Cushing

Zeenat Banu; Saiful Kassim

doi:10.1530/endoabs.38.P427

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

SFEBES2015 Poster Presentations Steroids (49 abstracts)

Cushing’s syndrome secondary to bilateral functioning adrenocortical adenoma

Zeenat Banu & Saiful Kassim

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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
Investigations	Patients 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 Metadrenaline	0.58 (0.0–2.0 μmol/24 h)
24 h urinary Normetadrenaline	0.78 (0.0-4.3 μmol/24 h)
Plasma aldosterone concentration	71 (40–310 ng/l)
Plasma renin activity	2.8 (3–40 mU/l)
Dehyroepiandrosterone sulphate	5.8 (0.26–6.68 μmol/l)
Androstenedione	21.2 (0.0–3.5 nmol/l)

Table 2 Results of Adrenal vein sampling
Peripheral	Right adrenal vein	Left adrenal vein	A:U Ratio
Baseline cortisol	475	2559	740	3.45 (>2)
Baseline aldosterone	146	3000	999
15 min post ACTH
Cortisol	719	17 500	10 743	1.62 (<2)
Aldosterone	260	14 540	10 570
25 min post ACTH
Cortisol	826	17 500	17 500	1.0 (<2)
Aldosterone	285	13 160	14 970

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.