Beckwith-Wiedemann syndrome

Discussion:

Left limb hemihypertrophy in a patient with hepatoblastoma (imaging following resection). Both are associated with Beckwith-Wiedemann syndrome. This is an overgrowth syndrome most commonly associated with Wilms tumor.

Associated left adrenal cystic lesion. Adrenal cysts and other adrenal abnormalities including malignancy are associated with hemihypertrophy and Beckwith-Wiedemann syndrome. No associated systemic symptoms. Normal catecholamines/AFP. No change in size on follow up imaging.

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