MEN 1 with adrenal Cushing's (a rare association)
K O Shaafi, S Russell & S Roberts
A 36-year-old gentleman presented to his GP 2 years ago with hypertension and was commenced on valsartan. In January 2008, he was noted to have mildly deranged LFT and abdominal ultrasound scan showed mild fatty liver infiltration and bilateral renal stones. He was referred to Urology for investigation of renal stones and a CT IVU showed multiple renal calculi, a 6×5×5 cm soft tissue enhancing lesion arising from the right adrenal gland and a 7 mm calcified density within the body of the pancreas of benign appearance. Adrenal protocol CT confirmed the adrenal lesion. At this stage he was referred to the endocrine team for assessment of the adrenal mass. On examination he had a plethoric facial appearance, centripedal obesity and gynaecomastia. Twenty-four hours urine collections for catecholamines and cortisol were normal on two occasions. An overnight dexamethasone suppression test and the low dose dexamethasone suppression tests were both positive, with suppressed basal ACTH. Routine bloods revealed elevated calcium 3.07 mmol/l with elevated PTH 16.3 pmol/l. Baseline pituitary profile revealed elevated prolactin 709 mU/l and normal gonadotrophins LH 2.3 IU/l, FSH 1.7 IU/l, testosterone 13.3 nmol/l.
He underwent laparoscopic right adrenalectomy. Histopathology confirmed cortisol producing adrenal adenoma. Since removal of the adrenal adenoma he has remained off antihypertensive medication. Postoperatively the cortisol level suppressed on low dose dexamethasone suppression test.
MRI pituitary showed 7 mm pituitary microadenoma and sesta Mibi parathyroid scan revealed increased uptake in the left inferior position suggestive of parathyroid adenoma, but there was also delayed uptake in the other three parathyroid glands. He is due for parathyroid surgery and he is likely to undergo four gland removal. We are currently in the process of investigating whether he has a pancreatic tumour with fasting gut hormones screen.
On review of the literature there are few reported cases of MEN I with concurrent adrenal adenoma producing cortisol. Forty percent of MEN cases are associated with adrenal nodular hyperplasia with no endocrinopathy. Therefore we would like to submit this interesting case for presentation at The Third Hammersmith Multidisciplinary Symposium.