ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Endocrine Abstracts (2017) 50 EP006 | DOI: 10.1530/endoabs.50.EP006

Rare case of bilateral massive adrenal myelolipoma in association with congenital adrenal hyperplasia

M. Ibrahim Shahbuddin & David Hughes

Royal Derby Hospital, Derby, UK.

Introduction Adrenal myelolipomas are rare small benign tumours composed of mature fat and hemopoietic tissues, which can be associated with congenital adrenal hyperplasia (CAH). We report a rare case of massive bilateral adrenal myelolipomas.

Case report A 48 year old gentleman with CAH diagnosed during childhood presented at a routine endocrine appointment. He had not regularly attended his appointments or taken his steroids for over seven years. Clinical examination at that time revealed bilateral enlarged testicles with a perineal scar from previous genital surgery along with a palpable abdominal mass. Coincidentally the patient had recently undergone an abdominal ultrasound organised by his primary care physician for suspected cholecystitis. The scan revealed bilateral abdominal masses which were subsequently characterised by CT abdomen as bilateral massive adrenal myelolipomas displacing abdominal viscera (right adrenal measuring 20 cm & left adrenal, 18 cm). Initiation of steroid therapy resulted in testicular shrinkage, erectile dysfunction and decline in early morning testosterone from 11 nmol/l to 0.3 nmol/l [normal=8.3–27.8 nmol/l]. In preparation for adrenalectomy detailed adrenal vasculature imaging was performed. He subsequently underwent successful elective bilateral adenalectomy of a 2.9 kg right adrenal measuring macroscopically 27×21 cm, 2.1 kg left adrenal measuring 25×15 cms and 85 g left adrenunculus. Histology showed completely excised myelolipomata with unremarkable medullary tissue with no evidence of malignancy. He made a good post-operative recovery and was discharged home on prednisolone, fludrocortisone and testosterone replacement.

Discussion Various theories have been proposed, but etiopathogenesis of adrenal myelolipomas still remains unclear. Being off steroids in the presence of CAH was probably the causative factor in our patient. Small tumours less than 5 cms can be managed conservatively with annual follow up but larger and the symptomatic tumours usually require surgical resection. Several cases of unilateral massive adrenal myelolipomas have been reported but bilateral massive adrenal myelolipomas are extremely rare.

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