Wilms Tumour: Your Way Forward

In nephroblastomatosis diffuse hyperplastic perilobar nephroblastomatosis , these abnormal groups of cells may grow in many places inside the kidney or make a thick layer around the kidney. When these groups of abnormal cells are found in a kidney after it was removed for Wilms tumor, the child has an increased risk of Wilms tumor in the other kidney. Frequent follow-up testing is important at least every 3 months, for at least 7 years after the child is treated.

Anything that increases the risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk to your child's doctor if you think your child may be at risk.

Wilms tumor may be part of a genetic syndrome that affects growth or development. A genetic syndrome is a set of signs and symptoms or conditions that occur together and is caused by certain changes in the genes. Certain conditions can also increase a child's risk of developing Wilms tumor. These and other genetic syndromes and conditions have been linked to Wilms tumor:. Screening tests are done in children with an increased risk of Wilms tumor. These tests may help find cancer early and decrease the chance of dying from cancer.

In general, children with an increased risk of Wilms tumor should be screened for Wilms tumor every three months until they are at least 8 years old. An ultrasound test of the abdomen is usually used for screening. Small Wilms tumors may be found and removed before symptoms occur. Children with Beckwith-Wiedemann syndrome or hemihyperplasia are also screened for liver and adrenal tumors that are linked to these genetic syndromes. A test to check the alpha-fetoprotein AFP level in the blood and an ultrasound of the abdomen are done until the child is 4 years old.

An ultrasound of the kidneys is done between the ages of 4 and 7 years old. A physical exam by a specialist geneticist or pediatric oncologist is done two times each year. In children with certain gene changes, a different schedule for ultrasound of the abdomen may be used.

Children with aniridia and a certain gene change are screened for Wilms tumor every three months until they are 8 years old. An ultrasound test of the abdomen is used for screening. Some children develop Wilms tumor in both kidneys. These often appear when Wilms tumor is first diagnosed, but Wilms tumor may also occur in the second kidney after the child is successfully treated for Wilms tumor in one kidney.

Children with an increased risk of a second Wilms tumor in the other kidney should be screened for Wilms tumor every three months for up to eight years. An ultrasound test of the abdomen may be used for screening. Prior chemotherapy or radiation therapy for a childhood cancer , such as neuroblastoma , soft tissue sarcoma, leukemia , or Wilms tumor may also increase the risk of renal cell cancer.

Genetic counseling a discussion with a trained professional about genetic diseases and whether genetic testing is needed may be needed if the child has one of the following syndromes or conditions:. Sometimes childhood kidney tumors do not cause signs and symptoms and the parent finds a mass in the abdomen by chance or the mass is found during a well-child health check up.

These and other signs and symptoms may be caused by kidney tumors or by other conditions. Check with your child's doctor if your child has any of the following:.

General Information About Wilms Tumor and Other Childhood Kidney Tumors

Wilms tumor that has spread to the lungs or liver may cause the following signs and symptoms:. The number of red blood cells , white blood cells , and platelets. The amount of hemoglobin the protein that carries oxygen in the red blood cells. An ultrasound transducer connected to a computer is pressed against the skin of the abdomen.

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The transducer bounces sound waves off internal organs and tissues to make echoes that form a sonogram computer picture. Computed tomography CT scan of the abdomen. The child lies on a table that slides through the CT scanner, which takes x-ray pictures of the inside of the abdomen. Magnetic resonance imaging MRI of the abdomen. The child lies on a table that slides into the MRI scanner, which takes pictures of the inside of the body.

The stage of the cancer.

Whether the tumor can be removed by surgery. Whether the patient is in a clinical trial. The prognosis chance of recovery and treatment options for Wilms tumor depend on the following:. The prognosis for clear cell sarcoma of the kidney depends on the following:. The process used to find out if cancer has spread outside of the kidney to other parts of the body is called staging.

The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The doctor will use results of the diagnostic and staging tests to help find out the stage of the disease. The following tests may be done to see if cancer has spread to other places in the body:.

