Wilms’ Tumor is a cancerous tumor that appears on the kidneys. Also referred to as nephroblastoma, Wills’ Tumor is a rare cancer that affects less than eight children in 100,000. The tumor usually appears before the age of five, but can be present in newborns. Children over the age of eight are rarely affected. One in ten children has a tumor in both kidneys, but most patients have just one tumor. Wilms’ tumor is the fifth most common malignancy and renal disorder in infants and children.
Wilms’ tumor may not be diagnosed early in a child’s life, because tumors can get large without causing any symptoms or pain. Symptoms may be non-specific, such as fever, blood in the urine, reduced appetite, weight loss, constipation, stomach pain (which can be mistaken for colic), nausea, vomiting or a non-specific tiredness.
The abdomen may show signs of unusual swelling and a child’s pediatrician may pick up on a tumor during an exam.
There are other conditions associated with Wilms’ tumor. These conditions are
Beckwith-Wiedemann syndrome (BWS), congenital hemihypertrophy, WAGR syndrome and, Denys-Drash syndrome. WAGR syndrome is a combination of conditions: Wilms tumor, aniridia, genitourinary malformations, and mental retardation. Aniridia is genetic eye condition that is present at birth. Hemihypertrophy is a condition in which one side of the body is noticeably larger than the other side.
Diagnosis of Wilms’ Tumor is made via imaging, blood and urine tests. Imaging is required to detect and locate the presence of a tumor. Imaging tests include computerized tomography CT, ultrasound and magnetic resonance imaging. If this cancer has spread, the first site is usually to the lungs. A doctor may order a chest imaging test to determine if the cancer has spread to the lungs. A bone scan may also be ordered to determine if any cancer is in the bones.
Diagnosis also determines the stage of the cancer. The stage of the cancer is based on where (if at all the cancer has spread). Stage I describes cancer that is only in the kidney. Stage II defines cancer that has spread to tissues and structures surrounding the kidney. Structures that are affected in Stage II are surrounding fat and blood vessels. In Stage II the cancer can usually be completely removed through surgery. Stage III describes cancer that has spread from the kidney to nearby lymph nodes or other structures within the abdomen. Surgery cannot remove all of the cancer in Stage III. Stage IV describes cancer that has spread to the lungs, liver or brain. Stage V defines cancer that is in both kidneys.
The stage of cancer helps to choose the course of treatment.
The exact cause of Wilms’ tumor is not known. What happens on a cellular level is, however. When kidney cells doe not mature during gestation, they begin to multiply after birth and become a malignant mass. This mass is a Wilms’ tumor. Some patients with Wilms’ tumor have other genetic defects, indicating that a genetic abnormality is involved. Wilms’ tumor can occur in multiple family members, from one generation to the next, indicating some hereditary factors may be involved.
Treatment for Willms’ Tumor includes surgery, chemotherapy and radiation therapy - a combination of any three of these. Sometimes radiation therapy is used prior to surgery to shrink the tumor, to make it more easily to excise. During surgery, the surgeion will examine both kidneys and surrounding tissues for signs of metastasized cancer. The surgeon may perform biopsies of surrounding tissue if cancer is suspected. Rarely are both kidney’s affected by Wilms’ tumor. The surgeon usually removes just the affected kidney. Chemotherapy and radiation therapy may follow surgery, not immediately, but within a short period of time. Chemotherapy and radiation therapy are used only when the cancer has spread to other organs or surrounding tissues. Ninety-percent of children who have Wilms’ tumor are living healthy lives two years after treatment and diagnosis.
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