Wilms' TumorWilms' tumor is a rare type of kidney cancer that affects children. It causes a tumor on one or both kidneys. Having certain genetic conditions or birth defects can increase the risk of developing Wilms' tumor. Children who are at risk should be screened for Wilms' tumor every three months until they turn eight.
Possible signs of a kidney tumor include a lump in the abdomen and blood in the urine. The tumor is usually diagnosed and removed in surgery. Other treatments include chemotherapy, radiation, biologic therapy or a combination. Biologic therapy, or immunotherapy, boosts your body's own ability to fight cancer. Most children with Wilms' tumor can be cured.
What causes Wilms' tumor?
The causes of all Wilms' tumor are not known. However, some of these tumors appear to result from changes in one or more of several genes. In the majority of cases, the genetic changes occur only in the kidney cells and not in other cells of the body. However, in some cases, other parts of the body are also affected. Some patients with Wilms' tumor are born with certain congenital anomalies, including absence of the iris of the eye (aniridia) or abnormalities of the urinary tract. Although it is rare, Wilms' tumor sometimes runs in families, which further supports a genetic connection.
DiagnosisSeveral tests are used to confirm a Wilms tumor diagnosis and determine the stage of the disease.
Tests that might be used include: -
- Ultrasonography (ultrasound or US), usually the first tool used to diagnose the condition, uses sound waves instead of X-rays to generate an image of the area doctors wish to view.
- Computed tomography (CT or CAT scan) produces a detailed cross-sectional view of an organ through X-rays. It is extremely useful in detecting tumors and determining whether cancer has spread to other areas.
- Magnetic resonance imaging (MRI) uses radio waves and strong magnets to produce detailed pictures of the internal parts of the body. This provides more intricate images that allow doctors to see if the cancer has invaded any major blood vessels near the kidney.
- X-rays are used to look for any metastasized areas, especially in the lungs.
- Bone scans use small amounts of radioactive material to highlight areas of diseased bone, if any exist.
- Laboratory tests such as blood tests and urinalysis check the general health of a patient and to detect any adverse side effects (such as low red or white blood cell counts) of the treatment.
Signs and symptoms of Wilms' tumorThe most common signs and symptoms of Wilms' tumor include: -
- A lump or mass in the abdomen of an otherwise well child
- Abdominal pain
- Blood in the urine
- High blood pressure
- Weight loss
- Urogenital infections
- Shortness of breath
- A general feeling of being tired and unwell
- Nausea and vomiting (these are rare)
TreatmentTreatment is determined by many factors, the most important being the stage of the cancer at diagnosis, and the condition, or histology, of the cancer cells when observed under a microscope. "Favorable" histology is associated with a good chance of a cure; tumors with "unfavorable" histology are more aggressive and difficult to cure. About 95% of Wilms tumors have favorable histology.
Doctors use a staging system to describe the extent of a metastasized tumor. It is extremely useful in determining prognosis (possibility for a cure) and the best course of treatment. For example, a child with very aggressive disease should be given an intensive regimen of medication to achieve the best chance for a cure. A child with less-invasive disease should be given the least amount needed to reduce long-term side effects from toxicity.
The stages are: -
- Stage I: - Cancer is found in one kidney only and can be completely removed by surgery. About 41% of all Wilms tumors are stage I.
- Stage II: - Cancer has spread beyond the kidney to the surrounding area, but can be completely removed by surgery. About 23% are stage II.
- Stage III: - Cancer has not spread beyond the abdomen, but cannot be completely removed by surgery. About 23% are stage III.
- Stage IV: - Cancer has spread to distant parts of the body; most commonly, the lungs, liver, bone, and/or brain. About 10% are stage IV.
- Stage V: - Cancer is found in both kidneys at diagnosis (also called bilateral tumors). About 5% are stage V.
Surgery is most often used to treat Wilms tumor. For stages I through IV, a radical nephrectomy ― removal of the cancer along with the entire kidney, ureter (tube that carries urine from the kidney to the bladder), adrenal gland (hormone-producing gland that sits on top of the kidney), and surrounding fatty tissue ― is done.
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