Cushing-Syndrom bei CRF-produzierendem mediastinalem Karzinoid

Autor: H. Gerl, H. Martin, G. Knappe, F. Stahl, W. Rohde, H. Wolff
Rok vydání: 2008
Předmět:
Zdroj: DMW - Deutsche Medizinische Wochenschrift. 115:332-336
ISSN: 1439-4413
0012-0472
DOI: 10.1055/s-2008-1065011
Popis: 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.
Databáze: OpenAIRE