Introduction: Cushings disease (CD) is five to six times more common than Cushings syndrome, with a peak incidence at 2040 years. It is usually an ACTH-secreting microadenoma, the local invasion being associated with one third of macroadenomas. The treatment of choice is the transsphenoidal surgery, but nearly 50% of the patients with macroadenomas ultimately require additional treatment. Cabergoline appears to be an attractive treatment option for CD, considering its efficacy comparable with that of ketoconazole, pasireotide, radiotherapy and repeat surgery.
Case report: We describe the case of a 63 years-old female patient whose case was presented in the early evolution of her Cushings disease in 2016. She was diagnosed with an ACTH-secreting invasive pituitary macroadenoma in 2015, based on the typical clinical and hormonal signs of hypercortisolism. After transsphenoidal surgery and Gamma-knife radiation in 2016, her urinary free cortisol and ACTH were still elevated, so Pasireotide was initiated but, despite her excellent response to this therapy, Pasireotide was discontinued after 2 months due to side effects. Since then, the hypercortisolism reoccurred in 2018, and considering the surgical and radiological contraindications, she was started on Cabergoline 1 mg/week. After 3 months she developed corticotropic insufficiency in need of substitution, which was interpreted at that time to be more likely the result of stereotactic radiosurgery. Unfortunately, after another 3 months, the patient was hypercortisolemic again and restarted Cabergoline, this time at a lower dose 0.5 mg/week, which seems, at the moment, to control the hypersecretion, without inducing insufficiency.
Conclusion: Our patient had a variable pattern of hypercortisolism which was difficult to control until the effect of Gamma-knife, because of the side effects to Pasireotide and the lack of medical therapy. As the latest studies reveal, Cabergoline is an attractive treatment option via its inhibitory effect on Dopamine 2 receptors in CD and the strongest predictors for response are previous surgery, prolonged duration of cabergoline therapy (at least 6 months) and lower doses (2 mg/week).
Keywords: Cushing disease, Cabergoline
18 - 21 May 2019
European Society of Endocrinology