ESPE Abstracts (2018) 89 P-P3-327

Evolutive Profile of Pauci-symptomatic Forms of Mc Cune Albright Syndrome

Audrey Cartaulta, Emilie Tryoena, Françoise Parisb, Céline Bara & Catherine Pienkowskia


aHopital Des Enfants, Toulouse, France; bHopital Lapeyroinie, Montpellier, France


In young girls, the occurrence of secretory ovarian cysts may be the first manifestation of Mc Cune Albright Syndrome. We reported the evolutive profile of 8 patients with peripheral precocious puberty (PP) with (n=5 cases) or without metrorrhagia (n=3). On the first episode, they were 3.8 years old (range 2.5 to 7.25 years), the average diameter of the ovarian cyst was 38.5 mm (range 25 to 88 mm), the mean estradiol level was 32.5 pg/ml (range 3 to 160), mean AMH level was 3.35 ng/ml (range 1.9 to 8.7). 2 patients had café-au-lait spots (cases 4 and 7). No patient had bone lesions detected on the holoskeleton and no patient had detected GSa protein mutation by peripheral blood analysis. Five patients underwent cystectomy, GSa protein mutation was positive on the follicular fluid in 4 cases. The cyst spontaneously regressed in 3 cases. The rates of E2 and AMH level were not correlated to the diameter of the cyst. The recurrence of the cyst was noted once (case 7 at 4 yrs), twice (case 4 at 7 and 10 yrs) and 6 times (case 1 at 3, 4, 6, 7, 9 and 11 yrs). This girl with 6 recurrences (case 1) was followed until the age of 27 yrs, she had no other recurrence after 11 yrs. Mc Cune Albright syndrome is a sporadic disease with unpredictable evolution. Only follow-up is essential because there is no predictive factor of recurrence at the initial diagnosis.

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