Wilms tumor
Wilms tumor is a form of kidney cancer that primarily develops in children. Nearly all cases of Wilms tumor are diagnosed before the age of 10, with two-thirds being found before age 5.
Wilms tumor is often first noticed because of abdominal swelling or a mass in the kidney that can be felt upon physical examination. Some affected children have abdominal pain, fever, a low number of red blood cells (anemia), blood in the urine (hematuria), or high blood pressure (hypertension). Additional signs of Wilms tumor can include loss of appetite, weight loss, nausea, vomiting, and tiredness (lethargy).
Wilms tumor can develop in one or both kidneys. About 5 to 10 percent of affected individuals develop multiple tumors in one or both kidneys. Wilms tumor may spread from the kidneys to other parts of the body (metastasize). In rare cases, Wilms tumor does not involve the kidneys and occurs instead in the genital tract, bladder, abdomen, chest, or lower back. It is unclear how Wilms tumor develops in these tissues.
With proper treatment, children with Wilms tumor have a 90 percent survival rate. However, the risk that the cancer will come back (recur) is between 15 and 50 percent, depending on traits of the original tumor. Tumors usually recur in the first 2 years following treatment and develop in the kidneys or other tissues, such as the lungs. Individuals who have had Wilms tumor may experience related health problems or late effects of their treatment in adulthood, such as decreased kidney function, heart disease, and development of additional cancers.
Other names
Embryonal adenosarcoma; Embryonal nephroma; Kidney Wilms tumor; Kidney, adenomyosarcoma, embryonal; Kidney, carcinosarcoma, embryonal; Kidney, embryoma; Kidney, embryonal mixed tumor; Nephroblastoma; Nephroma; Renal adenosarcoma; Renal cancer, Wilms; Renal Wilms tumor; Tumor, Wilms; Wilms' tumor
Inheritance pattern
Autosomal dominant; X-linked dominant; Not inherited
Related genes
TP53; IGF2; H19; WT1; CTNNB1; AMER1; REST; POU6F2; DROSHA; DGCR8
Source: MedlinePlus Genetics, U.S. National Library of Medicine.
