[Cushing's syndrome in CRF-producing mediastinal carcinoid].
A 36-year-old patient developed marked pigmentation, marked myopathy and severe hypokalaemic alkalosis which at first pointed towards an ectopic ACTH syndrome. The dexamethasone test at a high dose indicated cortisol suppression. A mediastinal tumour was seen radiologically, but the sella was of normal size. Computed tomography provided indirect signs of a sellar space-occupying lesion which suggested an ectopic production of corticotropin-releasing factor (CRF) as cause of the Cushing's syndrome. CRF concentration in antecubital venous blood was markedly elevated to 280 ng/l. The mediastinal tumour was excised and proved to be a carcinoid histologically. Postoperatively the CRF concentration fell to 70 ng/l. An extract of the carcinoid contained 15.5 ng/g wet-weight of CRF and 254 ng/g wet-weight of beta-endorphin. The patient died 5 weeks postoperatively of sepsis with bilateral pneumonia. At autopsy the hypophysis was of normal size but showed nodular ACTH-cell hyperplasia. This was thus a case of Cushing's syndrome resulting from ectopic CRF production in a mediastinal carcinoid tumour.
Gerl
H
H
Klinik für Innere Medizin Theodor Brugsch, Humboldt-Universität, Berlin.
Knappe
G
G
Rohde
W
W
Stahl
F
F
Wolff
H
H
Martin
H
H
D000328:Adult; D002276:Carcinoid Tumor / Q000150:complications* / Q000378:metabolism / Q000473:pathology / Q000601:surgery; D003346:Corticotropin-Releasing Hormone / Q000097:blood / Q000378:metabolism*; D003480:Cushing Syndrome / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D006801:Humans; D008297:Male; D008479:Mediastinal Neoplasms / Q000150:complications* / Q000378:metabolism / Q000473:pathology / Q000601:surgery; D008482:Mediastinum / Q000473:pathology / Q000601:surgery
D003346:Corticotropin-Releasing Hormone
Cushing-Syndrom bei CRF-produzierendem mediastinalem Karzinoid.