Endocrine Abstracts

We present the case of a 58-year-old woman who presented 30 years ago. She was noted to have abdominal obesity, moon-face and skin bruising/thinning, as well as hypertension. Serum cortisol was elevated and she was presumed to have Cushing’s disease (although we have no record of dynamic tests or imaging from the time of her original presentation). Owing to her desire for future fertility, she underwent bilateral adrenalectomy at the age of 31 years. She received hydrocortisone replacement therapy and was lost to follow-up.

She re-presented 15 years post-surgery having developed type 2 diabetes mellitus and osteoporosis. At this time, her serum cortisol and 24-h urinary cortisol were markedly elevated (803 nmol/l and 3022 nmol respectively) with an elevated serum ACTH 277 pg/ml suggestive of recurrence. At the time, both MRI pituitary and CT scan of her chest and abdomen were reported as normal (with prior bilateral adrenalectomy noted). Results from HDDST and CRH tests were consistent with Cushing’s disease. An iodocholesterol¹³¹ radio-labelled scan showed presence of adrenal rest tissue within the liver. IPSS confirmed pituitary-origin for raised ACTH and she was commenced on ketoconazole. At the age of 54 years, 23 years following her original surgery, she was demonstrated on pituitary MRI to have developed a microadenoma. She underwent subsequent trans-sphenoidal resection. Although histology failed to show presence of any corticotrophinoma, the patient has remained in biochemical remission from her Cushing’s disease.

This case raises some important learning points:: i) The need for careful investigation of Cushing’s and a targeted approach to management;

ii) the importance of clinical and biochemical facts when faced with an inconsistent histopathology report; and

iii) although, she does not fulfil criteria for Nelson’s syndrome, the importance of close follow-up of patients with Cushing’s disease is demonstrated.