[A case of Morris syndrome with extreme estrogen secretion and hypothalamo-hypophyseal-gonadal system disfunction]

A case is presented of a 23-year-old female hospitalized for infertility in whom full Morris syndrome was diagnosed. In the patient apart from testosterone level typical of a sexually mature man, high "castration" LH and FSH values and extreme hyper-estrogen secretion were found in hormonal investigation. In the histopathological pattern of the testicles removed from both inguinal canals numerous adenomas were demonstrated, originating from Sertoli cells and also, to a lesser degree, from Leydig cells. The unusual character of the case results also from complete insensitivity of the hypothalamo-hypophyseal system to circulating oestrogens and androgens (LH and FSH levels were not significantly changed after castration, and the GnRH stimulation test gave similar results before and after the operation). The authors discuss the possible mechanisms of the observed phenomena.