SFEBES2025 Poster Presentations Late Breaking (68 abstracts)
1Kings College Hospital, London, United Kingdom. 2Guys and St. Thomass Hospital, London, United Kingdom
*Beatrice Ranasinghe and Nirali Desai are joint first authors of this work 52 male presented with a 2 year history of easy bruising, hypertension, sleep apnoea, and poorly controlled type 2 diabetes (HbA1c of 125mmol/L). He had a cushingoid appearance, with central obesity, striae, proximal myopathy, and easy bruising. BMI of 29.7kg/m2 and blood pressure 163/107mmHg.
Results and treatment: Biochemical testing showed raised cortisol which does not suppress with dexamethasone. Low but detectable ACTH and plasma metanephrine raised >x4 ULN. Urine steroid profile did not demonstrate any common markers of adrenocortical carcinoma. CT adrenal - left sided 41mm adrenal lesion. 41HU pre-contrast with an absolute washout of 52%, atypical for adrenal adenoma FDG PET - increased metabolic activity in left adrenal mass (SUVmax 5.8) Pre-operative low dose metyrapone (250mg qds) was commenced which led to a pronounced response, cortisol reduced to mean 73nmol/l. Laparoscopic adrenalectomy was performed without complication and he was commenced on oral hydrocortisone. Post-operative biochemistry showed normal plasma metadrenaline and full suppression of cortisol on ONDST. Post operatively his HbA1c improved to 41 and he is no longer on insulin. Histology demonstrated a well-circumscribed nodule (36mm), comprising haphazardly arranged, adrenalcortical elements (positive for synaptophysin Melan A and calretinin) and medullary elements (positive for chromogranin and Gata 3). This is diagnostic of a mixed corticomedullary tumour (MCMT) of the adrenal gland
Discussion: This case illustrates clinical Cushings syndrome, with some paradoxical biochemical findings, caused by a mixed corticomedullary tumour, composed of separate populations of cortical and medullary cells that simultaneously produce adrenocortical hormones and catecholamines. These tumours are extremely rare, and to date approximately 30 cases have been reported.
Result | Reference range | |
Random cortisols | 606 and 428nmol/l | n/a |
ONDST | 735nmol/l | <50nmol/L |
Low dose dexamethasone suppression test | 551nmol/l | n/a |
ACTH | 11ng/L and 9ng/L | 0-30ng/L |
Plasma metanephrine | 2398 | 0-509pmol/L |
Plasma normetanephrine | 1739 | 0-1179pmol/L |
3-methoxytyramine | <157 | 0-179pmol/L |
DHEA-S | 4.6μmol/L | 2.2 - 15.2μmol/L |