Postpubertal Persistent Hyperestrogenemia in McCune-Albright Syndrome: Unilateral Oophorectomy Improved Fertility but Detected an Unexpected Borderline Epithelial Ovarian Tumor

Journal of Pediatric and Adolescent Gynecology
Nicolas ChevalierPatrick Fénichel

Abstract

McCune-Albright syndrome (MAS), due to a somatic mutation of the GNAS1 gene, begins usually in girls with peripheral precocious puberty. Ovarian autonomy may persist in adulthood with acyclic hyperestrogenemia, infertility, and a potential risk of estrogen-dependent cancer. A 22-year-old woman, with MAS, was referred for infertility with left macropolycystic ovary, hyperestrogenemia, and chronic anovulation unsuccessfully treated by controlled hyperstimulation. Once ovarian cyst punctures and cDNA analysis verified that GNAS1 mutation was restricted to the left ovary, unilateral ovariectomy was performed. It improved right ovarian function, allowed an in vitro fertilization-induced pregnancy, but revealed an unexpected borderline epithelial ovarian tumor. Several breast cancers have already been reported in young MAS patients but not a borderline epithelial ovarian tumor. In this context, we would recommend that persistent hyperestrogenemia in an adult be corrected and gynecological follow-up of the breasts, ovaries, and endometrium be implemented.

References

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Citations

Nov 3, 2016·Journal of Obstetrics and Gynaecology of India·A MishraS Sridhar
May 1, 2019·Orphanet Journal of Rare Diseases·Alison M BoyceMichael T Collins
Nov 2, 2019·Endocrine Reviews·Alison M Boyce, Michael T Collins
Jul 11, 2018·Frontiers in Endocrinology·Domenico CoricaMalgorzata Wasniewska
May 29, 2021·Journal of Gynecology Obstetrics and Human Reproduction·Mikaël AgopiantzGuillaume Gauchotte

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