Nephroblastomatosis

1. Nephroblastomatosis
Episode Finale

2. Nephroblastomatosis
• Nephroblastomatosis consists of diffuse or
multifocal involvement of the kidneys with
nephrogenic rests.

3. Nephroblastomatosis
• Nephrogenic rests are foci of metanephric
blastema that persist beyond 36 weeks gestation
and have the potential for malignant
transformation into Wilms tumor.

4. Nephroblastomatosis

5. Nephroblastomatosis
• It is currently believed that nephrogenic rests
give rise to approximately 30%–40% of Wilms
tumors, and they are found in up to 99% of
bilateral Wilms tumors.

6. Nephroblastomatosis
Imaging
• Microscopic foci of nephroblastomatosis are not
identifiable with any imaging modality.

7. Nephroblastomatosis
The role of imaging in evaluating patients suspected of
having nephroblastomatosis:
(a) Preoperative assessment in a patient suspected of
having Wilms tumor to exclude macroscopic NRs in the
contralateral kidney.
(b) Follow-up of patients with known micro- or macroscopic
nephroblastomatosis to detect neoplastic change.
(c) Screening of patients with syndromes associated with
nephroblastomatosis and Wilms tumor.

8. Nephroblastomatosis
US:
• Focal heterogenous well-defined mass.

9. Nephroblastomatosis
Plain X-Ray:

10. Nephroblastomatosis
IVU:

11. Nephroblastomatosis
US:
• Focal heterogenous well-defined mass.

12. Nephroblastomatosis
US:
• Focal heterogenous well-defined mass.

13. Nephroblastomatosis
US:
• Diffuse

14. Nephroblastomatosis
CT:
• Low-attenuation peripheral nodules with poor
enhancement relative to that of adjacent normal
renal parenchyma

15. Nephroblastomatosis
CT:

16. Nephroblastomatosis
CT:

17. Nephroblastomatosis
CT:

18. Nephroblastomatosis
CT:

19. Nephroblastomatosis
CT:

20. Nephroblastomatosis
CT:

21. Nephroblastomatosis
MRI:
• The nodules demonstrate low-signal-intensity
foci on both T1-weighted and T2-weighted
images.