Nephroblastomatosis

Case contributed by Dr. Foryoung

Presentation

An infant with no past medical history. On physical exam, the left side of the limbs are larger than the right.

Patient Data

Age: 11 months
Gender: Female
Ultrasound

Ultrasound at presentation

Left kidney is larger and demonstrates lobularity with hypoechoic cortical nodularity. Differential at the times is persistent fetal lobularity versus nephrogenic rest.

MRI

MRI at presentation

Coronal and axial T2 HASTE shows multiple nodular lesions of varying sizes and shapes in bilateral kidneys, left greater than right, predominantly peripherally located, with features most consistent with bilateral nephroblastomatosis.
Contrast MRI at the time of presentation shows no discrete heterogeneity or area of focally abnormal contrast enhancement is identified, as to convincingly suggest malignant transformation.

 

MRI

Study one year later

Pre and Post contrast MRI at a follow-up study 1 year from the initial presentation which demonstrates multiple hypointense nodules in both kidneys have increased in size as described above. Left lower pole lesion shows faint internal and peripheral enhancement. Findings are concerning for malignant transformation of the previously identified perilobar nephrogenic rests.

Case Discussion

Nephrogenic rest (NR): persistent metanephric blastema 1

  • in the periphery: perilobular
  • within the kidney: intralobular
  • multiple NRs: nephroblastomatosis

NRs are important to diagnose and follow because they are precursors to Wilms tumor.

In some studies, up to 35% of diffuse hyperplastic perilobar NR transform to Wilms Tumor. This is considered the most at risk group 

The patient, in this case, demonstrates this in the initial ultrasound images.

Other syndromes associated with NRs (and Wilms tumor) are

On imaging, the NRs appear homogeneous on all imaging modalities. This is a very important distinction to Wilms tumor, which is heterogeneous. The NRs do not enhance to the same degree as the surrounding kidney

The patient, in this case, demonstrates homogeneous enhancement less that kidney parenchyma on the initial MRI images.

Unfortunately, a repeat MRI one year later demonstrates increased peripheral and internal enhancement, findings concerning for malignant transformation.

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Case information

rID: 39984
Published: 30th Sep 2015
Last edited: 14th Sep 2019
Inclusion in quiz mode: Included

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