Volume : 6, Issue : 8, 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 :

<p>&nbsp;<span lang="EN-GB" style="font-family: Times, serif;">McCune-Alight syndrome(MAS) is a triad of polyostotic or monostotic fious dysplasia, caf&eacute;-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.</span><span lang="EN-GB"> </span><span lang="EN-GB" style="font-family: Times, serif;">The most common endocrine abnormality noted is precocious puberty (in females).</span><span lang="EN-GB" style="font-family: &quot;Times New Roman&quot;, serif;"> The other endrocrinopathies include hyperthyroidism, hyperparathyroidism, hypercortisolism, excessive secretion of growth hormone and prolactin</span><span lang="EN-GB" style="font-family: Times, serif;">. </span><span lang="EN-GB" style="font-family: &quot;Times New Roman&quot;, serif;">The association of </span><span lang="EN-GB" style="font-family: Times, serif;">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&eacute;-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.&nbsp;&nbsp;</span></p> <p class="MsoNormal" style="line-height:200%"><o:p></o:p></p>