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Enzym- und Hormonstudien bei einem virilisierenden Nebennierenrindentumor

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Zusammenfassung

Die Biogenese und der Stoffwechsel von C21- und C19-Steroiden in einem virilisierenden Nebennierenrindentumor, der von einer 69jährigen Frau stammte, wurde mit Hilfe von Inkubationsversuchen radioaktiv markierter Steroide untersucht. Die 17β-Hydroxysteroid-Oxydoreduktase in Schnitten des Tumorgewebes zeigte eine stark erhöhte Aktivität. Die Δ5-3β-Hydroxysteroid-Oxydoreduktase war unter physiologischen Bedingungen (pH 7,4) nicht nachweisbar; bei pH-Werten im stark alkalischen Bereich (pH 9,0) und hohen Cofaktor-Konzentrationen fand sich eine geringe Aktivität dieses Enzyms. Die Aktivität der 11β-Hydroxylase war ebenfalls stark vermindert: Aus Progesteron und 17α-Hydroxyprogesteron entstanden Cortisol und Cortison nur in sehr geringem Umfange. Aus Testosteron und Δ4-Androsten-3.17-dion wurden keine entsprechenden 11β-Hydroxyverbindungen gebildet. Die Aktivität der 21-Hydroxylase war nicht beeinträchtigt.

Die Ausscheidung der fraktionierten 17-Ketosteroide, der 17-Hydroxycorticosteroide, von Pregnandiol und Pregnantriol sowie der Oestrogene (Oestron, Oestradiol-17β und Oestriol) war vor der Operation in unterschiedlichem Ausmaß erhöht. Die Gesamt-17-Ketosteroid-Ausscheidung betrug dabei im Mittel 16,8 mg/24 Std; davon entfielen etwa 50% auf Dehydroepiandrosteron. Nach der Operation fiel die Steroidausscheidung im Urin zu altersentsprechenden Werten ab.

Die biochemischen Befunde werden mit den Angaben in der Literatur verglichen; die Probleme der histologischen Diagnose und der Prognose nicht metastasierender virilisierender Nebennierenrindentumore werden kurz erörtert.

Summary

The biogenesis and the metabolism of C21- and C19-steroids were studied in tissue slices of a virilising adrenal tumour of a 69 year old woman, using radioactive labelled steroids. The activity of the 17β-hydroxysteroid oxidoreductase was markedly enhanced. No Δ5-3β-hydroxysteroid oxidoreductase was found under physiological conditions (pH 7.4); however, at alkaline pH values (pH 9.0) and high concentrations of the appropriate cofactor, little activity of the enzyme could be detected. The activity of the 11β-hydroxylase in tumour slices was very low; only small amounts of cortisol and cortisone were formed from progesterone and 17α-hydroxyprogesterone. Neither testosterone nor Δ4-androsten-3.17-dione were hydroxylated at position 11β. The activity of the 21-hydroxylase was not altered.

The urinary excretion of the fractionated 17-oxosteroids, the 17-hydroxycorticosteroids, pregnandiol, pregnantriol and the oestrogens (oestrone, oestradiol-17β and oestriol) was elevated before operation. The mean value of the total excretion of 17-oxosteroids amounted to 16.8 mg/24h; about 50% of this was dehydroepiandrosterone. After operation, the excretion of steroids in urine fell to normal values.

The results of the biochemical studies are compared with those of the literature; the problems connected with the histological diagnosis and the prognosis of virilising adrenal tumours are discussed.

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  1. Chirurgische Universitätsklinik und -Poliklinik Bonn, Germany

    K. Dahm, J. M. Bayer & H. Breuer

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  2. J. M. Bayer
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Dahm, K., Bayer, J.M. & Breuer, H. Enzym- und Hormonstudien bei einem virilisierenden Nebennierenrindentumor. Klin Wochenschr 46, 520–526 (1968). https://doi.org/10.1007/BF01747770

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