Wilms' Tumor Overview
Wilms' Tumor, also known as nephroblastoma, is the most common type of kidney cancer found in children. It is named after Max Wilms, the surgeon who first identified the disease in 1899. Wilms' Tumor usually occurs when certain cells in one or both kidneys multiply uncontrollably, growing into a malignant, or cancerous, mass. Approximately 500 cases of Wilms' Tumor are diagnosed each year, most of which are curable. The disease is highly responsive to treatment.
Risks
Wilms' Tumor is usually randomly occurring and not the result of an inherited disposition to the disease. It is possible for a very small percentage of children with the disease to have a family member who has had Wilms' Tumor. In such a situation children develop tumors at an earlier age and may have other developmental abnormalities.
Symptoms
Most frequently diagnosed in children between the ages of 2 and 3, Wilms' Tumor can also occur in infants and adults. It is usually detected by a lump in the abdomen found by the parent or physician. The tumor tends to grow without any pain and the child may feel and appear healthy. In a quarter of children diagnosed with the disease there are symptoms of blood in the urine, anemia, malaise, weight loss, and fever.
Diagnosis
A sonogram followed by a CT scan to examine the chest, abdomen, and pelvis for signs of cancer are standard procedure when Wilms' Tumor is suspected. The abdomen is scanned in order to look for the tumor itself as well as for signs of the cancer spreading from the kidney to the kidney bed, lymph nodes, or liver. The chest is scanned to determine if cancer spread to the lungs.
Staging
Staging will take place to determine the extent to which the tumor spread. Once the tumor has been staged a treatment plan will be developed to most effectively treat the disease.
I. The cancer is limited to the kidney and can be completely removed by surgery. 40% of Wilms' Tumors are stage I.
II. The cancer spread to areas surrounding the kidney and can be completely removed by surgery. 23% of Wilms' Tumors are stage II.
III. The cancer spread to areas surrounding the kidney including blood vessels and lymph nodes and cannot be completely removed by surgery. 23% of Wilms' Tumors are stage III.
IV. The cancer spread beyond the kidneys vicinity into the lungs, liver, bone, and brain. 10% of Wilms’ Tumors are state IV.
V. Tumors are found in the kidney and each kidney is staged separately. 5% of Wilms' Tumors are stage V.
Treatment
There are three main types of treatment that can be used alone or in combination with one another. The treatments are surgery, chemotherapy, and radiation. Surgery is usually the first treatment method for Wilms' Tumor. There are three types of surgery:
1. Radical Nephrectomy: Removes the entire kidney and the tissues surrounding it
2. Simple Nephrectomy: Removes only the affected kidney
3. Partial Nephrectomy: Used when there are tumors in both kidneys. This form of surgery removes tumors and sections of the kidney surrounding the tumors.
Chemotherapy is used during or after surgery is completed. When the tumor is more advanced, radiation can be combined with chemotherapy and surgery. If the tumor is too large for immediate surgical removal then chemotherapy and/or radiation is used to shrink the tumor down so it can be operated on.
Contact a CCBF doctor
If you have any questions about hemophilia or want to schedule an appointment, please call 212-746-3400 to schedule a visit with Dr. Aledo.
Visit our Resources section for brochures, latest publications, and upcoming conferences to learn more about Wilms' Tumors.
