Zusammenfassung
Bei einem Knaben mit einer Pseudopubertas praecox wurde ein androgenproduzierender Hodentumor entfernt. Präoperativ war die Harnausscheidung der gesamten 17-Ketosteroide, von Androsteron und Ätiocholanolon, der 11-Oxy-17-Ketosteroide, insbesondere von 11β-Hydroxy-androsteron, von Pregnandiol, Pregnantriol sowie der Oestrogene abnorm erhöht und fiel nach der Operation auf Normwerte ab. Nach Inkubation von Glucose allein mit Tumorschnitten wurden 11β-Hydroxy-Δ 4-androstendion undΔ 4-Androstendion gefunden, nach Inkubation vonΔ 4-Androstendion: 11β-Hydroxy-Δ 4-androstendion, Testoseron und Adrenosteron, jedoch keine Oestrogene, nach Inkubation von Desoxycorticosteron: Corticosteron und 17α-Hydroxy-desoxy-corticosteron. Im V. spermatica-Blut wurden große MengenΔ 4-Androstendion, ferner 17α-Hydroxy-progesteron, 11β-Hydroxy-Δ 4-androstendion und Testosteron nachgewiesen.
Die biochemischen und morphologischen Gesichtspunkte sowie die klinischen Auswirkungen des Tumors werden diskutiert. Morphologisch hatte der Tumor den Aspekt eines Leydigzell-Adenoms. In Übereinstimmung hiermit wurde eine hohe Sekretion von Androgenen festgestellt. Durch den Nachweis einer 11β-Hydroxylase-Aktivität zeigte der Tumor jedoch gleichzeitig eine Eigenschaft, die sonst nur NNR-Zellen zukommt. Der Tumor wird vermutungsweise von atypischen Leydigzellen abgeleitet.
Diese Art von Leydigzell-Adenom wird der beidseitigen tumorartigen Hodenvergrößerung, die gelegentlich beim angeb. adrenogenitalen Syndrom beobachtet wird, gegenübergestellt. Die gemeinsamen und unterschiedlichen Eigenschaften der beiden Wucherungen werden besprochen.
Summary
An androgen producing tumour of the testis was removed from a boy with pseudopubertas praecox. Urinary excretion of total 17-oxosteroids, androsterone and aetiocholanolone, 11-oxy-17-oxosteroids (particularly 11β-hydroxy-androsterone), pregnandiol, pregnantriol and oestrogens were abnormally high before operation, and had decreased to normal after operation. After incubation of tumour slices with glucose alone androst-4-en-3,17-dione and 11β-hydroxyandrost-4-en-3,17-dione were found; after incubation with androst-4-en-3,17-dione: 11β-hydroxyandrost-4-en-3,17-dione, testosterone and adrenosterone, but no oestrogens were found; after incubation with desoxycorticosterone: corticosterone and 17α-hydroxycorticosterone. A large amount of androst-4-en-3,17-dione was found in Vena spermatica blood, as well as 17α-hydroxyprogesterone, 11β-hydroxyandrost-4-en-3,17-dione and testosterone.
The biochemical and morphological aspects are discussed in relation to the clinical manifestations of the tumour. The tumour had the morphological appearance of a Leydig cell adenoma and also showed a high rate of androgen secretion. The finding of 11β-hydroxylase activity in the tumour shows that it possessed a characteristic usually found only in adrenocortical cells. The tumour was conceivably derived from atypical Leydig cells.
This type of Leydig cell adenoma is compared with the bilateral tumour-like enlargement of the testis, which is ocassionally observed in congenital adrenogenital syndrome. The common and contrasting characteristics of the two growths are discussed.
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Herrn Prof. Dr.E. Rehn zum 85. Geburtstag gewidmet.
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Bayer, J.M., Tonutti, E., Nocke, W. et al. Klinische, morphologische und biochemische Untersuchungen bei einem androgenbildenden Hodentumor. Klin Wochenschr 43, 1022–1031 (1965). https://doi.org/10.1007/BF01746590
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DOI: https://doi.org/10.1007/BF01746590