Endocrine Abstracts

Background: Cushing’s disease (CD) develops as a result of ACTH-secreting pituitary adenoma. Transsphenoidal selective surgery (TSS) is the first-line therapy, and success is largely determined by the experience of the surgeon. There are several reasons for the persistence of the disease, but it is almost impossible to predict such an outcome in advance, which leads to multiple interventions and has a pronounced effect on the quality of life of patients.

Clinical case: A 33-year-old female addressed the clinic with typical symptoms of hypercorticism in October 2018. From the history: in 2016, ACTH-dependent hypercortisolism was revealed, but pituitary MRI showed no pathology. It was recommended to repeat the MRI after 6 months. She returned 2 years later due to poor health, diagnosed with CD (24hr urinary cortisol – 799 nmol/day (< 400), 1 mg dexamethasone suppression test (DST): 14.20 µg/dl (< 1.8); ACTH–71 pg/ml (< 46); 8 mg dexamethasone suppression test: cortisol was suppressed from 18.67 µg/dl to 1.99 µg/dl; MRI scan: pituitary microadenoma 4 × 3.2 mm). Although treatment at an expert level institution was recommended, the patient preferred a center that doesn`t belong to the institutions with the greatest experience in treating CD, where a TSS was performed on 09/17/18. Immunohistochemically (IHC): pituitary adenoma with expression of ACTH, GH, PRL. Post-operatively, remission didn`t occur (plasma cortisol – 732 nmol/l (< 536); ACTH-76 pg/ml), secondary hypothyroidism was detected. She was offered an immediate hypophysectomy, which she refused. Ketoconazole 200 mg/d, cabergoline 0.5 mg 2 times/week, levothyroxine 50 mg/day were prescribed. After discharge, she turned to the clinic to determine further tactics. Physical examination data corresponded to CD, persistent hypercortisolism was biochemically confirmed. MRI scan: pituitary microadenoma 5 × 5, 5 × 4 mm with postoperative changes. For TSS, she was referred to an expert level center, however, on the recommendation of a neurosurgeon, the operation was postponed due to predicted inefficiency. The maximum tolerated dose of ketoconazole was selected: 600 mg/day, which didn`t allow achieving eukortisolism: plasma cortisol - 1265 nmol/l. TSS was performed after excluding ACTH-ectopic tumors according to the results of petrosal sinus sampling, post-operatively, remission didn`t occur (IHC: intensive diffuse expression of ACTH). Currently, the patient is preparing for radiation therapy.

Conclusion: Treatment of patients with CD should be carried out exclusively in an expert level center. Studies are needed aimed at determining the risk of persistent CD to optimize treatment and reduce the time of persistence of hypercortisolism, the number of hospitalizations, economic costs and improve the quality of life of patients.