- By sahlhealth
- May 18, 2021
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Wilms‘ Tumor is a rare kidney cancer that primarily affects children. Also known as nephroblastoma, it’s the most common cancer of the kidneys in children. Wilms’ tumor most often affects children ages 3 to 4 and becomes much less common after age 5. Wilms’ tumor most often occurs in just one kidney, though it can sometimes be found in both kidneys at the same time. Over the years, advancements in the diagnosis and treatment of Wilms’ tumor have greatly improved the outlook (prognosis) for children with this disease. With appropriate treatment, the outlook for most children with Wilms’ tumor is very good.
Signs and symptoms
-Signs and symptoms of Wilms' tumor vary widely, and some children don't show any obvious signs. But most children with Wilms' tumor experience one or more of these signs and symptoms:
- An abdominal mass you can feel
- Abdominal swelling
- Abdominal pain
- Blood in the urine
- Nausea or vomiting or both
- Loss of appetite
- Shortness of breath
- High blood pressure
-Treatment for Wilms' tumor usually involves surgery and chemotherapy, and sometimes radiation therapy. Treatments may vary by the stage of the cancer. Because this type of cancer is rare, your child's doctor may recommend that you seek treatment at a children's cancer center that has experience treating this type of cancer.Surgery to remove all or part of a kidney.Treatment for Wilms' tumor may begin with surgery to remove all or part of a kidney (nephrectomy). Surgery is also used to confirm the diagnosis â€” the tissue removed during surgery is sent to a lab to determine whether it's cancerous and what type of cancer is in the tumor.
Surgery for Wilms' tumor may include:
- Removing part of the affected kidney. Partial nephrectomy involves removal of the tumor and a small part of the kidney tissue surrounding it. Partial nephrectomy may be an option if the cancer is very small or if your child has only one functioning kidney.
- Removing the affected kidney and surrounding tissue. In a radical nephrectomy, doctors remove the kidney and surrounding tissues, including part of the ureter and sometimes the adrenal gland. Nearby lymph nodes also are removed. The remaining kidney can increase its capacity and take over the job of filtering the blood.
- Removing all or part of both 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 may mean removing both kidneys, and your child would then need kidney dialysis. If a kidney transplant is an option, your child would no longer need dialysis.
-It's not clear what causes Wilms' tumor, but in rare cases, heredity may play a role.Cancer begins when cells develop errors in their DNA. The errors allow the cells to grow and divide uncontrollably and to go on living when other cells would die. The accumulating cells form a tumor. In Wilms' tumor, this process occurs in the kidney cells.In rare cases, the errors in DNA that lead to Wilms' tumor are passed from a parent to the child. In most cases, there is no known connection between parents and children that may lead to cancer.
-Factors that may increase the risk of Wilms' tumor include:
- African-American race. In the United States, African-American children have a slightly higher risk of developing Wilms' tumor than children of other races. Asian-American children appear to have a lower risk than children of other races.
- Family history of Wilms' tumor. If someone in your child's family has had Wilms' tumor, then your child has an increased risk of developing the disease.Wilms' tumor occurs more frequently in children with certain abnormalities or syndromes present at birth, including:
- Aniridia. In aniridia (an-ih-RID-e-uh), the iris â€” the colored portion of the eye â€” forms only partially or not at all.
- Hemihypertrophy. Hemihypertrophy (hem-e-hi-PUR-truh-fee) means one side of the body or a part of the body is noticeably larger than the other side.Wilms' tumor can occur as part of rare syndromes, including:
- WAGR syndrome. This syndrome includes Wilms' tumor, aniridia, genital and urinary system abnormalities, and intellectual disabilities.
- Denys-Drash syndrome. This syndrome includes Wilms' tumor, kidney 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 average (macrosomia). Other signs may include abdominal organs that jut out into the base of the umbilical cord, a large tongue (macroglossia), enlarged internal organs and ear abnormalities. There is also an increased risk of tumors, including a type of liver cancer called hepatoblastoma.
-Wilms' tumor can't be prevented by anything you or your child can do.If your child has risk factors for Wilms' tumor (such as known associated syndromes), the doctor may recommend periodic kidney ultrasounds to look for kidney abnormalities. Although this screening can't prevent Wilms' tumor, it may help detect the disease at an early stage.