April - June 2011, Volume 7, Issue 2

Chentli F, Belahcene S, Rezzoug M, Fedala NS, Belhimer F

McCune Albright Syndrome with a Somatolactotroph Pituitary Adenoma Causing Gigantism: Econography and Literature Review

Acta Endo (Buc) 2011, 7 (2): 273-282
doi: 10.4183/aeb.2011.273

McCune Albright syndrome (MAS) is a rare disease due to a sporadic mutation in Gs protein inducing polyostotic fibrous\r\ndysplasia, pigmented skin patches and hyper functioning endocrinopathies. We aimed to report its association with a hot thyroid nodule and gigantism.\r\nCase report. A man aged 37, with a history of pigmented skin lesions and lameness was referred for acromegaly. He was giant (height = 1.94 m/ target stature = 1.68 m), and had ?caf? au lait? spots. Biological analyses argued for pituitary mixed secretion (random growth hormone = 22 ng/ mL, N<5; prolactin = 27 ng/mL, N<10). Brain CT scan showed a pituitary process of 11x10 mm. Thyroid radioiodine scan revealed a hot nodule. Bone x-ray demonstrated large osteolytic lesions in the right femur and pelvis. He was operated on for endocrine tumours. The thyroid nodule\r\nwas benign and immunohistochemistry pituitary was positive for GH, prolactin and &#945; subunit.\r\nConclusion. Somatolactotrop adenoma causing gigantism associated with MAS is exceptional. It should be known, diagnosed, and treated early to avoid bone deformations\r\nand malignant transformation of osseous lesions under GH and/or IGF1 excess. One should also know that radiotherapy for the pituitary process is contraindicated, because of higher risk of sarcomatous transformation.

Keywords: McCune Albright syndrome; acromegaly gigantism; mixed\r\npituitary adenoma; thyroid hot nodule

Correspondence: Chentli Farida MD, Bab El Oued Hospital - Endocrine and Metabolic Diseases, 5 boulevard Said Touati Algiers 16000, Algeria, Tel: 0021321960040 Fax: 0021321960040 Email: endofarida@hotmail.fr