Endocrine Abstracts (2010) 21 P334

A rare cause of massive bilateral adrenal enlargement complicating management of congenital adrenal hyperplasia

Sath Nag, Atif Munir, Arif Ullah & Anjali Santhakumar

James Cook University Hospital, Middlesbrough, UK.

A 33-year-old lady presented to the surgeons with history of abdominal pain. CT scan abdomen done revealed massive adrenal enlargements reported as adrenal myelolipomas (left gland measuring 8.0×10.9×11.8 cm and multifocal lesions on the right with the larger one measuring 5.2×4.3×3.6 cm). She was referred to the endocrinology team where further history was elicited. She had been diagnosed as a child to have congenital adrenal hyperplasia (CAH) but was lost to follow up.

Her main complaints were severe hirsuitism and amenorrhoea and her current medication was hydrocortisone (10 mg twice a day).

Systemic examination demonstrated marked obesity and hirsuitism with no features of Cushings syndrome.

Serum testosterone was 3.4 nmol/l (0.5–2.6), 17-hydroxy-progesterone level 378 nmol/l (0–14) and ACTH 537 ng/l (10–80). Plasma catecholamines were normal.

The hydrocortisone was increased to 20 mg twice a day but caused weight gain and bloating. Despite concordance with medication (established with suppressed ACTH and 17-hydroxy-progesterone), she continued to be troubled by androgen excess.

She was subsequently switched to dexamethasone 1 mg to be taken at 10 pm at night.

However, her symptoms persisted with only a marginal reduction in the size of the adrenal masses.

She was referred for bilateral adrenalectomy as along with sub optimally controlled CAH, she remained at risk of spontaneous rupture and hemorrhage into the adrenal masses.

Discussion: Adrenal myelolipomas are rare non-functioning benign adrenal tumors with a reported incidence between 0.08 and 0.4%. To our knowledge <20 cases have been reported in association with CAH to date. Most are asymptomatic but nonspecific abdominal pain secondary to hemorrhage or mechanical compression can occur. Bilateral adrenalectomy for CAH patients poorly controlled on medical therapy is still little explored as a treatment with very little data available for adults.

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