Introduction: Pancreatic ACTHomas have a poor prognosis with severe and rapidly progressive clinical courses, influenced by hypercortisolaemia thus whenever possible, control of the cortisol levels should be obtained to reduce complications. We report the use of adrenal embolisation in a case of a life threatening paraneoplastic Cushing.
Case: A 51-year-old woman with a negative past medical history complained of general weakness. Cushings syndrome was suspected on the basis of hypokaliaemia, new onset hyperglucaemia and metabolic alkalosis found in the laboratory tests run on admission. She had no Cushingoid features except mild plethora. The suspicion was confirmed by high levels of urinary free cortisol, serum cortisol and ACTH, a lack of circadian rhythm of serum cortisol, and a low (1 mg) and high dose (8 mg) overnight of dexamethasone tests that failed to suppress. CT and whole body PET scan showed 2×3 cm mass in the pancreas tail. She underwent the excision of the pancreatic mass and pathology confirmed the neuroendocrine nature of the tumour. Cushingoid features were more obvious and liver metastases were observed so treatment with ketokonazol was started, initially decreasing cortisol levels, but refractory hypercortisolemia recurred although dose was increased and metopirone added. The patient became psychotic so mifepristone was commenced, but she continued to deteriorate. It was therefore decided that an attempt should be made for bilateral adrenalectomy, but the patient was unfit for surgery so a bilateral adrenal embolectomy was performed. A week after the procedure her condition improved and the urinary cortisol levels lowered. Unfortunately the patient died soon after due to a multiorganic failure.
Conclusions: Aggressive attitudes to control cortisol levels are necessary to reduce comorbidities in ectopic Cushing. This case shows that adrenal embolectomy might be of some use when the patient has become unfit for surgery and in whom medical therapy has failed.
16 - 20 May 2015
European Society of Endocrinology