Nephroblastomatosis

  • Etiology: focal or multifocal fetal metanephric blastema tissue (nephrogenic rests) that persist after birth and which can develop into Wilms tumor
  • CT: low density on contrast-enhanced exam
  • MRI: T1+T2 hypointense, if T2 hyperintense suspect Wilms tumor
  • Clinical: associated syndromes – Beckwith-Wiedemann, hemihypertrophy, Drash syndrome, sporadic aniridia, WAGR syndrome

Cases of Nephroblastomatosis

US and MRI of nephroblastomatosis
The diagnosis was Wilms tumor of the left kidney and nephrogenic rests from nephroblastomatosis in the right kidney.