Diseases And Treatments

Wilms' Tumor - What It Is, Symptoms and Treatments

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Wilms' Tumor - What It Is, Symptoms and Treatmentswhich many are unaware of. In addition, theWilms tumoris a rare kidney cancer that mainly affects children. Also known as nephroblastoma, it is the most common kidney cancer in children. OWilms tumorusually affects children 3 to 4 years old and becomes much less common after 5 years of age. OWilms tumorusually occurs in only one kidney, although it can sometimes be found in both kidneys at the same time. Over the years, advances in the diagnosis and treatment ofWilms tumorhave greatly improved the outlook (prognosis) for children with this disease. With appropriate treatment, the outlook for the majority of children withWilms tumorit is very good.Causes of Wilms Tumor:It is not clear what causes theWilms tumor, but in rare cases, heredity may play a role. Cancer begins when cells develop errors in their DNA. Errors allow cells to grow and divide uncontrollably and continue to live when other cells would die. The accumulating cells form a tumor. At the

Wilms tumor, this process occurs in the kidney cells. In rare cases, DNA errors that lead toWilms tumorare passed from one parent to the child. In most cases, there is no known connection between parents and children that can lead to cancer.

Symptoms of Wilms' Tumor:Signs and symptoms ofWilms tumorvary widely, and some children show no obvious signs. But the majority ofWilms tumoryou experience one or more of these signs and symptoms:

  • An abdominal mass that you can feel
  • Abdominal bloating
  • Abdominal pain

Other signs and symptoms may include:

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  • Fever
  • Blood in the urine
  • Nausea or vomiting or both
  • Constipation
  • loss of appetite
  • Shortness of breath
  • High pressure

When to Contact a Physician:Make an appointment with your child's doctor if you notice any signs or symptoms that worry you. OWilms tumoris rare, so it is much more likely that something else is causing symptoms, but it is important to check for any concerns.

Wilms Tumor Risk Factors:Factors that may increase the risk ofWilms tumorinclude:

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  • African-American race.In Brazil, African-American children have a slightly higher risk of developingWilms tumorthan children of other races. Asian-American children appear to be at lower risk than children of other races.
  • Family history of Wilms' Tumor.If someone in your child's family has hadWilms tumor, your child has an increased risk of developing the disease.

OWilms tumoroccurs more frequently in children with certain abnormalities or syndromes present at birth, including:

  • Aniridia.In aniridia (an-ih-RID-e-uh), the iris - the colored portion of the eye - forms only partially or not.
  • Hemihyperplasia.Hemihyperplasia (hem-e-hi-PUR-truh-fee) means that one side of the body or one part of the body is noticeably larger than the other side.

OWilms tumormay occur as part of rare syndromes, including:

  • WAGR syndrome. This syndrome includesWilms tumor, aniridia, abnormalities of the urinary and genital system, and intellectual disabilities (previously called mental retardation).
  • Denys-Drash Syndrome. This syndrome includesWilms tumor, renal disease and male pseudohermaphroditism (soo-do-her-MAF-roe-dit-iz-um), in which a boy is born with testicles but may exhibit female characteristics.
  • Beckwith-Wiedemann syndrome. Children with this syndrome tend to be significantly larger than the mean (macrosomia). Other signs may include abdominal organs that enter the base of the umbilical cord, a large tongue (macroglossia), enlarged internal organs and abnormalities of the ear. There is also an increased risk of tumors, including a type of liver cancer called hepatoblastoma.

Wilms Tumor Diagnosis:To diagnoseWilms tumor, your child's doctor may recommend:

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  • A physical examination.The doctor will look for possible signs ofWilms tumor.
  • Blood and urine tests.These laboratory tests can not detect theWilms tumor, but they can indicate how well the kidneys are working and discover certain kidney problems or low blood counts.
  • Image tests.Tests that create pictures of the kidneys help the doctor determine if your child has a kidney tumor. Imaging tests may include an ultrasound, computed tomography (CT) scan or magnetic resonance imaging (MRI).

