Ectopic ACTH syndrome can be cured by destroying all ACTH cancer tissue.

If corticorelin injection leads to little or no response in plasma levels of ACTH or cortisol, a diagnosis of Ectopic ACTH syndrome is confirmed.

Cushing's disease is not to be confused with Ectopic Cushing syndrome (a.k.a. Ectopic ACTH syndrome), which is often seen in Paraneoplastic syndrome.

AVPR1B is expressed at high levels in ACTH-secreting pituitary adenomas as well as in bronchial carcinoids responsible for the ectopic ACTH syndrome.