Endocrine Abstracts

Introduction: Ectopic ACTH syndrome is rare but is frequently severe condition because of the intensity of the hypercortisolism that may be dissociated from the tumoral condition. It should often be considered as an endocrine emergency requiring an emergency response both in terms of diagnostic procedures and therapeutic interventions. Patient management is complex and necessitates dual skills, in the diagnosis and treatment (1). Etomidate, an imidazole-derivative anesthetic agent, blocks 11-beta-hydroxylase. It is used intravenously at 0.3 mg/kg/h. Its use is limited by the requirement for administration by the intravenous route. However, it rapidly decreases cortisol concentration and may be used as an adjunct to be impending surgical procedure (2). We are presenting case of Cushing crisis presented with severe hypercortisolism treated with etomidate infusion, secondary to neuroendocrine tumor.

Case summary: 43-year-old lady with previous 47 mm right adrenal incidentaloma for which underwent right adrenalectomy in November 2020, histology reported as Adrenal oncocytoma. In September 2021 developed symptoms of lethargy and weight gain, seen in December 2021 found to be in florid Cushing, failed to suppress on low dose dexamethasone suppression test with ACTH of 583 ng/l (7.2-63.3), 9am Cortisol of 1484 nmol/l and 24-hour Urinary Free Cortisol of 14945 unit nmol (0-125 Unit nmol/24hr) with severe hypokalemia K 2.9mmol and high blood pressure. Started on Metyrapone 250mg BD titrated to 1g TDS and Spironolactone 100 mg OD and intravenous potassium. Cortisol persistently remained>1000 nmol/l for which started Etomidate infusion (0.04-0.05 mg/kg/hr) to control Hypercortisolism. a full endogenous blockade of achieved cortisol<300 nmol/l, started on block and replace regimen with Hydrocortisone. Cortisol measured 6 hourly whiles on etomidate infusion rate adjusted based on serial cortisol levels. Further images obtained PET CT scan revealed multiple liver lesions, normal pituitary MRI. CT Pancreas showed 24 mm soft tissue nodule at the lateral pancreatic head and bilobed 18 mm nodule at the medial aspect of the uncinate process. Liver biopsy showed well differentiated neuroendocrine tumor, appearance and immunohistochemical profile do not fit with metastatic spread of the previous adrenal tumor. Pancreatic endoscopic US guided biopsy showed well differentiated neuroendocrine tumor of intermediate grade (NET G2,2019 WHO). Discussed at the NET MDT decision of Adrenalectomy has been made. Elective Robotic left adrenalectomy in January 2022, good postoperative recovery, discharged home on Hydrocortisone replacement with plans to be seen in Oncology outpatient clinic to start chemotherapy treatment.