Precocious puberty in McCune-Albright syndrome: a case report

Abstract

McCune-Albright syndrome (MAS) is a rare disease characterized by a triad of fibrous
dysplasia, cafe-au-lait spots and peripheral precocious puberty. We reported a 5-year-
8-month old girl with MAS who has been followed-up for 2 years and 8 months. She
was referred to pediatric endocrinology clinic in our hospital for vaginal bleeding at age
of 2 years 11 months. She had peripheral precocious puberty, i.e. increased estrogen
level associated with very low gonadotropins, and cafe-au-lait spots on her face and was
diagnosed as MAS. The patient was treated with estrogen receptor blocker (tamoxifen).
She had no menses during the 2 years and 8 months of tamoxifen treatment. Her growth
rate and bone maturation were also in normal ranges. However, at the end of tamoxifen
treatment she had an episode of vaginal bleeding so that we had to change to other
treatment modalities.