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Wilms tumor - Information Overview
Last reviewed: 04.07.2025
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Wilms' tumor is named after the German surgeon Max Wilms (1867-1918), who first published a description of seven cases of this tumor in children in 1899.
Epidemiology of Wilms' tumor
Wilms tumor accounts for 5.8% of all malignant neoplasms in children. The incidence of Wilms tumor in the pediatric population is 7.20 per 100,000. The average age of those affected is 36 months for boys and 43 months for girls. The peak incidence is recorded at the age of 2 to 4 years. In the age group under 5 years, this tumor ranks 3-4 in incidence, accounting for about half of all neoplasms detected in children of this age. The incidence ratio between boys and girls is the same.
[ Causes and pathogenesis of Wilms tumor
Wilms' tumor in 60% of cases is a consequence of somatic mutation, 40% of Wilms' tumors are caused by hereditary-determined mutations. Mutations of recessive suppressor genes WT1, WT2 and p53, located in chromosome 11, are of great importance in the pathogenesis of this tumor. According to Knudson's two-stage theory of carcinogenesis, the starting mechanism of Wilms' tumor can be considered a mutation in the germ cell, and then a change in the alternative gene in the homologous chromosome. In addition to idiopathic aberrations, Wilms tumor may be a manifestation of such hereditary syndromes as Beckwith-Wiedemann syndrome, WAGR (Wilms tumor, aniridia, genitourinary anomalies, and mental retardation), hemihypertrophy, Denys-Drash syndrome (intersex disorders, nephropathy, Wilms tumor), and Le-Fraumeni syndrome.
Symptoms of Wilms' Tumor
The most common symptom of Wilms tumor in children is the asymptomatic appearance of a palpable tumor (61.6%). Often, the neoplasm is detected during examination of a child in the absence of any complaints (9.2%). In addition, macrohematuria (15.1%), constipation (4 3% weight loss (3.8%), urinary infection (3.2%), and diarrhea (3.2%) are possible. Rarely described manifestations of Wilms tumor in children are nausea, vomiting, pain, the appearance of an abdominal hernia with a large tumor, and increased blood pressure.
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Wilms tumor treatment
The best results are achieved with a multimodal approach, including surgery, chemotherapy and radiation therapy. All patients undergo nephrectomy and cytostatic therapy.
The optimal sequence of surgery and chemotherapy remains controversial.
Radiation therapy is performed in an adjuvant mode, with a high prevalence of the tumor process, as well as in the presence of unfavorable factors for disease progression. Treatment algorithms are determined based on the stage of the disease and tumor anaplasia.
In North America, Wilms tumor is treated with immediate nephrectomy followed by chemotherapy with or without postoperative radiation therapy.