for tumors that have spread to the lungs. Researchers are trying to figure out if a Wilms tumor with a favorable histology that has spread to the lungs should be treated with radiation therapy if the lung tumors go away after 6 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 6 weeks.

  • New treatment methods. Doctors are looking at the effect of using only surgery as a treatment for people with a small, stage I Wilms tumor. After surgery, they 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 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 tumors, explore these related items that will take you outside of this guide:

The next section in this guide is Coping with Treatment. It offers some guidance on how to cope with the physical, emotional, and social changes that cancer and its treatment can bring. Use the menu to choose a different section to read in this guide.

Wilms Tumor - Childhood - Coping with Treatment

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ON THIS PAGE: You will learn more about coping with the physical, emotional, social, and financial effects of childhood cancer and its treatment. This page includes several links outside of this guide to other sections of this website. Use the menu to see other pages.

Every cancer treatment can cause side effects or changes to your child’s body and how he or she feels. For many reasons, people do not experience the same side effects even when they are given the same treatment for the same type of tumor. This can make it hard to predict how your child will feel during treatment.

As your family prepares to start treatment, it is normal to fear treatment-related side effects. It may help to know that your child's health care team will work to prevent and relieve side effects. Doctors call this part of treatment “palliative care” or "supportive care." It is an important part of your child’s treatment plan, regardless of his or her age or the stage of disease.

Coping with physical side effects

Common physical side effects from each treatment option for a Wilms tumor are described in the Types of Treatment section. Learn more about side effects of cancer and its treatment, along with ways to prevent or control them. Changes to your child’s physical health depend on several factors, including the tumor’s stage, the length and dose of treatment, and your child’s general health.

Sometimes, physical side effects can last after treatment ends. Doctors call these long-term side effects. They call side effects that occur months or years after treatment late effects. Treating long-term side effects and late effects is an important part of care for childhood cancer survivors. 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.

Coping with emotional and social effects

Your family can have emotional and social effects as well as physical effects after a cancer diagnosis. This may include dealing with difficult emotions, such as sadness, anxiety, or anger, or managing stress. Sometimes, patients and their families have problems expressing how they feel to their loved ones, or people do not know what to say in response.

People with cancer and their families are encouraged to share their feelings with a member of their health care team. You can also find coping strategies for emotional and social effects in a separate section of this website. This section includes many resources for finding support and information to meet your family’s needs.

Coping with financial effects

Cancer treatment can be expensive. It is often a big source of stress and anxiety for families dealing with a cancer diagnosis. In addition to treatment costs, many people find they have extra, unplanned expenses related to their child’s care. Families are encouraged to talk about financial concerns with a member of their health care team. Learn more about managing financial considerations in a separate part of this website.

Caring for a child with cancer

Family members and friends often play an important role in taking care of a child with a Wilms tumor. This is called being a caregiver. As a parent or guardian, you are the primary caregiver for your child. However, friends and family members can give your family valuable support, even if they live far away.

When your child has cancer, you may have an additional range of responsibilities. These may include giving medications or managing symptoms and side effects. However, it is important to seek help from others. Below are some of the responsibilities your family or friends could help with:

  • Providing short-term care for your child

  • Giving support and encouragement

  • Assisting with meals or household chores

  • Helping with insurance and billing issues

Learn more about caregiving.

Talking with your child's health care team about side effects

Before starting treatment, talk with your child’s doctor about possible side effects. Ask:

  • Which side effects are most likely?

  • When are they are likely to happen?

  • What can we do to prevent or relieve them?

Be sure to tell your child's health care team about any side effects that happen during treatment and afterward, too. Tell them even if you don’t think the side effects are serious. This discussion should include physical, emotional, and social effects of cancer.

Also, ask how much care your child may need at home and with daily tasks during and after treatment. This can help you make a caregiving plan.

The next section in this guide is Late Effects of Treatment. It describes some of the side effects that can occur long after treatment has finished. Use the menu to choose a different section to read in this guide.

