Male pseudohermaphroditism by persistent mullerian structures

Abstract

We have studied 2 cases of nonfamilial male pseudohermaphroditism by persistent müllerian ducts. The first case, found in a fourteen-year-old male, can be described as the classic form of cryptorchism which resisted hormonal treatment and in which a rudimentary uterus with fallopian tubes and atrophic testicles were found at exploratory laparotomy. The second case was discovered in a thirty-nine-year-old man who had bilateral crytorchism. He presented with an abdominal mass which was found to be a seminoma in the right intra-abdominal testicle, and a well-defined uterus with fallopian tubes and an atrophic left testicle were seen. Neither case presented phenotype disturbances. Both karyotypes were 46 XY. A total resection of the female sexual organs and testicles was performed in both patients, and the mass was removed in the second case. The tumoral degeneration of the intra-abdominal testicles in this syndrome is found in similar proportion to the simple cryptorchid testicles. Our second case is the tenth one of a testicular tumor in pseudohermaphroditism by persistent mullerian ducts to be reported in the literature.