A CASE REPORT OF FIBROUS DYSPLASIA WITH ACROMEGALY: MC CUNE ALBRIGHT SYNDROME

Asha R. Iyengar; Seema Patil; Sanjana Marpuri; Shalini Dina Simon

Volume : VI, Issue : VIII, August - 2017

A CASE REPORT OF FIBROUS DYSPLASIA WITH ACROMEGALY: MC CUNE ALBRIGHT SYNDROME

Asha R. Iyengar, Seema Patil, Sanjana Marpuri, Shalini Dina Simon

Abstract :

McCune-Alight syndrome(MAS) is a triad of polyostotic or monostotic fious dysplasia, caf�-au-lait spots and hyperfunctioning endocrinopathies. However, a few cases have been reported by Alight wherein only 2 of the above features were noted. The polyostotic variant occurs more often in subjects with MAS as compared to monostotic/craniofacial forms. The most common endocrine abnormality noted is precocious puberty (in females). The other endrocrinopathies include hyperthyroidism, hyperparathyroidism, hypercortisolism, excessive secretion of growth hormone and prolactin. The association of acromegaly with MAS is rare and affects around 10-20% of subjects. In the present case report, a rare association of craniofacial fious dysplasia with pituitary macroadenoma resulting in acromegaly in a 23-year-old male is discussed. The subject did not present with caf�-au-lait spots. He had undergone surgery and radiotherapy for the pituitary adenoma 5 years prior to reporting to the institution. Patient is currently on somatostatin analogues. Facial recontouring is planned after complete regression of the tumour.