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McCune-Albright Syndrome — RACP Paediatrics MCQ

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HardEndocrinologyMcCune-Albright SyndromeRACP Paediatrics

A 7-year-old girl has tall stature, precocious puberty (breast Tanner 3, pubic hair Tanner 2, menses), and multiple café-au-lait spots with irregular borders. X-rays show polyostotic fibrous dysplasia. What is the diagnosis?

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Correct answer: A — McCune-Albright syndrome

McCune-Albright syndrome: activating GNAS1 mutation causing the triad of polyostotic fibrous dysplasia, café-au-lait spots with irregular ('coast of Maine') borders, and peripheral precocious puberty (GnRH-independent). Other endocrinopathies (hyperthyroidism, Cushing, GH excess) may also occur.

Reference: RCH Melbourne – 2024 – Clinical Practice Guidelines: Precocious Puberty