Follow up of precocious pseudopuberty associated with isolated ovarian follicular cysts

The clinical outcomes of seven girls presenting with pseudosexual precocity caused by isolated autonomous ovarian follicular cysts are presented. Six of the seven girls, aged 11 months to 6.9 years, had a unilateral ovarian c yst detected by ultrasound at the first acute episode. Plasma oestradiol wa s raised in only five of the cases, but all had a low response to luteinisi ng hormone releasing hormone stimulation. Follow up lasted for up to eight years with recurrent episodes of variable frequency and severity in all sev en patients. Evidence of McCune-Albright syndrome appeared later in only th ree patients. It could not be predicted from the initial symptoms or the cl inical course. Mutations of the G(s)alpha protein leading to activation wer e investigated in the lymphocytes and ovarian and bone tissues of four pati ents. Only one patient showed a mutation in bone tissue. Close follow up wi th repeated searches for skeletal lesions remains necessary since the distr ibution of somatic mutations cannot be assessed by molecular studies. Most patients with recurrent ovarian cysts require a conservative approach.