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Mccune-Albright Syndrome, a Rare Form of Precocious Puberty: Diagnosis, Treatment, and Follow-Up

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Mccune-Albright Syndrome, a Rare Form of Precocious Puberty: Diagnosis, Treatment, and Follow-Up Original article Arch Argent Pediatr 2021;119(5):e420-e427 / e420 McCune-Albright syndrome, a rare form of precocious puberty: diagnosis, treatment, and follow-up Mirta Gryngarten, M.D.a, Haydeé Comar, M.D.b, Andrea Arcari, M.D.a, Elisabeth Boulgourdjian, M.D.a and María E. Escobar, M.D.a Collaborator: Horacio Domené, Ph.D.c a. Center for Endocrinological Research (Centro ABSTRACT Acronyms and abbreviations de Investigaciones Introduction. McCune-Albright syndrome CPP: central precocious puberty. Endocrinológicas, (MAS) is a genetic disorder defined by fibrous CEDIE) “Dr. dysplasia of bone, café-au-lait skin spots, and E2: estradiol. César Bergadá”, autonomous hyperfunction of one or more FDB: fibrous dysplasia of bone. National Scientific endocrine organs. MAS is caused by activating FSH: follicle-stimulating hormone. and Technical mutations of the GNAS1 gene. The most GH: growth hormone. Research Council frequent type of endocrinopathy is gonadal (Consejo Nacional endocrinopathy in the form of peripheral GnRH: gonadotropin-releasing de Investigaciones precocious puberty. hormone. Científicas y Técnicas, Objective. To describe the clinical characteristics, IFMA: immunofluorometric assay. CONICET). laboratory and imaging tests at the time of LH: luteinizing hormone. diagnosis and over the course of the disease, Pediatric MAS: McCune-Albright syndrome. Endocrinology focusing on the classical triad of MAS. Foundation Population and methods. Observational, MPH: midparental height. (Fundación de descriptive, retrospective clinical study of PP: precocious puberty. Endocrinología patients with MAS seen at the Department of PPP: peripheral precocious puberty. Infantil, FEI). Endocrinology of Hospital de Niños Ricardo Gutiérrez between 1974 and 2019. RIA: radioimmunoassay. Department of Results. Twelve girls are described, all of whom Endocrinology, developed peripheral precocious puberty (PPP) Hospital de Niños secondary to functional ovarian cysts. Their Ricardo Gutiérrez, INTRODUCTION age at presentation was early (2.6 ± 1.3 years). Autonomous City Gonadotropin levels were suppressed or in the Precocious puberty in girls of Buenos Aires, prepubertal range with generally high estradiol is defined as the onset of pubertal Argentina. levels. Ten girls had café-au-lait skin spots since development before 8 years of age.1 b. Melipal Children’s birth. During the course of disease, polyostotic Two types of precocious puberty Center (Centro fibrous dysplasia was detected in all patients. Pediátrico Melipal, The treatments used to reduce ovarian cyst (PP) have been established based CPM), San Carlos recurrence and hyperestrogenism effects showed on their originating mechanism: de Bariloche, varied effectiveness. central precocious puberty (CPP), Argentina. Conclusions. In this series, the onset of PPP caused by the early activation c. Center for helped to make an early diagnosis of MAS Endocrinological and was difficult to treat. The course of disease of the hypothalamic-pituitary- Research (Centro showed persistent gonadal hyperfunction and gonadal (HPG) axis, and peripheral de Investigaciones worsening of bone injuries. precocious puberty (PPP), caused by Endocrinológicas, Key words: McCune-Albright syndrome, precocious an autonomous gonadal activity. One puberty, fibrous dysplasia of bone. CEDIE) “Dr. César of the etiologies of PPP is McCune- Bergadá”. http://dx.doi.org/10.5546/aap.2021.eng.e420 Albright syndrome (MAS), which is E-mail address: accompanied by the involvement of Haydeé Comar, M.D.: To cite: different organs and systems. haydeecomar@gmail. Gryngarten M, Comar H, Arcari A, Boulgourdjian E, Escobar ME. McCune-Albright MAS was first described in 1936 com syndrome, a rare form of precocious puberty: by Donovan McCune2 and in 1937 by diagnosis, treatment, and follow-up. Arch Argent Pediatr 3 Funding: 2021;119(5):e420-e427. Fuller Albright. The association of None. precocious puberty, fibrous dysplasia of bone (FDB), and café-au-lait skin Conflict of interest: None. spots is currently known as the typical MAS triad.4-6 It may be associated with Received: 12-1-2020 different endocrinopathies and other Accepted: 4-12-2021 organ involvement.6-8 MAS prevalence McCune-Albright syndrome, a rare form of precocious puberty: diagnosis, treatment, and follow-up / e421 ranges from 1/100 000 to 1/1 000 000 in the inhibited if the baseline and/or post-GnRH general population.4 It is a sporadic disorder stimulation values were < 2 standard deviations caused by a postzygotic activating mutation of the (SD) of the normal pre-pubertal values for the GNAS1 gene that codes the stimulatory G protein assay