• Wilms’ Tumor Overview

    Approximately 500 children, mostly between 2 and 4 years of age, are diagnosed with Wilms' tumor each year in the United States. Most children are successfully treated, usually by partial or full surgical removal of the tumor (depending its size and location). Wilms’ tumor most often starts in the kidneys but, in rare cases, can occur in other parts of the body. It is the most common type of kidney cancer. These tumors can be very large and may spread to other body tissues, most often the lungs.

    While Wilms’ tumor can occur at any age between infancy and age 15, it is usually detected by the time a child is 3. Typically, only one kidney is affected, but nearly 5 percent of children with Wilms’ tumor have tumors in both kidneys. 

    Wilms' Tumor Treatment at Dana-Farber/Boston Children’s 

    Children with Wilms’ tumor are treated at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center through the Kidney Tumor Program within our Solid Tumor Center

    Symptoms & Diagnosis

    The symptoms of Wilms’ tumor may resemble other, more common conditions or medical problems. The most common symptoms are: 

    • Swelling of the abdomen
    • Blood in the urine
    • Abdominal pain
    • Decreased appetite
    • Weakness or tiredness
    • Fever
    • High blood pressure
    • Constipation 

    In addition to a complete physical examination, doctors often recommend a number of different tests to diagnose Wilms’ tumor, including:

    • Abdominal ultrasound – an imaging test that can provide an outline of the kidneys and the tumor and determine if there are problems in the renal or other major veins in the abdomen or determine if there are lesions or tumors in the opposite kidney
    • X-ray – chest or abdominal X-rays can help determine the size and location of the tumor and whether it has spread
    • Computerized tomography (CT or CAT) scan 
    • Magnetic resonance imaging (MRI) – used in some cases to look for tumors in blood vessels or other organs
    • Blood and urine tests – to evaluate kidney function
    • Biopsy – to determine what type of tumor is present

    After all tests are completed, doctors will be able to outline the best treatment options.

    Treatment & Care Options

    A number of treatments are recommended for Wilms’ tumor, including:

    • Surgery – often removal of the entire kidney and tissue around it, as well as the ureter and adrenal gland (which sits on top of the kidney); for children with tumors in both kidneys, kidney-sparing surgery aims to remove the tumor while leaving healthy kidney tissue alone. Surgeons from the Urologic Tumors Program at Boston Children's Hospital are brought in when surgery is required.
    • Chemotherapy and radiation therapy following surgery 
    • Stem cell transplant – used in difficult-to-treat cases

    Progressive or Recurrent Disease

    Anywhere from 15 to 50 percent of children with Wilms’ tumor will experience recurrence, which is treated with additional surgery, chemotherapy  and/or radiation therapy.

    Long-term Outlook

    Anywhere from 15 to 50 percent of children with Wilms’ tumor will experience recurrence, which is treated with additional surgery, chemotherapy  and/or radiation therapy.With prompt, aggressive treatment, most children with Wilms’ tumor are successfully treated and experience relatively few side effects. More aggressive tumors that have spread significantly may be harder to cure. The goal of treatment is both to cure the cancer and minimize the long-term effects of therapy. Children, however, are at some risk of late-effects of the cancer and its treatment, including reduced kidney function, heart and lung problems after chemotherapy or radiation therapy, and slowed or decreased growth and development.
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