Treatment of Ectopic ACTH Syndrome: Surgical Management and Remission Goals
Ectopic ACTH syndrome requires targeted management of the responsible tumor. Evidence-based protocols define both the operative approach and the cortisol thresholds that confirm successful remission.
Treatment Approach (Partial Overview)
The primary intervention involves a surgical approach directed at the ectopic ACTH-secreting tumor, with lymph node evaluation carried out as clinically indicated.
Complete sequencing, eligibility criteria, and additional management options are in the full protocol →
Remission Targets
Remission is assessed within 7 days of tumor resection. Criteria: morning serum cortisol
< 5 µg/dL (<138 nmol/L)
or urine free cortisol
< 28–56 nmol/d (<10–20 µg/d).
References
DOI: 10.1210/jc.2015-1818
- We recommend localizing and resecting ectopic ACTH-secreting tumors with node dissection as appropriate.
- We recommend initial resection of primary lesion(s) underlying CD, ectopic and adrenal (cancer, adenoma, and bilateral disease) etiologies, unless surgery is not possible or unlikely to significantly reduce glucocorticoid excess.
- 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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