Endocrine Abstracts (2015) 38 P427 | DOI: 10.1530/endoabs.38.P427

Cushing's syndrome secondary to bilateral functioning adrenocortical adenoma

Zeenat Banu & Saiful Kassim

Derriford Hospital, Plymouth, UK.

Sixty seven years old lady was admitted with profound weakness and weight loss for 3–4 months. She has background of Primary hyperparathyroidism, HTN and Osteoporosis.

Examination revealed BP of 175/90 mmHg, slightly plethoric with proximal myopathy and bruising. CTTAP bilateral axillary and para aortic lymphadenopathy and right adrenal adenoma 32×26 mm. CT guided biopsy which showed CLL. She was worked up for hypercortisolism (Table 1).

She had overnight 1 mg DST which showed cortisol levels of 156 nmol/l. Her 48 h DST results were 793–158 nmol/l. She had MRI adrenal which revealed right adrenal of 3.3 cm and left adrenal of 1.4 cm with normal MRI pituitary. DEXA Scan showed osteoporosis. Octreotide Scan no uptake in both adrenals. Hormonal dynamic studies revealed bilateral autonomous secretion of cortisol (Table 2). She underwent laparoscopic bilateral adrenalectomies, commenced replacement therapy with hydrocortisone (HCT) and fludrocortisone postoperatively. Histopathology showed nodular proliferation of predominantly lipid containing cells suggestive of bilateral adrenal cortical adenomata.

Table 1
InvestigationsPatients values (normal values)
24 h urinary free cortisol (1st)429 (50–300 nmol/24 h)
24 h urinary free cortisol (2nd)306 (50–300 nmol/24 h)
Plasma ACTH (1st)6 (0.0–40 ng/l)
Plasma ACTH (2nd)<5 (0.0–40 ng/l)
24 h urinary Metadrenaline0.58 (0.0–2.0 μmol/24 h)
24 h urinary Normetadrenaline0.78 (0.0-4.3 μmol/24 h)
Plasma aldosterone concentration71 (40–310 ng/l)
Plasma renin activity2.8 (3–40 mU/l)
Dehyroepiandrosterone sulphate5.8 (0.26–6.68 μmol/l)
Androstenedione21.2 (0.0–3.5 nmol/l)
Table 2 Results of Adrenal vein sampling
PeripheralRight adrenal veinLeft adrenal veinA:U Ratio
Baseline cortisol47525597403.45 (>2)
Baseline aldosterone1463000999
15 min post ACTH
Cortisol71917 50010 7431.62 (<2)
Aldosterone26014 54010 570
25 min post ACTH
Cortisol82617 50017 5001.0 (<2)
Aldosterone28513 16014 970

Bilateral functioning adrenal adenomas are very rare. Only 25 cases are reported since 1977. Abnormal adrenal expression of receptors for various hormones can lead to ACTH independent bilateral macro nodular hyperplasia (AIMAH). These are multiple nodules, not encapsulated with hypertrophic adjacent areas. However cortisol producing adrenal adenomas are usually single, unilateral and encapsulated, usually associated with suppressed ACTH levels to atrophy adjacent non nodular area.

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