Wilms’ tumour and the WT1 protein

A small amount of radioactive material is injected into the child's vein and travels through the blood. The radioactive material collects in the bones. As the child lies on a table that slides under the scanner, the radioactive material is detected and images are made on a computer screen. Cancer can spread through tissue , the lymph system , and the blood:. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began the primary tumor and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor.

For example, if Wilms tumor spreads to the lung , the cancer cells in the lung are actually Wilms tumor cells. The disease is metastatic Wilms tumor, not lung cancer. Many cancer deaths are caused when cancer moves from the original tumor and spreads to other tissues and organs.

This is called metastatic cancer. This animation shows how cancer cells travel from the place in the body where they first formed to other parts of the body. The histology how the cells look under a microscope of the tumor affects the prognosis and the treatment of Wilms tumor. The histology may be favorable or anaplastic unfavorable. Tumors with a favorable histology have a better prognosis and respond better to chemotherapy than anaplastic tumors. Tumor cells that are anaplastic divide quickly and under a microscope do not look like the type of cells they came from.

Anaplastic tumors are harder to treat with chemotherapy than other Wilms tumors at the same stage. In stage I , the tumor was completely removed by surgery and all of the following are true:. In stage II , the tumor was completely removed by surgery and no cancer cells were found at the edges of the area where the cancer was removed. Cancer has not spread to lymph nodes. Before the tumor was removed, one of the following was true:. In stage III , cancer remains in the abdomen after surgery and one of the following may be true:. In stage IV , cancer has spread through the blood to organs such as the lungs , liver , bone, or brain, or to lymph nodes outside of the abdomen and pelvis.

In stage V , cancer cells are found in both kidneys when the cancer is first diagnosed. Recurrent cancer is cancer that has recurred come back after it has been treated. Different types of treatment are available for children with Wilms and other childhood kidney tumors. Some treatments are standard the currently used treatment , and some are being tested in clinical trials.

A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. Your child's treatment will be overseen by a pediatric oncologist , a doctor who specializes in treating children with cancer.

The pediatric oncologist works with other pediatric health care providers who are experts in treating children with Wilms tumor or other childhood kidney tumors and who specialize in certain areas of medicine. These may include the following specialists:. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following:.

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Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. Clinical trials are being done to find out if lower doses of chemotherapy and radiation can be used to lessen the late effects of treatment without changing how well the treatment works. Two types of surgery are used to treat kidney tumors:.

After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left.

Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Sometimes, a second-look surgery is done to see if cancer remains after chemotherapy or radiation therapy. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy:. The way the radiation therapy is given depends on the type and stage of the cancer being treated and whether a biopsy was done before surgery to remove the tumor.

External radiation therapy is used to treat Wilms tumor and other childhood kidney tumors. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body systemic chemotherapy. When chemotherapy is placed directly into the cerebrospinal fluid , an organ , or a body cavity such as the abdomen , the drugs mainly affect cancer cells in those areas regional chemotherapy.

Combination chemotherapy is treatment using two or more anticancer drugs. The way the chemotherapy is given depends on the type and stage of the cancer being treated. Systemic chemotherapy is used to treat Wilms tumor and other childhood kidney tumors. Sometimes the tumor cannot be removed by surgery for one of the following reasons:.

In this case, a biopsy is done first. Then chemotherapy is given to reduce the size of the tumor before surgery, in order to save as much healthy tissue as possible and lessen problems after surgery. This is called neoadjuvant chemotherapy. Radiation therapy is given after surgery. Immunotherapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or biologic therapy.

Interferon and interleukin-2 IL-2 are types of immunotherapy used to treat childhood renal cell cancer. Interferon affects the division of cancer cells and can slow tumor growth. IL-2 boosts the growth and activity of many immune cells, especially lymphocytes a type of white blood cell. Lymphocytes can attack and kill cancer cells. High-dose chemotherapy with stem cell rescue is a method of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells immature blood cells are removed from the blood or bone marrow of the patient or a donor and are frozen and stored.

