Beckwith-Wiedeman syndrome with bilateral Wilms tumours

Case contributed by Dr Ian Bickle

Presentation

Polyhydramnios during pregnancy. Palpable left upper quadrant mass. Normal MRI within the last 8 months. Tumour?

Patient Data

Age: 19 months
Gender: Female
MRI

Performed 8 months prior due to body asymmetry.

Hemihypertrophy of the soft tissues of the thorax and abdomen.

The volume and size of the soft tissues are greater on the left than the right.

The kidneys are normal in appearance.

Ultrasound performed indicating a left sided renal mass.

Large solid enhancing mass occupying most of the left kidney.

Several lesions in the right kidney, the largest 1.7cm in the lower pole which is solid.

Hemihypertrophy of the torso.

Lungs clear.

Case Discussion

Beckwith-Wiedemann syndrome (BWS) is a congenital overgrowth disorder.  There is a significantly increased risk of malignancy, in particular, Wilms tumour.

The prior MRI during the first year of life showed normal kidneys.   Within less than a year bilateral Wilms tumours were evident.

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Case information

rID: 60850
Published: 2nd Jul 2018
Last edited: 2nd Jul 2018
Inclusion in quiz mode: Included

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