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Restoration of ovulation after unilateral ovariectomy in a woman with McCune–Albright syndrome: a case report

Department of Obstetric Gynecology, University Hospital, Rennes, France¹ Department of Endocrinology, Saint Antoine University Hospital, AP-HP, Paris, France² Department of Hormonology, Montpellier and Institut National de la Santé et de la Recherche Médicale, University Hospital, Montpellier, France and ³ Department of Biochemistry, University Hospital, Rennes, France

(Correspondence should be addressed to M-C Laurent who is now at DOGMR, CHU Anne de Bretagne, 16 Bd de Bulgarie, BP 90347, F-35 203 Rennes Cedex 2, France; Email: laurentmc{at}chu-rennes.fr)

Abstract

Introduction: McCune–Albright syndrome (MAS) is characterized by peripheral precocious puberty, café-au-lait spots, and polyostotic fibrous dysplasia. This syndrome is due to a post-zygotic mutation of the GNAS1 gene with mosaic distribution and unilateral predominance. Clinical manifestations depend on the tissues carrying the mutation. We describe the ovarian function before and after unilateral ovariectomy in a woman with MAS and bilateral distribution of the GNAS1 gene mutation.

Case report: A 33-year-old patient, previously diagnosed as having MAS, presented irregular menstrual cycles (30–180 days) and monophasic temperature curves. Transvaginal ultrasound and blood tests were repeated at 3-day intervals over 3 months. Findings included a persistent quiescent left ovary, a persistent polycystic right ovary, constantly high estradiol-17β (E₂) levels, and very low FSH and LH levels. She also presented severe persistent pelvic pain. Because of unilateral ovarian activity, a unilateral right ovariectomy was performed as well as biopsy of the remaining left ovary. A GNAS1 gene mutation was identified in both ovaries. A regular monthly menstrual cycle was immediately restored. On day 3 of the menstrual cycle, E₂ level was 30 pg/ml, FSH level was 7.5 mIU/ml, and LH level was 6.4 mIU/ml. On day 17, pelvic ultrasound showed one follicle of 25 mm in the left ovary. On day 21, the progesterone level was 13.1 ng/ml.

Discussion: This is the first report of ovulation being restored following unilateral ovariectomy in an adult patient suffering from severe MAS with GNAS1 gene mutation identified in both ovaries.