Multidisciplinary approach and clinical management of McCune-Albright syndrome

Read full text
Authors: Jingna Wang, Yanmei Sang
Cited by: 1
References: 72

Abstract

McCune-Albright syndrome (MAS) is a rare, sporadic mosaic disorder classically characterized by fibrous dysplasia (FD) of bone, café-au-lait macules, and endocrine hyperfunction. The estimated prevalence ranges from 1 in 100,000 to 1 in 1,000,000 individuals, with no clear differences across ethnic groups. The central pathogenic mechanism involves postzygotic somatic gain-of-function mutations in the GNAS gene, which encodes the α subunit of the stimulatory G protein (Gsα). The currently identified pathogenic variants are concentrated at codons R201 (e.g., R201H and R201C) and Q227. This review systematically summarizes the molecular genetic basis, phenotypic heterogeneity, histopathological and imaging characteristics, diagnostic strategies, precision treatment approaches, and long-term prognosis of MAS. By integrating recent advances, we also highlight emerging research directions and provide a structured framework to support clinical diagnosis and multidisciplinary management.

Fields of Study & Subjects

Bone Tumor Diagnosis and Treatments Genomics and Rare Diseases Osteomyelitis and Bone Disorders Research

Publication details

Journal / source
Frontiers in Endocrinology
Publisher
Frontiers Media SA
Volume
17
Pages
1786683–1786683
Publication date
2 April 2026
Language
EN