Restoration of Ovarian Function after Unilateral Oophorectomy in McCune-Albright Syndrome.

INTRODUCTION

McCune-Albright syndrome (MAS) is characterized by café-au-lait-skin-pigmentation, polyostotic-fibrous-dysplasia and gonadotropin-independent precocious puberty. We report a female patient whose ovarian function normalized following unilateral oophorectomy at young age.

CASE: The patient initially presented with peripheral precocious puberty at age 1 and was treated with aromatase inhibitors and selective estrogen receptor modulator until the age of 12 years for pubertal suppression. Cystectomy was performed at age 16 due to persistent right complex ovarian cysts with menorrhagia. Genetic testing of the cyst tissue confirmed GNAS mutation. Persistence of a complex ovarian cyst, extremely elevated estradiol levels, and refractory uterine bleeding led to right salpingo-oophorectomy and levonorgestrel intrauterine device placement at age 19, resulting in the restoration of ovarian function.

SUMMARY/

CONCLUSION

This report describes a single case of ovarian function restoration after unilateral oophorectomy in a patient with MAS, but should not be generalized to all MAS patients.