After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These re-infused stem cells grow into and restore the body's blood cells. High-dose chemotherapy with stem cell rescue may be used to treat recurrent Wilms tumor.

Information about clinical trials is available from the NCI website. Targeted therapy is a treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Targeted therapy used to treat childhood kidney tumors may include the following:. Targeted therapy is being studied for the treatment of childhood kidney tumors that have recurred come back. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process.

Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring coming back or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Clinical trials supported by other organizations can be found on the ClinicalTrials.

Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred come back.

These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of stage I Wilms tumor with favorable histology may include:. Treatment of stage I anaplastic Wilms tumor may include:. Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Treatment of stage II Wilms tumor with favorable histology may include:.

PDQ Cancer Information Summaries [Internet].

Treatment of stage II anaplastic Wilms tumor may include:. Treatment of stage III Wilms tumor with favorable histology may include:. Treatment of stage III anaplastic Wilms tumor may include:. Treatment of stage IV Wilms tumor with favorable histology may include:. Treatment of stage IV anaplastic Wilms tumor may include:. Treatment of stage V Wilms tumor may be different for each patient and may include:.

If a kidney transplant is needed because of kidney problems, it is delayed until 1 to 2 years after treatment is completed and there are no signs of cancer. Treatment of renal cell cancer may include:. There is no standard treatment for rhabdoid tumor of the kidney. Treatment of clear cell sarcoma of the kidney may include:.

Treatment for certain patients with stage III congenital mesoblastic nephroma may include:. There is no standard treatment for Ewing sarcoma of the kidney. It may also be treated in the same way that Ewing sarcoma is treated. There is no standard treatment for primary renal myoepithelial carcinoma. Treatment of cystic partially differentiated nephroblastoma may include:. Treatment of multilocular cystic nephroma usually includes:. Treatment of primary renal synovial sarcoma usually includes:.

There is no standard treatment for anaplastic sarcoma of the kidney. Treatment is usually the same treatment given for anaplastic Wilms tumor. Treatment of recurrent Wilms tumor may include:. Treatment of recurrent rhabdoid tumor of the kidney may include:. Treatment of recurrent clear cell sarcoma of the kidney may include:.

Treatment of recurrent congenital mesoblastic nephroma may include:. Treatment of other recurrent childhood kidney tumors is usually within a clinical trial. For more information from the National Cancer Institute about Wilms tumor and other childhood kidney tumors, see the following:. For more childhood cancer information and other general cancer resources, see the following:. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine.

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The PDQ summaries are based on an independent review of the medical literature. The date on each summary "Updated" is the date of the most recent change. A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works.

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More information about contacting us or receiving help with the Cancer. Questions can also be submitted to Cancer. Turn recording back on. National Center for Biotechnology Information , U. Show details Bethesda MD: Nephroblastomatosis is not cancer but may become Wilms tumor. Having certain genetic syndromes or other conditions can increase the risk of Wilms tumor. Having certain conditions may increase the risk of renal cell cancer. Treatment for Wilms tumor and other childhood kidney tumors may include genetic counseling. Signs of Wilms tumor and other childhood kidney tumors include a lump in the abdomen and blood in the urine.

Tests that examine the kidney and the blood are used to detect find and diagnose Wilms tumor and other childhood kidney tumors. Certain factors affect prognosis chance of recovery and treatment options. Childhood kidney tumors are diseases in which malignant cancer cells form in the tissues of the kidney. There are many types of childhood kidney tumors. Wilms Tumor In Wilms tumor , one or more tumors may be found in one or both kidneys.

Rhabdoid Tumor of the Kidney Rhabdoid tumor of the kidney is a type of kidney cancer that occurs mostly in infants and young children. Children with a certain change in the SMARCB1 gene are checked regularly to see if a rhabdoid tumor has formed in the kidney or has spread to the brain: Children younger than one year old have an ultrasound of the abdomen every two to three months and an ultrasound of the head every month.