Wilms Tumor - Childhood - Late Effects of Treatment

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ON THIS PAGE: You will find out more about side effects that can occur long after treatment for a Wilms tumor has finished. Use the menu to see other pages.

Although many children with a Wilms tumor are treated successfully, they often have an increased risk of developing other diseases or conditions later in life, because 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 1 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 may develop scar tissue that puts them at risk for developing a bowel obstruction throughout their lives. 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 whose cancer had spread to their lungs and received radiation therapy to that location 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. Scoliosis, or curvature of the spine, and underdevelopment of nearby soft tissue may occur in children who received radiation therapy. 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 for a secondary cancer 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 closely monitored 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. Birth defects are also more common.

The next section in this guide is Follow-up Care. It explains the importance of checkups after treatment has finished. Use the menu to choose a different section to read in this guide.

Wilms Tumor - Childhood - Follow-Up Care

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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. Use the menu to see other pages.

Care for children diagnosed with cancer doesn’t end when active treatment has finished. Your child’s health care team will continue to check to make sure the Wilms 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.

Your child’s follow-up care may include regular physical examinations, medical tests, or both. Doctors want to keep track of your child’s recovery in the months and years ahead. 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 frequent, such as every 3 months. The time between checkups will increase until 5 years after treatment, when your child will be scheduled for a follow-up visit once a year.

Learn more about the importance of follow-up care.

Watching for recurrence

One goal of follow-up care is to check for a recurrence. Cancer 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. A recurrence of Wilms tumor is unlikely, but if it does happen, it is generally within the first 2 years after treatment ends.

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 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.

The anticipation before having a follow-up test or waiting for test results can add stress to you or a family member. This is sometimes called “scan-xiety.” Learn more about how to cope with this type of stress.

Managing long-term and late side effects of childhood cancer

The more time that has passed 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, people will have blood tests to make sure no changes have occurred in their kidney function. This is especially important for people who have 1 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.

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 when treatment is completed.

Some children continue to see their oncologist, while others transition back to the care of their family doctor or another health care professional. This decision depends on several factors, including:

  • The type and stage of tumor

  • Side effects

  • Health insurance rules

  • Your family’s personal preferences

Talk with the 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, and with 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. It describes how to cope with challenges in everyday life after a cancer diagnosis. Use the menu to choose a different section to read in this guide.

Wilms Tumor - Childhood - Survivorship

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ON THIS PAGE: You will read about how to cope with challenges in everyday life after your child’s cancer diagnosis. Use the menu to see other pages.

What is survivorship?

The word “survivorship” means different things to different people, but it often describes 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 person 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 about coping with everyday life.

One source of stress may occur when their frequent visits to the health care team end after completing 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 when 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

  • 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 place where your child received treatment.

Changing role of caregivers

Parents, other family members, and friends may also go through periods of transition. 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.

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.

It is important that your child has recommended medical checkups 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 the 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 that 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: Get this 44-page booklet that helps people transition into life after treatment. It includes blank treatment summary and survivorship care plan forms. The free booklet is also available as a PDF, so it is easy to print out.

  • 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 Health Care Team to help start conversations with your child’s cancer care team. Use the menu to choose a different section to read in this guide.

Wilms Tumor - Childhood - Questions to Ask the Health Care Team

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

Talking often with the health care team 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 a digital 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 available for 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 leading 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 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 of medical care, who can help me?

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

  • Whom should I call with questions or problems?

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?

  • Will my child have pain or be uncomfortable while receiving this 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 chance that the tumor will come back? Should I watch for specific signs or symptoms?

  • What long-term side effects or late effects are possible based on the 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 leading my child’s follow-up care?

  • How long will my child need follow-up care?

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

The next section in this guide is Additional Resources. It offers some more resources on this website beyond this guide that may be helpful to you. Use the menu to choose a different section to read in this guide.

Wilms Tumor - Childhood - Additional Resources

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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. Use the menu to go back and see other pages.

Cancer.Net includes many other sections about the medical and emotional aspects of cancer 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.

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 to select another section, to continue reading this guide.