Published in:
01-09-2014 | GI Image
Giant Adrenal Myelolipoma
Published in: Journal of Gastrointestinal Surgery | Issue 9/2014
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A 44-year-old Caucasian woman presented to the emergency department for an evaluation of 5 days of progressive abdominal distention, nausea, and bilious emesis. The patient’s medical history was notable for congenital virilizing adrenal hyperplasia resulting in pseudohermaphroditism. She reported a remote treatment with steroid therapy terminated in adolescence due to poor medical follow up due to depression related to her gender identity. Upon discontinuation of her therapy, she gradually manifested male phenotype resulting in male pattern baldness, coarse facial hair, and a baritone voice. Physical exam was notable for a firm, non-tender, distended abdomen, hirsutism, and ambiguous genitalia. A contrast-enhanced computed tomographic (CT) scan was obtained demonstrating a very large, 26 cm × 24 cm × 9.5 cm, left-sided retroperitoneal mass of unclear origin and a right lower quadrant small bowel obstruction with pneumatosis (Fig. 1). The patient was taken emergently for an exploratory laparotomy with complete excision of the retroperitoneal mass and reduction of a closed loop small bowel obstruction. Pathologic examination of the mass resulted as a giant adrenal myelolipoma measuring up to 34 cm in its largest diameter and weighing 5,090 g (Fig. 2a, b).
Fig. 1
Large left adrenal myelolipoma presenting as small bowel obstruction. Coronal CT image of the abdomen after intravenous administration of iodinated contrast demonstrates a large heterogeneous mass centered in the left abdomen which contains soft tissue and fatty components (bracketed by the thick white arrows). The large mass is separate from the left kidney and displaces the left kidney inferiorly and to the right (thick black arrow). There are multiple dilated loops of small bowel in the lower right abdomen (thin black arrows) with crowding, pneumatosis and twisting of the mesenteric vessels at the transition point (thin white arrow) consistent with a closed loop obstruction
Fig. 2
Ovoid encapsulated mass composed of benign fibroadipose tissue associated with extramedullary hematopoiesis. Individual nodules measuring up to 13 cm across. The usual appearing adrenal structures are not seen. Nodular hyperplastic adrenal tissue consistent with myelolipoma (giant adrenal myelolipoma)
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