Difficulties in Management of Hypercortisolism

Dr FW Ahmed, Dr D Sennik, Dr J Wright and Professor D Russell-Jones

Royal Surrey County Hospital.

Abstract: 50 year old lady first came to our clinic in 2006 for the assessment of 3.4cms adrenal incidentaloma.  She had complaints of abdominal distension/obesity, proximal muscle weakness, tiredness, bruising, thinning of the skins and hirsutism. These symptoms started few years earlier. She also had osteopenia of her left hip with stress fractures in her feet and rib fracture following a fall. Her other medical problems includes tonsillectomy, hiatus hernia, ovarian cyst, spinal fusion, macular degeneration, glaucoma, diverticulosis and pacemaker for sick sinus syndrome.


On examination her BMI was 30.9kg/m2 and her weight was more centrally distributed. She had bruising, thinning of skin and buffalo hump.


Her cortisol level did not suppress after LDDST. Her ACTH level was 49ng/L. CRH test showed a 20% increase in cortisol (968nmol/L to 1427nmol/L) and 50% increase in ACTH (52ng/L to 117ng/L) respectively. However her pituitary CT scan was normal. It was decided in multidisciplinary meeting to proceed with left adrenalectomy. Histology showed left adrenal adenoma.


Following surgery in 2006 her symptoms improved. Investigations 6 months after surgery also normalised; cortisol level of <30 nmol/L following LDDST.


She represented in 2010 with symptoms of abdominal distension, proximal weakness and changes in facial appearance. Her investigations are again suggestive of ACTH dependent Cushing’s. She was referred for petrosal sinus sampling. Petrosal sinus sampling showed a basal IPS: P ratio ³ 2.0 indicating a pituitary source. A CRH stimulated ratio was also ³ 3.0. Interpretation for lateralization was difficult.


CT abdomen showed 10mm nodule within the body of the right adrenal gland.


For further management her case was discussed in our multidisciplinary meeting with Hammersmith Hospital. It was decided to perform CT head with pituitary reconstruction before any surgical option is considered.   Patient is very distressed and keen to undergo right adrenalectomy.

We would like to discuss following points:

What is the best surgical option (transsphenoidal pituitary surgery vs adrenalectomy) in light of limited pituitary imaging?

If patient undergoes adrenalectomy what is the risk of Nelson syndrome? How this patient should be followed up as MRI pituitary is not possible?