Bilateral cystic adrenal masses in the neonate associated with the incomplete form of Beckwith-Wiedemann syndrome.
ABSTRACT We report a case of bilateral multilocular cystic adrenal masses in a neonate who demonstrated somatic hypertrophy and macroglossia, which are considered to constitute an incomplete form of Beckwith-Wiedemann syndrome (BWS). After surgical removal of the masses, histological study revealed benign hemorrhagic macrocysts with cortical cytomegaly. There was no evidence of tumor or adrenal apoplexia. Although very rare, association of unilateral hemorrhagic adrenal macrocysts with BWS has been reported before; this is the first case of bilateral benign adrenal macrocysts associated with BWS.
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ABSTRACT: A new pseudotumorous lesion found in the adrenal cortex of six infants with Beckwith-Wiedemann syndrome is described. These cystic masses were discovered either prenatally by using sonography or early in the neonatal period as palpable flank masses. Imaging studies, including sonography and CT, could not confidently exclude malignancy. After the masses were removed surgically, histologic examination showed them all to be benign hemorrhagic macrocysts within the capsule or permanent cortex (in contrast to neonatal adrenal hemorrhage, which usually occurs more centrally in the fetal cortex). The cysts were as large as 8 cm in diameter, and in one case a solitary cyst was predominant. Hemihypertrophy was present in all cases. Four of the six lesions were right-sided, and there was a male-female ratio of 5:1. Benign hemorrhagic adrenocortical macrocysts are a cause of abdominal mass in the fetus and neonate with Beckwith-Wiedemann syndrome.American Journal of Roentgenology 10/1991; 157(3):549-52. · 2.90 Impact Factor