Giant Adrenal Myelolipoma

Keywords Giantmyelolipoma .CongenitaladrenalhyperplasiaCase PresentationA 44-year-old Caucasian woman presented to the emergencydepartmentfor anevaluationof5 daysofprogressive abdom-inaldistention,nausea,andbiliousemesis.Thepatient’smed-ical history was notable for congenital virilizing adrenal hy-perplasia resulting in pseudohermaphroditism. She reported aremote treatment with steroid therapy terminated in adoles-cenceduetopoormedicalfollowupduetodepressionrelatedto her gender identity. Upon discontinuation of her therapy,she gradually manifested male phenotype resulting in malepattern baldness, coarse facial hair, and a baritone voice.Physical exam was notable for a firm, non-tender, distendedabdomen, hirsutism, and ambiguous genitalia. A contrast-enhanced computed tomographic (CT) scan was obtaineddemonstrating a very large, 26 cm×24 cm×9.5 cm, left-sided retroperitoneal mass of unclear origin and a right lowerquadrant small bowel obstruction with pneumatosis (Fig. 1).The patient was taken emergently for an exploratorylaparotomywithcompleteexcisionoftheretroperitonealmassand reduction of a closed loop small bowel obstruction. Path-ologic examination of the mass resulted as a giant adrenalmyelolipoma measuring up to 34 cm in its largest diameterandweighing5,090g(Fig.2a, b).Adrenal myelolipomas are composed of mature adiposeand hematopoietictissues.The adipose and myeloid elementsare found in varied proportions. They are biochemically inac-tiveneoplasmsandbenigninnature.Theywerefirstdescribedby Gierke in 1905 and were formally termed myelolipoma in1929 by Oberling.