McCune-Albright syndrome (MAS) is characterized by polyostotic fibrous dysplasia, cafe-au-lait hyperpigmentation of the skin, and acromegaly. Ablative therapy in affected patients must target the entire pituitary gland, making medical management the preferred therapy. Targeted medical therapies, including somatostatin and growth hormone (GH) receptor blockade, have shown promise in achievement of biochemical remission. We present the case of a 15-year-old girl with MAS who was discovered to have pituitary enlargement on computed tomography scan. She presented with cafe-au-lait spots, fibrous dysplasia of the sphenoid bone, and elevated insulin-like growth factor 1 and GH levels that failed to suppress on oral glucose tolerance testing. Treatment with a somatostatin analog was shown to be a safe and effective long-term management strategy of acromegaly that provides advantages over surgical resection.
- Fibrous dysplasia
ASJC Scopus subject areas
- Endocrinology, Diabetes and Metabolism