This clinical scenario involves fibrous dysplasia of bone in female patients where the condition drives gonadotropin-independent sex steroid production, leading to precocious puberty, advanced bone age, and recurrent vaginal bleeding — findings that together signal the need for hormonal intervention.
Gonadotropin-independent sex steroid production in this setting results in early sexual development and recurrent ovarian cysts. Treatment is indicated specifically when bone age is advanced and frequent bleeding episodes are present.
Treatment for precocious puberty is indicated if bone age is advanced and there is frequent bleeding.
Gonadotropin-independent sex steroid production resulting in precocious puberty, recurrent ovarian cysts in girls and women or autonomous testosterone production in boys and men.
First line therapy is letrozole, with tamoxifen or fulvestrant as second line or adjuvants.
DOI: 10.1186/s13023-019-1102-9
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