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[edit] Discussion of Nephroblastomatosis

  • Nephroblastomatosis consists of diffuse or multifocal involvement of the kidneys with nephrogenic rests.
  • Nephrogenic rests are foci of metanephric blastema that persist beyond 36 weeks gestation and have the potential for malignant transformation into Wilm's tumor.
  • Nephrogenic rest are found incidentally in 1% of infants.
  • Currently believed that nephrogenic rests give rise to approximately 30%–40% of Wilms tumors
  • Nephrogenic rests are found in up to 99% of bilateral Wilms tumors.

  • Treatment for nephrogenic rests is controversial. Some investigators recommend chemotherapy, whereas others maintain that close serial radiologic evaluation of enlarging masses is sufficient.

[edit] Imaging Findings for Nephroblastomatosis

  • At CT, macroscopic nephrogenic rests appear as low-attenuation peripheral nodules with poor enhancement relative to that of adjacent normal renal parenchyma.
  • At MR imaging, the nodules demonstrate low-signal-intensity foci on both T1-weighted and T2-weighted images.
  • US may demonstrate hypoechoic nodules but is less sensitive than MR imaging and CT.
  • Diffuse nephroblastomatosis is usually seen as reniform enlargement with a thick peripheral rind of tissue that may show striated enhancement. At US, the enlarged kidney may have diffusely decreased echogenicity.
  • Lymphoma can mimic the appearance of nephroblastomatosis but is unusual in infants and young children.

[edit] Images

Patient #1

[edit] See Also

[edit] External Links

[edit] References for Nephroblastomatosis