Embryonal carcinoma in two cases of complete androgen insensitivity syndrome (CAIS) is reported. In both cases gonadectomy carried out for prophylactic purposes led to the discovery of a localized embryonal carcinoma with areas of anaplastic seminoma in one case. In non-neoplastic tissue, gonad morphology in both cases was typical of AIS. Prevalently hypotrophic aspects, especially in the interstitial gland, were found in case 2. This may explain the different endocrine profile in the two cases before gonadectomy. Our study, aside from series of psycho-sexual problems, shows, according to all Authors, that the most serious complication is the high risk of malignancy after puberty in patients with AIS.

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