Abstract
McCune-Albright syndrome (MAS) is a complex congenital disorder due to mutations at the onset of embryonic activation of postzygotic somatic cells in the GNAS1 gene. The phenotype is heterogeneous and includes polyostotic/monostotic fibrous dysplasia, cafe-au-lait skin spots, and hyperfunctioning endocrinopathies including excess of growth hormone (GH). Similarly, acromegaly as a manifestation of endocrine hyperfunction is uncommon and affects about 20% of patients with MAS. Here, we describe a 31-year-old woman with severe facial involvement of polyostotic fibrous dysplasia, in association with acromegaly and McCune-Albright syndrome, in which it was satisfactorily managed by surgical recontouring, with no clinical signs of relapse at a 12-year follow-up. Patients with craniomaxillofacial fibrous dysplasia associated with acromegaly may present significant facial asymmetries, which can be satisfactorily solved by cosmetic treatment, especially in patients with psychological problems and problems of severe social acceptance.
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