Mc Cune Albright syndrome: gynecological perspective

Authors

  • Thangamani M. Department of Obstetrics and Gynecology, Government Rajaji Hospital Associated with Madurai Medical College, Madurai, Tamil Nadu, India
  • Priyadharshini P. Department of Obstetrics and Gynecology, Government Rajaji Hospital Associated with Madurai Medical College, Madurai, Tamil Nadu, India
  • Dharani E. Department of Obstetrics and Gynecology, Government Rajaji Hospital Associated with Madurai Medical College, Madurai, Tamil Nadu, India

DOI:

https://doi.org/10.18203/2320-1770.ijrcog20230530

Keywords:

McCune Albright syndrome, Precocious puberty, Polyostotic fibrous dysplasia, Café au lait spots

Abstract

The key features of McCune-Albright syndrome include sexual precocious puberty, polyostotic fibrous dysplasia and café au lait spots. It is associated with hyperfunction of multiple endocrine glands. Other endocrine dysfunctions often associated are growth hormone excess, hyperthyroidism, Cushing’s syndrome, hyperprolactinemia and phosphate wasting. We had a case of McCune-Albright syndrome with precocious puberty and irregular cycles and was managed. It is a rare cause of precocious puberty and should be kept in mind while dealing with such cases.

References

Chanson P, Salenave S, Young J. Ovarian dysfunction by activating mutation of GS alpha: McCune-Albright syndrome as a model. Ann Endocrinol (Paris). 2010;71(3):210-3.

Jung AJ, Soskin S, Paris F, Lipsker D. McCune-Albright syndrome revealed by Blaschko-linear café-au-lait spots on the back]. Ann Dermatol Venereol. 2016;143(1):21-6.

Collins MT,Singer FR, Eugster E. McCune-Albright syndrome and the extraskeletal manifestations of fibrous dysplasia. Orphanet J Rare Dis. 2012;7(1):S4.

Schoelwer M, Eugster EA. Treatment of peripheral precocious puberty. Endocr Dev. 2016;29:230-9.

Estrada A, Boyce AM, Brillante BA, Guthrie LC, Gafni RI, Collins MT. Long-term outcomes of letrozole treatment for precocious puberty in girls with McCune-Albright syndrome. Eur J Endocrinol. 2016;175(5):477-83.

Majoor BC, Boyce AM, Bovée JV, Smit VT, Collins MT, Cleton-Jansen A, et al. Increased risk of breast cancer at a young age in women with fibrous dysplasia. J Bone Miner Res. 2018;33:84-90.

Boyce AM, Collins MT. Fibrous Dysplasia/McCune-Albright Syndrome: A Rare, Mosaic Disease of Gα s Activation. Endocr Rev. 2020;41(2):345-70.

Laven JS, Lumbroso S, Sultan C, Fauser BC. Dynamics of ovarian function in an adult woman with McCune--Albright syndrome. J Clin Endocrinol Metab. 2001;86(6):2625-30.

Laven JS, Lumbroso S, Sultan C, Fauser BC. Management of infertility in a patient presenting with ovarian dysfunction and McCune-Albright syndrome. J Clin Endocrinol Metab. 2004;89(3):1076-8.

Chanson P, Salenave S, Young J. Ovarian dysfunction by activating mutation of GS alpha: McCune-Albright syndrome as a model. Ann Endocrinol (Paris). 2010;71(3):210-3.

Chevalier N, Paris F, Fontana S, Delotte J, Gaspari L, Ferrari P, et al. Postpubertal persistent Hyperestrogenemia in McCuneAlbright syndrome: unilateral oophorectomy improved fertility but detected an unexpected borderline epithelial ovarian tumor. J Pediatr Adolesc Gynecol. 2015;28(6):e169-72.

Lavoué V, Morcel K, Bouchard P, Sultan C, Massart C, Grall JY, et al. Restoration of ovulation after unilateral ovariectomy in a woman with McCune-Albright syndrome: a case report. Eur J Endocrinol. 2008;158(1):131-4.

Alammari R, Lightfoot M, Hur HC. Impact of cystectomy on ovarian reserve: review of the literature. J Minim Invasive Gynecol. 2017;24(2):247-57.

Boyce AM, Casey RK, Ovejero Crespo D, Murdock CM. Gynecologic and reproductive outcomes in fibrous dysplasia/McCune-Albright syndrome. Orphanet J Rare Dis. 2019;14(1):90.

Downloads

Published

2023-02-27

Issue

Section

Case Reports