Table 6.1-1. Overview of treatments in Cushing syndrome

Treatment

Adrenal etiology Pituitary etiology Ectopic ACTH secretion

Surgical

– Adenoma/carcinoma: tumor resection

– Macronodular or micronodular adrenal hyperplasia: resection of largest adrenal gland 

– Resection of pituitary tumor

– Bilateral adrenalectomy in selected cases

– Resection of primary tumor

– Tumor debulking

– Bilateral adrenalectomy in selected cases

Medical

– Steroidogenesis inhibitors (ketoconazole, metyrapone, osilodrostat, mitotane, etomidate)

– Glucocorticoid receptor antagonists (mifepristone)

– Chemotherapy/mitotane for adrenal carcinoma

– Second-generation somatostatin analogues (pasireotide)

– Dopamine agonists (cabergoline)

– Steroidogenesis inhibitors (ketoconazole, metyrapone, osilodrostat, mitotane, etomidate)

– Glucocorticoid receptor antagonists (mifepristone)

– Steroidogenesis inhibitors (ketoconazole, metyrapone, osilodrostat, mitotane, etomidate)

– Glucocorticoid receptor antagonists (mifepristone)

– Somatostatin analogues (octreotide/lanreotide)

– Everolimus

– Tyrosine kinase inhibitors

– Chemotherapy

Radiotherapy

External-beam radiation therapy for adrenal carcinoma

Conventional versus stereotactic fractionated radiotherapy

External-beam radiation therapy

Other specialized therapies

Not applicable

Not applicable

– Hepatic artery embolization

– Radiofrequency ablation

– Peptide-receptor radiolabeled therapy

ACTH, adrenocorticotropic hormone.