Medical expert of the article
New publications
Wilms tumor: an overview of information
Last reviewed: 23.04.2024
All iLive content is medically reviewed or fact checked to ensure as much factual accuracy as possible.
We have strict sourcing guidelines and only link to reputable media sites, academic research institutions and, whenever possible, medically peer reviewed studies. Note that the numbers in parentheses ([1], [2], etc.) are clickable links to these studies.
If you feel that any of our content is inaccurate, out-of-date, or otherwise questionable, please select it and press Ctrl + Enter.
Wilms tumor is named in honor of the German surgeon Max Wilms (1867-1918), who first published a description of seven observations of the appearance of this neoplasm in children in 1899.
Epidemiology of Wilms tumor
The tumor of Wilms is 5.8% of all malignant neoplasms in children. The incidence of Wilms tumor in the child population is 7.20 per 100 000. The average age of the sick 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 up to 5 years, this tumor ranks 3-4 th in terms of the incidence rate, accounting for about half of all tumors detected in children of this age. The incidence of morbidity between boys and girls is the same.
[Causes and pathogenesis of Wilms tumor
Wilms tumor in 60% of cases is the result of somatic mutation, 40% of Wilms tumors are caused by hereditary-deterministic mutations. Great importance in the pathogenesis of this tumor is given to mutations of recessive suppressor genes WT1, WT2 and p53, located in the 11th chromosome. According to the two-stage theory of carcinogenesis Knudson, the starting mechanism of the appearance of the Wilms tumor can be considered a mutation in the germ cell, and then the alteration of the alternative gene in a homologous chromosome. In addition to idiopathic aberrations, Wilms' tumor can become a manifestation of such hereditary syndromes as Beckwith-Wiedemann syndrome, WAGR (Wilms tumor, aniridia, urogenital abnormalities and mental retardation), hemygypertrophy, 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 a new growth is detected when the child is examined in the absence of any complaints (9.2%). In addition, macrohematuria (15.1%), constipation (4 3% decrease in body weight (3.8%) of urinary infection (3.2%) and diarrhea (3.2%) may occur, and Wilms tumor in children - nausea, vomiting, pain, the appearance of abdominal hernia with a large tumor and an increase in blood pressure.
Where does it hurt?
What do need to examine?
What tests are needed?
Who to contact?
Treatment of Wilms tumor
The best results are achieved by a multimodal approach, including surgery, chemotherapy and radiation therapy. All patients undergo nephrectomy and cytostatic therapy.
The question of the optimal sequence of surgery and chemotherapy remains controversial.
Radiation therapy is performed in the adjuvant mode, with a high prevalence of the tumor process, as well as in the presence of adverse factors of disease progression. Treatment algorithms are determined based on the stage of disease and tumor anaplasia.
In North America, Wilms' tumor is treated with immediate nephrectomy followed by chemotherapy with or without post-operative radiotherapy.