Acromegaly, multinodular goiter and silent polyostotic fibrous dysplasia. A variant of the McCune-Albright syndrome.
A 36-year-old woman is reported with a possible variant of the McCune-Albright syndrome. The triad was incomplete because of the absence of skin pigmentation and since the sexual precocity was not evident. The presence of a pituitary mass and the secretory dynamics of growth hormone and prolactin were suggestive of a mammosomatotroph cell adenoma. A toxic multinodular goiter was also associated, but unique was the spontaneous normalization of the thyroid function. Unusual was the silent evolution of the polyostotic fibrous dysplasia, which was only fortuitously discovered during magnetic resonance imaging of the pituitary region. Treatment of the acromegaly with the long-acting somatostatin analogue octreotide resulted in an important inhibition of the GH secretion and in a reduction of the volume of the pituitary adenoma.
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Department of Endocrinology, University of Antwerp, Wilrijk, Belgium.
Beckers
A
A
Van de Vyver
F L
FL
De Schepper
A
A
Stevenaert
A
A
Hennen
G
G
D000172:Acromegaly / Q000175:diagnosis / Q000473:pathology*; D000236:Adenoma / Q000175:diagnosis / Q000188:drug therapy; D000328:Adult; D001921:Brain / Q000033:anatomy & histology; D001971:Bromocriptine / Q000494:pharmacology; D005260:Female; D005359:Fibrous Dysplasia, Polyostotic / Q000175:diagnosis / Q000473:pathology*; D006042:Goiter / Q000175:diagnosis / Q000473:pathology*; D013006:Growth Hormone / Q000097:blood; D006801:Humans; D008279:Magnetic Resonance Imaging; D008599:Menstruation Disturbances / Q000473:pathology; D015282:Octreotide / Q000627:therapeutic use; D010911:Pituitary Neoplasms / Q000175:diagnosis / Q000188:drug therapy; D011388:Prolactin / Q000097:blood
D001971:Bromocriptine; D011388:Prolactin; D013006:Growth Hormone; D015282:Octreotide