Nephroblastoma is one of the pediatric abdominal solid tumors with a better prognosis than neuroblastoma, and has a very good chance of being treated. Nephroblastoma is the most common malignant kidney tumor in pediatric patients, which was first reported by Wilms and is therefore also called “Wilms tumor”. In the past 20 years, due to the development of comprehensive treatment measures such as surgery, chemotherapy and radiotherapy, as well as the application of multicenter research results such as the Renoblastoma Research Group in the United States and the International Society of Pediatric Oncology in Europe, the efficacy of treatment has been significantly improved, complications in low-risk patients have been gradually reduced, and the long-term survival rate of high-risk patients has been further improved. Incidence of nephroblastoma The incidence of nephroblastoma in infants and children is about 1-2 per million population, and it is more likely to occur in infants and children aged 1-3 years. The tumor originates from the posterior renal blastoderm and is an embryonal tumor that occurs in the remnant immature kidney and may be associated with genitourinary malformations, limb deformities or mental retardation. Symptoms of nephroblastoma An abdominal mass is the most common presentation and can be discovered incidentally while changing clothes or bathing. 95% of patients have a mass palpated at the first visit. The mass is located in the upper abdomen on one side of the ribs, with smooth surface, moderate firmness, no pressure pain, and may have some mobility in the early stage. When the tumor is rapidly enlarging/huge, it may produce compression symptoms, shortness of breath, lack of appetite, emaciation and irritability. About 1/3 of children have abdominal pain, ranging from local discomfort and mild pain to severe pain and colic, and if accompanied by fever, anemia and hypertension, it often indicates subperitoneal hemorrhage. There is also acute abdomen caused by tumor rupture. About 25% of children have microscopic hematuria and 10-15% have carnal hematuria. Hematuria is most often induced by minor trauma to the enlarged kidney or related to tumor invasion of the renal pelvis and does not indicate advanced tumor. Increased blood pressure occurs in about 30% of cases, either due to renin production by tumor cells or high renin-angiotensin due to renal vascular embolism or renal artery compression. Often, blood pressure returns to normal after tumor removal. It may be combined with acute renal failure, varicocele, and hypoglycemia. Erythrocytosis is rare and the cause may be related to tumor production of erythropoietin. Combined with nephrotic syndrome, it is called Wilms’ nephritis. Inferior vena cava obstruction may lead to hepatomegaly and ascites, and if it invades the right atrium, it may lead to congestive heart failure. Hematogenous metastasis may spread to all parts of the body, and pulmonary metastasis is the most common, which may lead to cough, pleural effusion, chest pain, hypothermia, anemia and cachexia. How to treat nephroblastoma Radical nephrectomy is a common surgical procedure for nephroblastoma treatment. In addition to removal of the primary tumor and kidney, retroperitoneal lymph node dissection is also required. Partial nephrectomy or tumor enucleation is feasible when conditions allow and in the case of bilateral nephroblastoma. In addition to surgical resection, chemotherapy and radiotherapy are other important tools currently considered to improve their survival. In the stage without chemotherapy and radiotherapy, the overall survival rate of nephroblastoma is only 20-40%, while with the development of comprehensive treatment, the overall survival rate can reach 85-90%. Even more, the overall survival rate of children with FH pathology (good prognosis) can reach 94-100%, while the overall survival rate of children with UH pathology (poor prognosis) can also reach about 70%. The prognosis is mainly related to the stage of the disease and the type of pathology.