McCune-Albright syndrome: growth hormone and prolactin hypersecretion.
McCune-Albright syndrome (MAS) has a special interest for endocrinologists as its pathogenesis results in hypersecretion of hormones in peripheral endocrine tissues. This can be expressed as precocious puberty, mainly in girls, primary hyperthyroidism, growth hormone (GH) and/or prolactin excess, hyperparathyroidism and hypercortisolism. The incidence of GH excess among patients with MAS has been assessed as up to 21%. The pathogenesis of GH hypersecretion in MAS is not completely understood, whereas it seems to be different from the aetiology of acromegaly/gigantism in non-MAS patients. The clinical expression of GH excess can be masked because of precocious puberty or craniofacial fibrous dysplasia, indicating the necessity for screening. Medical treatment is usually the only option in MAS patients with GH excess, as transsphenoidal surgery is usually restricted due to massive thickening of the skull base, whereas radiotherapy is contraindicated due to probable higher predisposition to sarcomatous transformation. The use of bromocriptine, cabergoline and octreotide, or the combination of these, has shown variable results, whereas pegvisomant, a GH receptor antagonist, is a new promising option, although not yet used in patients with MAS.
Christoforidis
Athanasios
A
Department of Endocrinology, Great Ormond Street Hospital and the Middlesex Hospital (UCLH), London, UK.
Maniadaki
Ilianna
I
Stanhope
Richard
R
D000293:Adolescent; D005260:Female; D005359:Fibrous Dysplasia, Polyostotic / Q000097:blood* / Q000150:complications; D013006:Growth Hormone / Q000097:blood* / Q000378:metabolism; D006801:Humans; D007334:Insulin-Like Growth Factor I / Q000032:analysis*; D008297:Male; D010900:Pituitary Diseases / Q000097:blood / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D010902:Pituitary Gland / Q000378:metabolism; D011388:Prolactin / Q000097:blood* / Q000378:metabolism
D007334:Insulin-Like Growth Factor I; D011388:Prolactin; D013006:Growth Hormone