PMID: 7016888Jan 1, 1981Paper

Autonomous cortisol secretion by a metastatic Leydig cell carcinoma associated with Klinefelter's syndrome

Journal of Cancer Research and Clinical Oncology
K KnyrimC G Schmidt

Abstract

We present the case of a 39-year-old man with Klinefelter's syndrome and a metastatic Leydig cell carcinoma in whom autonomous cortisol production induced by the interstitial cell tumor was found. Apart from the Cushing's syndrome the endocrine activity of the tumor was demonstrated by the secretion of estradiol, estrone, alkaline phosphatase, and testosterone. This is, to our knowledge, the first description of a Cushing's syndrome not caused via ACTH production but directly induced by ectopic steroid production. While being resistent to chemotherapy and radiation, the tumor responded favorably to treatment with o,p'-DDD. The reduction of tumor size was accompanied by a continuous decrease of serum markers. The etiology of the tumor is discussed in the light of the hormonal derangement caused by the genetic abnormality of Klinefelter's syndrome.

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Citations

Jan 1, 1988·Urology·P R CarrollR S Chaganti
Oct 23, 2008·Nature Clinical Practice. Endocrinology & Metabolism·Shilpa H JainRobert G Dluhy
Sep 1, 1990·The Journal of Clinical Investigation·Y de KeyzerX Bertagna
Jul 15, 2015·Reproductive Biology and Endocrinology : RB&E·Salwan MaqdasyIgor Tauveron
Feb 10, 2017·European Journal of Endocrinology·Johanne Marie HolstJens Otto L Jørgensen
Oct 2, 2020·Hormones : International Journal of Endocrinology and Metabolism·Alexsandra P RojasAlan N Peiris
Jan 1, 1994·The Journal of Urology·J BanielR S Foster

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