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2014 ; 99
(6
): 1955-69
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Acromegaly and McCune-Albright syndrome
#MMPMID24517150
Salenave S
; Boyce AM
; Collins MT
; Chanson P
J Clin Endocrinol Metab
2014[Jun]; 99
(6
): 1955-69
PMID24517150
show ga
BACKGROUND: McCune-Albright syndrome (MAS) includes the triad of poly/monostotic
fibrous dysplasia, café-au-lait spots, and hyperfunctioning endocrinopathies.
Acromegaly affects around 20% of MAS patients. AIMS: The objective was to review
all reported cases of acromegaly associated with MAS. METHODS: All studies and
case reports of acromegaly in patients with MAS were systematically sought in the
world literature up to January 2013. We also included new data (from three
unreported cases) and updated data on 23 previously reported patients from our
two centers. RESULTS: We reviewed the cases of 112 patients (65 males). Mean age
at diagnosis of acromegaly was 24.4 years (range, 3-64). Among the 40 pediatric
patients, 23 (57%) had precocious puberty. GH/IGF-1 excess was suggested by
accelerated growth in 85% of pediatric cases. Acromegaly was almost always
associated with skull base fibrous dysplasia. Modern imaging techniques (computed
tomography or magnetic resonance imaging) revealed an adenoma in 54% of the
patients (macroadenoma in more than two-thirds). Median GH levels and mean IGF-1
SD score at diagnosis were 57 ?g/L (2.8 to 291 ?g/L) and 8 (2.3 to 24),
respectively. Hyperprolactinemia was present in 81% (mean, 149 ?g/L; range,
21-600). Pituitary surgery, performed in 25 cases, very rarely cured the GH/IGF-1
excess. Somatostatin analogs improved GH/IGF-1 levels in most patients but
achieved control of acromegaly in only 17 (30%) of 56 patients. Pegvisomant
achieved normal IGF-1 levels in 10 of 13 cases. CONCLUSION: Acromegaly, which is
present in 20-30% of patients with MAS, raises particular diagnostic and
therapeutic issues.