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Ectopic ACTH Management — SCE Medical Oncology MCQ

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ModerateOncological EmergenciesEctopic ACTH ManagementSCE Medical Oncology

A 55-year-old man with cancer develops Cushing syndrome secondary to ectopic ACTH production from a bronchial carcinoid. His cortisol is markedly elevated with bilateral adrenal hyperplasia. He is awaiting definitive surgery. What medical management controls the hypercortisolism?

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Correct answer: A — Metyrapone (11β-hydroxylase inhibitor) — rapidly reduces cortisol synthesis; ketoconazole and osilodrostat are alternatives; in severe cases, bilateral adrenalectomy may be required

Ectopic ACTH syndrome (from NET/carcinoid, SCLC, medullary thyroid cancer) causes severe hypercortisolism. Medical management while awaiting definitive tumour treatment: (1) metyrapone (first-line in UK — 11β-hydroxylase inhibitor, rapid onset), (2) ketoconazole (inhibits multiple steroidogenic enzymes — liver monitoring required), (3) osilodrostat (newer, potent 11β-hydroxylase inhibitor), (4) mifepristone (glucocorticoid receptor antagonist — does not reduce cortisol levels but blocks effects). For life-threatening hypercortisolism refractory to medical therapy, emergency bilateral adrenalectomy may be needed.

Reference: NICE; BNF Metyrapone; Endocrine Society Cushing Guidelines; ESMO Oncological Emergencies