Treatment of Cushing's Syndrome Caused by an Ectopic ACTH-Secreting Tumor
Clinical scenario
This protocol addresses ACTH-dependent Cushing's syndrome arising from an ectopic ACTH-secreting tumor — a recognised cause of Cushing's syndrome alongside intrinsic adrenal abnormalities and corticotrope (pituitary) tumors.
Treatment approach (partial)
The primary intervention centres on localization of the ectopic ACTH source followed by a surgical procedure targeting that tumor. The complete approach, including further steps and procedural details, is in the full structured protocol.
Remission targets
- Morning serum cortisol < 5 µg/dL (< 138 nmol/L) within 7 days of tumor resection
- Urine free cortisol < 10–20 µg/d within 7 days of 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 localizing and resecting ectopic ACTH-secreting tumors with node dissection as appropriate.
- 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.
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