A syndrome characterized by recurrent symptomatic functional ovarian cysts in young women

A syndrome characterized by the persistent recurrence of symptomatic functional ovarian cysts, elevated plasma estradiol levels, and infertility in four young patients is described. Two of the patients experienced recurring ovarian cysts following total abdominal hysterectomy and bilateral salpingo-oophorectomy. Pathologic findings confirmed the presence of ovarian tissue in all cysts. Recurrent cysts formation is attributed to either an increased production of gonadotropins or a hypersensitive response to normal gonadotropins. The development of recurrent cysts following bilateral oophorectomy indicates that some ovarian or "ovarian-like" tissue is present within the pelvic cavity, anatomically unrelated to the normally positioned ovaries. This extraovarian tissue may become sensitive to circulating gonadotropin levels in the absence of normal ovarian tissue. The source of this extraovarian tissue is discussed from an embryologic standpoint.