In this review we report the literature about ovarian function of young women with McCune-Albright Syndrome and describe our personal experience in the follow-up of a small cohort.Collectively, the existing data demonstrate that ovarian hyperfunction with ovarian cysts and hyperestrogenism persists in those women who had precocious puberty. The recording of menstrual cycles and the analysis of gonadotropin and estrogen secretion indicate that, when hypothalamic-pituitary pubertal activation begins, alternating episodes of gonadotropin control and ovarian autonomy can be seen. The persistence of estrogen hypersecretion causes menstrual disturbances and hypofertility. The long term consequences of this condition are hypothesized to be an increased risk of ductal breast cancer, which seems to be higher when growth hormone hypersecretion is also present. Therefore, young MAS women should receive counseling regarding fertility and ongoing surveillance for the development of estrogen related diseases.
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