Beckwith-Wiedemann syndrome
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Patient Data
Macroglossia on the lateral view of face. Liver clips from resection of hepatoblastoma on the chest x-ray. Hemihypertrophy on the frontal projections of the limbs.
Status post partial hepatectomy for resection of hepatoblastoma.
The left adrenal is enlarged with a central low density cyst. The differential for this includes an adrenal cyst, but malignancy associated with ipsilateral hemihypertrophy needs to be considered, including neuroblastoma, pheochromocytoma
Normal tracer uptake throughout the imaged skeleton. The left lower limbs is longer than the right.
Case 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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