used.17,18 α-subunit (Gsα), leading to constitutive activation The molecular analysis to detect the GNAS1 of adenylyl cyclase with unrestricted production gene mutation was done in DNA from peripheral and excess intracellular cAMP, hyperplasia, and blood and tissue using the allele-specific uncontrolled secretion of cell products.9,10 polymerase chain reaction (AS-PCR).19 The most frequent type of endocrinopathy The following imaging tests were done: pelvic is gonadal endocrinopathy in the form of PPP. ultrasound, x-ray for bone age (BA), bone scan, Although male and female children may be others (bone x-rays, CAT scan, MRI) based on affected, a significantly higher prevalence has clinical data. been described in girls, with a 9:1 ratio.11-14 The Results are expressed as mean and SD or autonomous activation of the ovary leads to median (Me) and range. Wilcoxon’s test was the intermittent development of follicular cysts, used for paired samples to assess treatment which cause varying levels of estradiol and outcomes. Auxological data were unified by clinical signs of hyperestrogenism. Some girls translating them into a SD score (SDS) based on may secondarily develop CPP. the Argentine reference tables by Lejarraga et al.20 Different treatments have been used to The study was approved by the Research manage MAS, aimed at reducing the recurrence Ethics Committee (REC approval no.: 14.48) and of ovarian cysts, counteracting the manifestations the Research and Teaching Committee of HNRG. of estrogen excess, and stopping the advancement The principles established in the World Medical of bone injuries.13 Association’s Declaration of Helsinki and Law The objective of this study was to describe the 3301/09 for the Protection of the Rights of Health clinical characteristics and supplementary tests Research Subjects of the Autonomous City of in a cohort of girls with MAS, the effectiveness Buenos Aires were met. of different treatments, and the long-term course of patients. RESULTS Ten patients showed the typical MAS triad, POPULATION AND METHODS while 2 had MAS with 2 manifestations (Table 1). This was an observational, descriptive, retrospective clinical study carried out at the Diagnosis and management Department of Endocrinology of Hospital de In all patients, the first manifestation of Niños Ricardo Gutiérrez (HNRG). The medical disease was precocious puberty at an onset age records of girls with MAS seen between 1974 (mean ± SD) of 1.8 ± 1.3 years. Chronological age and 2019 were analyzed, at the time of diagnosis (CA) at diagnosis was 2.3 ± 1.3 years, while BA and over the course of their condition until was 3.7 ± 1.6 years. BA was advanced in 6 girls, adolescence. Inclusion criteria: MAS showing the whereas the rest had a BA that matched their triad of PPP, café-au-lait skin spots, and FDB or CA. The first sign was simultaneous breast MAS with only two manifestations. Exclusion development (thelarche) and vaginal bleeding in criteria: Girls with recurrent ovarian cysts, with 9/12 patients (Table 2). In 8/12 patients, unilateral no other syndrome components. ovarian cysts with a diameter between 29 and The following were analyzed: age at onset of the first sign of puberty, vaginal bleeding (VB), café-au-lait skin spots, anthropometric data, age at menarche, menstrual cycle alterations, TABLE 1. Clinical manifestations bone alterations, and final height compared to Clinical manifestations n: 12 midparental height (MPH). Lab tests, estradiol (E2), FSH, and LH at baseline and after GnRH Fibrous dysplasia of bone 12 stimulation were done using a RIA until 1994 Precocious puberty 12 and an IFMA thereafter. A serum E2 level Café-au-lait skin spots 10 Hyperthyroidism 2 ≥ 14 pg/mL15 with both methods was considered 16 Growth hormone hypersecretion 2 indicative of puberty. Gonadotropin levels Hyperprolactinemia 2 assessed by RIA or IFMA were considered e422 / Arch Argent Pediatr 2021;119(5):e420-e427 / Original article 53 mm were observed. E2 and gonadotropin triad. In 1 of them, several samples were obtained levels are shown in Table 3. Serum E2 values for surgery and the mutation was found in her ranged between undetectable (< 10 pg/ml) and skin, muscle, bone, and thyroid tissue. 90 pg/ml. In 3 patients whose ultrasound did Data related to PPP treatment are shown in not show ovarian cysts, high E2 levels were Figure 1. The first line of treatment in all girls observed. In 9 girls, serum gonadotropins were were weekly medroxyprogesterone acetate determined after GnRH stimulation and the (MPA) injections for 6.1 ± 2.6 years. Tamoxifen value was prepubertal or inhibited, whereas only was administered to 3 patients for 1.2 to 2.4 years. baseline levels were determined in 3 girls, who With the emergence of aromatase inhibitors, MPA had an inhibited value, thus confirming their PPP was interrupted in 2 girls and treatment with diagnosis. Six patients had
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