Nephroblastomatosis

Episode Finale

Nephroblastomatosis

• Nephroblastomatosis consists of diffuse or

multifocal involvement of the kidneys with

nephrogenic rests.

Nephroblastomatosis

• Nephrogenic rests are foci of metanephric

blastema that persist beyond 36 weeks gestation

and have the potential for malignant

transformation into Wilms tumor.

Nephroblastomatosis

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.

Nephroblastomatosis

Imaging

• Microscopic foci of nephroblastomatosis are not

identifiable with any imaging modality.

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.

Nephroblastomatosis

US:

• Focal heterogenous well-defined mass.

Nephroblastomatosis

Plain X-Ray:

Nephroblastomatosis

IVU:

Nephroblastomatosis

US:

• Focal heterogenous well-defined mass.

Nephroblastomatosis

US:

• Focal heterogenous well-defined mass.

Nephroblastomatosis

US:

• Diffuse

Nephroblastomatosis

CT:

• Low-attenuation peripheral nodules with poor

enhancement relative to that of adjacent normal

renal parenchyma

Nephroblastomatosis

CT:

Nephroblastomatosis

CT:

Nephroblastomatosis

CT:

Nephroblastomatosis

CT:

Nephroblastomatosis

CT:

Nephroblastomatosis

CT:

Nephroblastomatosis

MRI:

• The nodules demonstrate low-signal-intensity

foci on both T1-weighted and T2-weighted

images.