Endocrine Abstracts

Introduction: A transsphenoidal adenectomy (TSFE) is primary treatment of Cushing’s disease. Pharmacological management when the disease persists after surgery is a challenge.

Case report: A 47-year-old man was initially diagnosed with CD (petrosal sinus sampling with CRH testing included) after suffering from multiple organ failure in sepsis. Microadenoma 2×4 mm was suspected on the pituitary MRI. Clinical symptoms were markedly improved after first TSFE, but normalisation was not achieved, so he underwent reoperation. No adenoma was present on control MRI, but CD remained active – altered diurnal rhythm, morning cortisol 574 nmol/l, midnight cortisol 555 nmol/l, urinary free cortisol (UFC) 1008 nmol/24 h, altered suppression in suppression tests (111 nmol/l), and ACTH 115 pg/ml. Ketoconazole was no longer available so treatment with cabergoline according to national guidelines was started. Because no effect was observed we started treatment with pasireotide 0.6 mg b.i.d. In 2 months this treatment led to normalization of UFC (208 nmol/24 h), plasma cortisol in normal range, ACTH 49 pg/ml. Mild elevation of HbA1c was observed (from 5.6 to 8.0…6.6…6.3), but diabetes was under control with oral antihyperglycaemic agents. Patient was doing well, reporting better quality of life and physical condition. Unfortunately 16 months after pasireotide introduction he was admitted for diabetes decompensation (glycaemia 37 mmol/l) and bad condition for symptomatic CD (plasma cortisol >2000 mmol/l, ACTH 321 pg/ml, and UFC 12240 nmol/24 h). Pasireotide was discontinued, treatment with insulin was started. For fast deteriorating of his clinical status due to active CD he was indicated to laparoscopic bilateral adrenalectomy as ‘ultimum refugium’. The patient is now dispensarised for eventual Nelson’s syndrome.

Conclusion: Mild hyperglycaemia at the start of pasireotide is not the reason for discontinuation. According to our best knowledge this is the first case reported of sudden lack of efficacy of pasireotide after long-term effective treatment.

Disclosure: Supported by MH CZ – DRO (UHHK, 00179906) and the programme PRVOUK P37/08.