Wilms’ tumor is one of the most common abdominal tumors in pediatrics, accounting for 5-6% of all pediatric cancers. Its incidence is approximately 1:10,000 live births, or 6-7 per million children under 15 years of age per year. WAGR syndrome is a rare group of syndromes consisting of Wilms’ tumor, aniridia, genitourinary tract anomalies, and mental retardation, and its incidence is low. The incidence of nephroblastoma is increased in patients with three congenital malformation syndromes. The three congenital malformation syndromes are: 1. WAGR syndrome, characterized by Wilms’ tumor, iris agenesis, genitourinary tract malformation, and mental retardation. 33% of patients develop Wilms’ tumor. patients with WAGR syndrome have a deletion of chromosome 11p13; 11p13 was found to contain the oncogene WT1, which is associated with Wilms’ tumor. 2. Denys-Drash syndrome, characterized by gonadal hypoplasia (male pseudohermaphroditism) and renal lesions occurring at an early age (e.g. diffuse glomerular tethering sclerosis) and leading to renal failure. Wilms’ tumor is found in a high percentage of patients with this syndrome. The genetic abnormalities are mainly mutations in the WT1 gene. 3Beckwith-Wiedemann syndrome, characterized by organomegaly, megaglossus, eccentric hypertrophy, umbilical protrusion, and adrenocortical cell hypertrophy. Patients are prone to Wilms’ tumor, and deletions of chromosome 11p15.5 are often detected. Deletion of the WT1 gene is an important part of the pathogenesis of the disease. The WT1 gene encodes a transcriptional repressor, and by binding to the promoter WT1 can repress the transcription of the gene in question; therefore, WT1 is also considered to be an oncogene. Its deletion or inactivation may trigger the development of Wilms’ tumor. In addition to the WT1 gene, there are two other genes associated with Wilms tumor, including the WT2 gene located on 11p15.5 and another unknown gene. Wilms tumorigenesis also follows the “second strike doctrine” proposed by Knudson. The vast majority of Wilms tumors are non-hereditary or sporadic, and the tumors are usually unilateral; about 10% of cases are bilateral, but only about 1% of these cases are found to have a family history, and the majority of bilateral Wilms tumors have de novo germ cell mutations. Wilms’ tumor was first described by Dr. Max Wilms in 1899 and is also known as nephroblastoma. It is the most common malignant tumor of the kidney in childhood, mostly occurring in children and occasionally in adults, with no significant gender differences. It may be asymptomatic in early stages. Children mainly present with weakness and large abdominal masses. The mass is rapidly growing, round or oval, rubbery and hard, with a smooth or mildly lobulated surface, neat margins, and no pressure pain. The tumor rarely invades the renal pelvis and calyces, so hematuria is also not obvious. Fever and nephrogenic hypertension are common. Urography can show deformation and displacement of renal pelvis and calyces, and in severe cases, renal function is damaged, and renal pelvis and calyces are not visible. The tumor may metastasize to the lung, liver, pleura, para-aortic and hilar lymph nodes via blood vessels and lymphatic vessels. Familial Wilms’ tumor is usually bilateral, with an early age of onset. Such cases are rare. Diagnosis and prevention】 The diagnosis and prenatal diagnosis of Wilms’ tumor are based on clinical manifestations, imaging and pathological examination. Karyotype analysis and FISH testing or determination of WT1 gene mutation can help in the diagnosis. A combination of surgical resection and chemotherapy has good results. The 2-year survival rate of Wilms’ tumor can reach 60%-94%, and no recurrence in 2-3 years is the standard of cure. Genetic counseling] Most Wilms tumors are sporadic, but patients should be advised to undergo chromosomal analysis and DNA analysis, with special attention to familial inheritance. The risk of recurrence is 50% for confirmed hereditary cases.