Treatment of Cushing's Syndrome in Cushing's Disease (ACTH-Dependent, Pituitary Corticotrope Adenoma)
This protocol covers Cushing's syndrome arising in the context of Cushing's disease — ACTH-dependent hypercortisolism driven by a corticotrope adenoma of the pituitary gland.
Cushing's disease is a specific cause of ACTH-dependent Cushing's syndrome in which a pituitary corticotrope tumor secretes excess ACTH, leading to bilateral adrenal overproduction of cortisol. Causes of Cushing's syndrome include intrinsic adrenal abnormalities as well as ACTH secretion from a corticotrope tumor or from an ectopic source.
The recommended first-line intervention is surgical — transsphenoidal selective adenomectomy (TSS) of the pituitary corticotrope adenoma, carried out by an experienced pituitary surgeon. Both established surgical routes have been used. The complete structured regimen, including candidacy criteria, perioperative considerations, and subsequent management steps, is available in the full protocol.
Biochemical remission is defined as morning serum cortisol below 5 µg/dL (<138 nmol/L) or urine free cortisol below 10–20 µg/d, assessed within 7 days of selective tumor resection.
References
DOI: 10.1210/jc.2015-1818- The common causes of CS include intrinsic adrenal gland abnormalities and ACTH secretion from a corticotrope tumor (CD) or from an ectopic tumor (EAS).
- We recommend transsphenoidal selective adenomectomy (TSS) by an experienced pituitary surgeon as the optimal treatment for CD in pediatric and adult patients.
- Many centers have replaced the transnasal route with an endoscopic endonasal approach, but both methods can be effective.
- Remission is generally defined as morning serum cortisol values <5 µg/dL (<138 nmol/L) or UFC <28–56 nmol/d (<10–20 µg/d) within 7 days of selective tumor resection.