Zusammenfassung
Gonadoblastome sind primäre Keimzellgeschwülste, welche eine reaktive Wucherung der Sertolizellen oder Granulosazellen ausgelöst haben. Dies kommt im normalen Hoden nur ganz ausnahmsweise vor (2 Fälle). Etwas weniger selten ist diese Reaktion in normalen Ovarien (8 Fälle), wo die Reaktion in schon bestehenden Stromawucherungen beobachtet wird. Die Reaktion variiert in Form und Grad. Sie kann verbunden sein mit Oestrogensekretion und schließlich einen geschwulstartigen Charakter annehmen. Sie kommt besonders häufig und in charakteristischer Form in dysgenetischen Keimdrüsen vor (50 Fälle), wo sie entweder von den Samenkanälchen oder von wenig ausdifferenzierten Keimzellen und Granulosazellnestern, und zwar unmittelbar angrenzend an das Keimgewebe ihren Ausgang nimmt. In dieser letzten Form verhält sich das Gonadoblastom wie eine in bezug auf den Geschlechtscharakter nicht voll ausdifferenzierte Geschlechtszelle, doch vorwiegend in Richtung männliches Geschlecht mit hormonalfunktionellen Auswirkungen in der Pubertät. Letztere scheinen vom Hypophysenvorderlappen-Gonadotropin abhängig zu sein, wie auch der Übergang in eine Neubildung.
Summary
There are described (1) a seedling gonadoblastoma (gonocytoma III) in a girl of 19 with primary amenorrhoea, an XO/XY karyotype and dysgenetic ovaries: there were no testicular rudiments and the tumour made no connection with any other structure; (2) a large gonadoblastoma (gonocytoma II) in a chromatin positive girl of 16 with puberty menorrhagia, showing diverse interrelated patterns including dysgerminoma, fibroma, granulosal cell tumour and embryonal carcinoma; and (3) a seminoma in a normal man of 23 with 4 small foci of intratubular gonadoblastoma at the tumour margin.
A survey of the literature, classification and main features of the tumour suggest that: (1) 50 cases are classifiable as gonocytoma III and 10 as gonocytoma II; (2) tumours arising in normal ovaries are histologically diverse whilst the rest resemble one another; (3) the tumour arises as a primary germ cell neoplasm in either testicular or ovarian tissue, evoking an enveloping Sertoli cell response in the former and a stromal response with subsequent granulosal metaplasia in the latter; (4) it can probably arise from dysgenetic structures lying at the periphery of malformed testis and the centre of malformed ovary; and (5) pituitary gonadotrophin promotes both its development and endocrine effects.
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Hughesdon, P.E., Kumarasamy, T. Mixed germ cell tumours (gonadoblastomas) in normal and dysgenetic gonads. Virchows Arch. Abt. A Path. Anat. 349, 258–280 (1970). https://doi.org/10.1007/BF00544577
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DOI: https://doi.org/10.1007/BF00544577