SAT-484 - Bridging the Cortisol Crisis: Use of Etomidate and Steroidogenesis Inhibitors in Metastatic Adrenocortical Carcinoma

Adrenocortical carcinoma is a rare and aggressive malignancy that may present with severe hypercortisolism and rapid clinical deterioration. We report the case of a 64-year-old woman with a history of severe obesity status post Roux-en-Y gastric bypass, who was initially followed by endocrinology for osteoporosis. During preoperative evaluation for lumbar spine fusion, imaging revealed bilateral adrenal nodules measuring 2.8 × 2.5 cm on the left and 2.9 × 2.2 cm on the right on computed tomography.
Four months later, abdominal magnetic resonance imaging demonstrated interval doubling in size of the left adrenal lesion. Radiographic assessment suggested possible metastatic disease rather than primary adrenal malignancy, and biopsy was recommended. Following negative metanephrine testing, fine-needle aspiration confirmed adrenocortical carcinoma. Biochemical evaluation showed two abnormal dexamethasone suppression tests 4.1 and 3.5 µg/dL( normal < 1.8 µg/dL) and suppressed ACTH < 5 pg/mL.(normal 6-50pcg/ml)
The patient underwent left adrenalectomy, which revealed a 7.5 cm high-grade adrenocortical carcinoma. Postoperatively, she was found to have multiple liver and bone metastases. Although she initially declined chemotherapy, she later agreed to initiate mitotane; however, treatment was discontinued after several days due to severe hypokalemia and diarrhea.
Subsequent evaluation demonstrated severe hypercortisolism, with a 24-hour urinary free cortisol of 2953 µg (normal 4–50 µg), morning cortisol of 56 µg/dL, and suppressed ACTH. Her course was complicated by refractory hypokalemia (2.2–2.6 mEq/L) and uncontrolled hypertension despite aggressive supplementation, requiring initiation of high-dose spironolactone and amiloride. Continuous etomidate infusion was started for rapid cortisol suppression to stabilize electrolytes and allow initiation of chemotherapy with doxorubicin and etoposide.
She was later transitioned to levoketoconazole after four days of etomidate therapy; however, drug interaction with etoposide led to severe bone marrow suppression and pancytopenia, necessitating a switch to Osilodrostat. Despite aggressive medical management, her hospital course was complicated by persistent myelosuppression, hypotension, and progressive respiratory failure. Given continued clinical decline, the patient elected to transition to hospice care.
This case highlights the challenges of managing severe hypercortisolism in metastatic adrenocortical carcinoma, the importance of rapid cortisol control using both intravenous and oral steroidogenesis inhibitors, and the significant morbidity associated with advanced disease despite multimodal therapy. Early recognition and multidisciplinary management are critical to optimizing outcomes in this life-threatening condition.

*Unless otherwise noted, all abstracts presented at ENDO must not be released to the press or the public until the date and time of presentation. For oral presentations, the abstracts are embargoed until the session begins. The Endocrine Society reserves the right to lift the embargo on specific abstracts that are selected for promotion prior to or during ENDO.*