Nephroblastomatosis

Case contributed by Ammar Haouimi
Diagnosis almost certain

Presentation

Distended abdomen with palpable masses

Patient Data

Age: 10 months
Gender: Female
ct
This study is a stack
Axial
non-contrast
This study is a stack
Axial renal
parenchymal phase
This study is a stack
Coronal renal
parenchymal phase
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Info

Both kidneys are diffusely enlarged with lobulated contours. The normal renal cortical tissue is replaced by multiple soft tissue masses of low attenuation (nephrogenic rests), compressing the enhancing renal parenchyma centrally.

Case Discussion

Features consistent with nephroblastomatosis.

Nephroblastomatosis is a rare condition, considered as a precursor of nephroblastoma (Wilms tumor). It is characterized by the presence of multiple residual embryonal cells (nephrogenic rests). When isolated, the recommended treatment consists of chemotherapy followed by monitoring. In the presence of an associated nephroblastoma, treatment consists of preoperative chemotherapy followed by surgery, then postoperative chemotherapy and monitoring.

 

Additional contributor: Dr. R Bouguelaa, MD

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