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 cyst detected by ultrasound at the first acute episode. Plasma oestradiol was raised in only five of the cases, but all had a low response to luteinising hormone releasing hormone stimulation. Follow up lasted for up to eight years with recurrent episodes of variable frequency and severity in all seven patients. Evidence of McCune-Albright syndrome appeared later in only three patients. It could not be predicted from the initial symptoms or the clinical course. Mutations of the G(s)alpha protein leading to activation were investigated in the lymphocytes and ovarian and bone tissues of four patients. Only one patient showed a mutation in bone tissue. Close follow up with repeated searches for skeletal lesions remains necessary since the distribution of somatic mutations cannot be assessed by molecular studies. Most patients with recurrent ovarian cysts require a conservative approach.
Rodriguez-Macias
K A
KA
Pediatric Endocrinology Unit, Hôpital des Enfants Malades, 149 rue de Sèvres, 75015 Paris, France.
Thibaud
E
E
Houang
M
M
Duflos
C
C
Beldjord
C
C
Rappaport
R
R
D002648:Child; D002675:Child, Preschool; D004958:Estradiol / Q000097:blood; D005260:Female; D005640:Follicle Stimulating Hormone / Q000097:blood; D005500:Follow-Up Studies; D019204:GTP-Binding Proteins / Q000235:genetics; D006801:Humans; D007223:Infant; D007986:Luteinizing Hormone / Q000097:blood; D020125:Mutation, Missense; D010048:Ovarian Cysts / Q000097:blood / Q000150:complications* / Q000235:genetics; D011629:Puberty, Precocious / Q000097:blood / Q000209:etiology*; D012008:Recurrence
D004958:Estradiol; D007986:Luteinizing Hormone; D005640:Follicle Stimulating Hormone; D019204:GTP-Binding Proteins