Renal cell carcinoma (cancer of the kidney) occurs rarely in children. The
annual incidence rate is approximately 4 cases per 2 million children.
Renal cell carcinoma may be associated with von Hippel-Landau disease, a
hereditary condition. Renal cell carcinoma has also been associated with
tuberous sclerosis, a hereditary disease characterized by benign (noncancerous)
fatty cysts in the kidney. It may also be associated with sickle cell
anemia and/or neuroblastoma. Renal cell carcinoma usually presents as an
abdominal mass, and there may be discomfort, pain, or blood in the urine.
The tumor can spread to the lungs, bones, liver, and lymph nodes and often
has spread before the diagnosis is made. The primary treatment includes
total surgical removal of the kidney and associated lymph nodes. Treatment
of metastatic disease (cancer in other parts of the body) is presently
unsatisfactory but usually includes the use of immune system modulators
such as interferon-alfa and interleukin-2. Rare spontaneous disappearance
of lung metastasis may occur with removal of the primary tumor.
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