Endocrine Abstracts

Adrenal cortical carcinoma (ACC) is extremely rare, the incidence is 0.5 - 2 cases per million populations per year. Most ACCs occur as sporadic tumours. It is more common in females and can occur at any age with a peak in fifth and sixth decades of life. We present a 45-year-old otherwise well and fit lady presented with headache, lethargy, and two stone rapid weight gain over a period of 6 months. She also had associated nocturia, insomnia, night sweats, hirsutism, and acne. Her initial clinical examination revealed new onset of hypertension 190/120 mmHg and spontaneous hypokalaemia required attendance to ambulatory care unit for intravenous potassium replacement. She had raised aldosterone at 1600, supressed renin with low potassium of 2.5. Her overnight dexamethasone suppression test failed to supress cortisol this was 730. Her ACTH was supressed < 3 and a 24-hour urine cortisol was high at 729 (150-350). Her testosterone was raised at 4.5 pmol and adrenal androgens were also raised. She did not have diabetes or osteoporosis, HbA1c was 41mmol/mol. Urgent adrenal imaging revealed a 15 cm large heterogenous left adrenal mass radiologically keeping in with likely ACC. Her urine steroid profile revealed the metabolites confirming the diagnosis of ACC. Overall biochemical findings were keeping in with multiple hormones producing ACC (producing glucocorticoids, mineralocorticoids and androgens). Her completion staging CT has not confirmed any distant metastasis. She was commenced on Amlodipine, Spironolactone and Ketoconazole for preoperative optimisation. Then she underwent radical left adrenalectomy, left nephrectomy, distal pancreatectomy, and splenectomy. She had a uneventful recovery and remained on hydrocortisone replacement and was commenced on Mitotane for a planned two year course. Her initial imaging 3 months after surgery was satisfactory, however her repeat PET scan in 6 months revealed PET avid lesion in adrenal bed this was thought to be incomplete clearance rather than recurrence. MDT decision was to commence on systemic EDP chemotherapy for 6 months and repeat PET to consider redo surgery. Her latest imaging following completion EDP chemotherapy revealed complete metabolic remission. She is to continue mitotane for 2 years and to continue 3 monthly imaging. She has developed a left ovarian cyst now under surveillance and primary hypothyroidism requiring Levothyroxine replacement thought to be secondary to effects of chemotherapy. She currently also remains on Hydrocortisone replacement until she completes Mitotane thereafter short synacthen test to review her adrenal reserves.