Management of Cushing’s disease when surgery is a tricky option – a case report

Abstract

INTRODUCTION/OBJECTIVES: Cushing’s disease, a form of Cushing’s syndrome, is caused by excess ACTH production, a hormone that regulates cortisol production, by a benign tumor in the pituitary gland. As a result, cortisol levels are elevated, while ACTH levels are not suppressed due to autonomous secretion. The primary treatment option is surgery. Other therapeutic modalities include drug therapy, radiation, and bilateral adrenalectomy in selected cases. CASE PRESENTATION: In 2019, in a 65-year-old female patient with bilateral cortical adenoma, hypercortisolemia was detected. Further testing revealed elevated late-night salivary cortisol and insufficient cortisol suppression in Liddle’s test. ACTH was not suppressed. Initially, no pituitary tumor was detected on NMR scans. Fluconazole was used as her initial treatment to inhibit steroidogenesis, but without a proper response. Then the patient has been prescribed metyrapone. Three months after starting metyrapone, the patient began to experience side effects, including stomach pain and irregular blood pressure. After re-running pituitary tests, a microadenoma was found too small for surgery. Gamma knife radiosurgery was conducted, but one month postoperatively, cortisol levels remained high and cabergoline treatment was introduced without adequate response over time. In 2022, low– dose pasireotide eventually replaced cabergoline. The patient’s cortisol level is currently normal with clinical improvement and is being regularly checked. CONCLUSION: Cushing’s disease is a serious condition with systematic deteriorating effects. Treatment is still challenging, and there is still enough space for new treatment options that may benefit the patient. CR54 Management of metabolic crisis in three-dayold newborn Tea Kržaka, Lorena Lojea, Ivo Barićb, Dorotea Ninkovićb