A 68-year-old woman presented with hypertension, oedema, weight gain and moon face. She had signs of proximal muscle weakness, thinning of scalp hair and bruising on her legs. Initial tests showed 24 hour urine free cortisol elevated at 946 nmol (0300 nmol).
Serum cortisol showed no circadian fluctuation (0900: 1094 nmol/l, 2400 hrs: 886 nmol/l) and no suppression by dexamethasone (2 mg/24 h for 48 h: 1067 nmol/l). ACTH levels were slightly raised (99pg/ml), indicating ACTH-dependent Cushings syndrome.
While awaiting IPSS, she was admitted from the clinic with abdominal pain and chest infection, and treated with IV fluids and antibiotics. During the admission she underwent inferior petrosal sinus sampling and no central:peripheral gradient was found in ACTH levels, with no response to CRH. MR scan showed a partially empty pituitary fossa with an intrasellar cyst. During her stay she developed severe abdominal pain and back pain and was found to have pubic ramus and vertebral fractures. She rapidly deteriorated, developing septic shock from her chest infection and required ventilation and inotropic support. She developed multi-organ failure and cerebral infarct and died, 3 weeks after her admission, on the ITU.
Post mortem showed a large left adrenal gland mass weighing 506 grams suggesting adrenocortical carcinoma and an atrophic right adrenal gland and pituitary gland. Histology confirmed the diagnosis.
This patient had developed Cushings syndrome which followed an aggressive course. Investigations suggested that this was ACTH dependent, with an ectopic source of ACTH, but the patient proved to have adrenal carcinoma. Whether there was ACTH secretion from the adrenal carcinoma causing detectable ACTH levels remains to be confirmed.
01 - 05 Apr 2006
European Society of Endocrinology