Staging:Once your child's doctor diagnoses theWilms tumor, he or she works to determine the extent (stage) of the cancer. The doctor may recommend a chest x-ray or chest computed tomography and bone scan to determine if the cancer has spread beyond the kidneys.

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The doctor assigns a stage to the cancer, which helps determine the treatment options. In Brazil, the guidelines developed through the National Wilms Tumor study of the Children's Oncology Group include these five steps:

  • Stage I. The cancer is found only in one kidney, is completely contained in the kidney and can be completely removed with surgery.
  • Stage II.The cancer has spread to the tissues and structures beyond the affected kidney, such as the nearby fat or blood vessels, but can still be completely removed by surgery.
  • Phase III.The cancer has spread beyond the kidney area to nearby lymph nodes or other structures within the abdomen, the tumor may spill into the abdomen before or during surgery, or may not be completely removed by surgery.
  • Stage IV.The cancer has spread out of the kidney to distant structures, such as the lungs, liver, bones or brain.
  • Phase V.Cancer cells are found in both kidneys (bilateral tumors).

Wilms Tumor Treatments:The treatment forWilms tumorusually involves surgery and chemotherapy, and sometimes radiation therapy. Treatments may vary according to the stage of cancer. Because this type of cancer is rare, your child's doctor may recommend that you seek treatment at a child cancer center that has experience in treating this type of cancer.

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Surgery To Remove All Or Part Of Kidney:The treatment forWilms tumormay begin with surgery to remove all or part of a kidney (nephrectomy). The surgery is also used to confirm the diagnosis - tissue removed during surgery is sent to a laboratory to determine if it is cancerous and what type of cancer is in the tumor. Surgery for theWilms tumormay include:

  • Removing Part of Affected Kidney.Partial nephrectomy involves removal of the tumor and a small part of the surrounding renal tissue. Partial nephrectomy may be an option if the cancer is very small or if your child has only one kidney functioning.
  • Removing affected kidney and surrounding tissue.In a radical nephrectomy, doctors remove the surrounding kidney and tissues, including part of the ureter and sometimes the adrenal gland. The nearby lymph nodes are also removed. The remaining kidney can increase its capacity and take control of blood filtration.
  • Removing the Whole or Part of the Two Kidneys.If the cancer affects both kidneys, the surgeon removes as much cancer as possible from both kidneys. In a small number of cases, this could mean the removal of both kidneys, and your child would then need renal dialysis. If a kidney transplant is an option, your child would not need dialysis any more.

Chemotherapy:Chemotherapy uses powerful drugs to kill cancer cells throughout the body. The treatment forWilms tumorusually involves a combination of drugs, administered through a vein, that work together to kill cancer cells. What side effects your child may experience will depend on which medications are used.

Common side effects include nausea, vomiting, loss of appetite, hair loss and increased risk of infection. Ask your doctor what side effects may occur during treatment, and if there are any potential long-term complications. Chemotherapy can be used before surgery to shrink tumors and make them easier to remove.

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It can be used after surgery to kill any cancer cells that may remain in the body. Chemotherapy may also be an option for children whose cancers are too advanced to be completely removed by surgery. For children who have cancer in both kidneys, chemotherapy is given before surgery. This may make it more likely that surgeons can save at least one kidney to preserve kidney function.

Radiation Therapy:Depending on the stage of theWilms tumor, radiation therapy may be recommended. Radiation therapy uses high energy beams to kill cancer cells. During radiation therapy, your child is carefully positioned at a table and a large machine moves around your child, targeting precisely bundles of energy in the cancer. Possible side effects include nausea, diarrhea, tiredness and skin irritation like sunburn.

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Radiation therapy can be used after surgery to kill any cancer cells that were not removed during the operation. It may also be an option to control the cancer that has spread to other areas of the body, depending on where the cancer has spread.

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Clinical Trials:Your child's doctor may recommend taking part in a clinical trial. These studies allow your child to have a chance in the latest cancer treatments, but they can not guarantee a cure. Discuss the benefits and risks of clinical trials with your child's physician. Most children with cancer enroll in a clinical trial, if available. However, enrollment in a clinical trial is up to you and your child.

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