Sixty seven years old lady was admitted with profound weakness and weight loss for 34 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 793158 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.
|Investigations||Patients values (normal values)|
|24 h urinary free cortisol (1st)||429 (50300 nmol/24 h)|
|24 h urinary free cortisol (2nd)||306 (50300 nmol/24 h)|
|Plasma ACTH (1st)||6 (0.040 ng/l)|
|Plasma ACTH (2nd)||<5 (0.040 ng/l)|
|24 h urinary Metadrenaline||0.58 (0.02.0 μmol/24 h)|
|24 h urinary Normetadrenaline||0.78 (0.0-4.3 μmol/24 h)|
|Plasma aldosterone concentration||71 (40310 ng/l)|
|Plasma renin activity||2.8 (340 mU/l)|
|Dehyroepiandrosterone sulphate||5.8 (0.266.68 μmol/l)|
|Androstenedione||21.2 (0.03.5 nmol/l)|
|Peripheral||Right adrenal vein||Left adrenal vein||A:U Ratio|
|Baseline cortisol||475||2559||740||3.45 (>2)|
|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.