Causes and pathogenesis of nephroblastoma

  Nephroblastoma May result from abnormal proliferation of undifferentiated tubule- and glomerulus-forming posterior renal germ. Nephroblastoma proliferative complex, which may also be a preneoplastic lesion of Wilms’ tumor. The deletion of the tumor suppressor genes WT1 and WT2 has been recognized in recent years to be associated with the development of some nephroblastomas.  The cause of nephroblastoma is unknown, and there is a certain tendency of familial occurrence with an incidence of 1% to 2%. It is also believed to be hereditary, and several children in a family can grow this tumor successively; Schweisguth reported that 5 pairs of siblings were among 600 cases. The New China Hospital also encountered two pairs of brothers who developed this tumor sequentially, and a young father who had unilateral nephroblastoma surgery as a child and whose son also developed nephroblastoma at the age of 3. Brown et al. encountered an extremely rare case of three consecutive generations of the disease.  Pathogenesis Nephroblastoma is embryonic in origin and develops in the parenchyma of the kidney, where it grows to an abnormal shape and invades the surrounding renal tissue. Almost all renal tumors in pediatric patients belong to this category. They originate from the primitive mesoderm, so a variety of undifferentiated tissues can be seen in pathological sections. Sometimes the tumor breaks through the peritoneum and invades the diaphragm, adrenal glands and colon. In 10% to 45% of surgical specimens, tumor tissue is seen in the renal veins.