[Gonadoblastoma. Review of the literature. Apropos of a case].

Gonadoblastoma, which was described for the first time by Scully in 1953, is a rare tumour of the gonads. Only about 150 cases have been reported. Anatomo-pathologically it shows itself by a combination of large primitive germinal cells, small cells which resemble the immature cells found in the granulosa and in the Sertoli and sometimes Leydig-theco interstitial cells. Calcification is suggestive. Gonadoblastomas usually develop in a dysgenetic gonad. Pure gonadic dysgenesis is more commonly found. The subjects usually are of feminine phenotype and can demonstrate somatic or sexual abnormalities. The caryotypes most frequently found are 46 XY or 45 XO/46 XY. It is rare for the Y chromosome to be absent. All patients with gonadoblastomas should be HY antigen positive. The tumour is benign but the germinal epithelium can change towards a seminoma type. Because of this it is wise to carry out laparotomy with removal of both adnexae if there is a gonadoblastoma present. The case we have studied illustrates the typical profile of a gonadoblastoma. It occurred in a young woman who had pure gonadic dysgenesis with a caryotype 46 XY (Swyer's syndrome). It was bilateral associated on the left side with a seminoma, and for this reason we carried out bilateral extirpation of the adnexae followed up by therapy with cobalt.