A phaeochromocytoma presenting with Cushing's syndrome associated with increased concentrations of circulating corticotrophin-releasing factor.
The case is described of a 61-year-old male who presented with hypertension and Cushing's syndrome which resolved on excision of a unilateral adrenal mass. Histology of the tumour revealed a benign phaeochromocytoma which immunostained for corticotrophin-releasing factor (CRF-41) but not for ACTH. Preoperative plasma concentrations of immunoreactive CRF-41 were increased, and gradients for both CRF-41 and ACTH were demonstrated across the tumour. Post-operatively, CRF-41 was undetectable in plasma. The tumour contained high concentrations of immunoreactive CRF-41 which co-eluted with synthetic human CRF-41 on reversed-phase high-performance liquid chromatography. Tumour CRF-41 stimulated the release of ACTH in a dose-dependent manner from isolated rat anterior pituitary cells. We conclude that this tumour secreted CRF-41 and ACTH and had the capacity to produce ACTH-dependent Cushing's syndrome directly by secreting ACTH and indirectly by secreting CRF-41 to stimulate ACTH secretion from the anterior pituitary.
D000310:Adrenal Gland Neoplasms / Q000097:blood / Q000150:complications*; D000324:Adrenocorticotropic Hormone / Q000097:blood; D003346:Corticotropin-Releasing Hormone / Q000097:blood*; D003480:Cushing Syndrome / Q000209:etiology*; D006801:Humans; D006973:Hypertension / Q000209:etiology; D008297:Male; D008875:Middle Aged; D010673:Pheochromocytoma / Q000097:blood / Q000150:complications*
D000324:Adrenocorticotropic Hormone; D003346:Corticotropin-Releasing Hormone