Treatment of Fibrous Dysplasia of Bone with Gonadotropin-Independent Precocious Puberty in Female Patients

Clinical Scenario

This protocol addresses female patients with fibrous dysplasia of bone who present with gonadotropin-independent precocious puberty, advanced bone age, and frequent vaginal bleeding.

Female sex Gonadotropin-independent precocious puberty Advanced bone age Frequent vaginal bleeding

When Treatment Is Indicated

Treatment for precocious puberty is indicated when bone age is advanced and there is frequent bleeding. In fibrous dysplasia, gonadotropin-independent sex steroid production can drive precocious puberty and recurrent ovarian cysts in female patients.

Treatment Approach

First-line management of precocious puberty in this setting involves letrozole.

Full regimen, sequencing, and supporting options are in the complete protocol…

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References

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 View source ↗