Children one to four years old have an ultrasound of the abdomen and an MRI of the brain and spine every three months. Clear Cell Sarcoma of the Kidney Clear cell sarcoma of the kidney is a type of kidney tumor that may spread to the lung, bone, brain, or soft tissue. Congenital Mesoblastic Nephroma Congenital mesoblastic nephroma is a tumor of the kidney that is often diagnosed during the first year of life. Ewing Sarcoma of the Kidney Ewing sarcoma previously called neuroepithelial tumor of the kidney is rare and usually occurs in young adults. Primary Renal Myoepithelial Carcinoma Primary renal myoepithelial carcinoma is a rare type of cancer that usually affects soft tissues, but sometimes forms in the internal organs such as the kidney.

Cystic Partially Differentiated Nephroblastoma Cystic partially differentiated nephroblastoma is a very rare type of Wilms tumor made up of cysts. Multilocular Cystic Nephroma Multilocular cystic nephromas are benign tumors made up of cysts and are most common in infants, young children, and adult women. Primary Renal Synovial Sarcoma Primary renal synovial sarcoma is a cyst-like tumor of the kidney and is most common in young adults. Anaplastic Sarcoma of the Kidney Anaplastic sarcoma of the kidney is a rare tumor that is most common in children or adolescents younger than 15 years of age.

These and other genetic syndromes and conditions have been linked to Wilms tumor: WAGR syndrome Wilms tumor, aniridia , abnormal genitourinary system , and mental retardation. Denys-Drash syndrome abnormal genitourinary system. Beckwith-Wiedemann syndrome abnormally large growth of one side of the body or a body part, large tongue, umbilical hernia at birth, and abnormal genitourinary system. A family history of Wilms tumor. Aniridia the iris , the colored part of the eye, is missing. Urinary tract problems such as cryptorchidism or hypospadias.

Tests are used to screen for Wilms tumor. Renal cell cancer may be related to the following conditions: Von Hippel-Lindau disease an inherited condition that causes abnormal growth of blood vessels. Children with Von Hippel-Lindau disease should be checked yearly for renal cell cancer with an ultrasound of the abdomen or an MRI magnetic resonance imaging beginning at age 8 to 11 years.

Tuberous sclerosis an inherited disease marked by noncancerous fatty cysts in the kidney. Familial renal cell cancer an inherited condition that occurs when certain changes in the genes that cause kidney cancer are passed down from the parent to the child. Hereditary leiomyomatosis an inherited disorder that increases the risk of having cancer of the kidney, skin, and uterus.

Genetic counseling a discussion with a trained professional about genetic diseases and whether genetic testing is needed may be needed if the child has one of the following syndromes or conditions: A genetic syndrome or condition that increases the risk of Wilms tumor. An inherited condition that increases the risk of renal cell cancer. But sometimes cancer is caused by mutations that we are born with.

Around 1, children are diagnosed with cancer each year in the UK, and most of these cases are due to mutations present at birth. A cancer diagnosis in someone so young can be devastating for their families and loved ones, but the good news is that, thanks to research, around three-quarters of these children now survive.

Worldwide Cancer Research has funded research into a wide range of childhood cancers. It is thought to start from immature kidney cells in the developing embryo in the womb. In most cases, the cause is unknown but a small number are due to genetic factors including faulty genes like WT1 Wilms Tumour 1. The WT1 gene acts as a tumour suppressor, which means that when it is working properly, it stops tumours from forming. After these findings, Professor Roberts obtained grants from several other funding bodies, and he continues to study WT1 and BASP1, more than ten years later.

They found that the WT1 protein sticks to something called alpha-actinin. This is particularly relevant to cancer, because cancer cells often have an abnormal shape and structure, so changes that affect WT1 protein or alpha-actinin might play a role in cancer-causing changes to our cells.

With our funding, Dr Aswin Menke was studying the role of the WT1 gene in leukaemia, by investigating its role in normal blood development and in leukaemia.