Wilms' tumor and related abnormalities in the fetus and newborn.
Mesoblastic nephroma, a benign tumor, is the most common renal neoplasm in neonates. Wilms' tumor (WT) may occur in newborn infants, but is more common in older children. The molecular genetics of WT involves one or more genes located on Chromosome #11 and probably other locations not yet elicidated. Germline mutations cause less than 5% of WTs; most WTs are sporadic. Precursor lesions to WT called nephrogenic rests may be detected before evolution to WT by imaging studies. Developmental anomalies comprising several different syndromes are associated with nephrogenic rests and predisposition to WT. Prospective surveillance for WT may be feasible in high risk infants identified on the basis of physical findings followed by testing for predisposing gene defects and periodic imaging of the kidneys and other organs at risk until the period of risk has ended.
Bove
K E
KE
Childrens Hospital Research Foundation, and the Department of Pathology, University of Cincinnati College of Medicine, OH, USA. Kevin.Bove@UC.edu
D002880:Chromosomes, Human, Pair 11; D006801:Humans; D007231:Infant, Newborn; D007680:Kidney Neoplasms / Q000151:congenital / Q000175:diagnosis / Q000235:genetics; D009154:Mutation; D015997:Neonatal Screening; D009396:Wilms Tumor / Q000151:congenital / Q000175:diagnosis / Q000235:genetics