[Ectopic ACTH secretion: a heterogeneous entity].
OBJECTIVE
ACTH-secreting non-pituitary tumors are a rare cause of Cushing's disease. We report the clinical course, prognostic aspects and molecular analysis data in three patients for whom the diagnosis was confirmed but who had variable clinical features and laboratory results.


METHODS
Patient n degree 1 had severe hypercorticism which rapidly progressed to death 13 months after diagnosis. In patient n degree 2, signs of hypercorticism severe, leading to death 5 years after discovery of the causal carcinoid tumor. Patient n degree 3 had moderate hypercorticism and has survived for more than 25 years.


CONCLUSIONS
These 3 ectopic tumors are representative examples of a wide range of possible ACTH-secreting ectopic tumors. In highly malignant poorly-differentiated tumors such as small-cell anaplastic carcinomas, ACTH production is aberrant and poorly controlled, and thus not particularly effective. At the other extreme, typical benign bronchial carcinomas have a high degree of neuroendocrine differentiation and secrete ACTH in a well-controlled manner difficult to distinguish from corticotropic adenomas, further exaggerating the diagnostic pitfalls.
Ankotche
A
A
Clinique des Maladies endocriniennes, Hôpital Cochin, Paris.
Raffin-Sanson
M L
ML
Mosnier-Pudard
H
H
Bertagna
X
X
Luton
J P
JP
D000182:ACTH Syndrome, Ectopic / Q000097:blood / Q000235:genetics / Q000503:physiopathology*; D000308:Adrenocortical Hyperfunction / Q000209:etiology; D000324:Adrenocorticotropic Hormone / Q000378:metabolism; D000328:Adult; D001984:Bronchial Neoplasms / Q000175:diagnosis / Q000378:metabolism*; D002276:Carcinoid Tumor / Q000175:diagnosis / Q000378:metabolism*; D018288:Carcinoma, Small Cell / Q000175:diagnosis / Q000378:metabolism*; D003480:Cushing Syndrome / Q000175:diagnosis; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000175:diagnosis / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D011333:Pro-Opiomelanocortin / Q000235:genetics / Q000378:metabolism
D011333:Pro-Opiomelanocortin; D000324:Adrenocorticotropic Hormone