Beckwith-Wiedemann syndrome

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These large, lobulated adrenals arose in a case of Beckwith-Wiedemann syndrome. Other abnormalities seen with this rare syndrome include linear fissure of the external ear lobule, macroglossia, hemihypertrophy, genital tract abnormalities, omphalocele, and microcephaly, among others.

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The large cells with large, bizarre nuclei represent adrenal cytomegaly of the fetal cortex. This occurs with Beckwith-Wiedemann syndrome. It may uncommonly occur as a focal incidental finding.

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