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J Neurol Surg A Cent Eur Neurosurg 2013; 74(S 01): e140-e144
DOI: 10.1055/s-0032-1330957
Case Report
Georg Thieme Verlag KG Stuttgart · New York

Gigantism Treated by Pure Endoscopic Endonasal Approach in a Case of McCune-Albright Syndrome with Sphenoid Fibrous Dysplasia: A Case Report

Guive Sharifi
1   Department of Neurosurgery, Loghman Hakim Hospital, Shaheed Beheshti University of Medical Sciences, Tehran, Islamic Republic of Iran
,
Maryam Jalessi
2   Endoscopic Pituitary and Skull Base Surgery Unit, ENT Head and Neck Research Center and Department, Hazrat Rasoul Akram Hospital, Tehran University of Medical Sciences, Tehran, Islamic Republic of Iran
,
Farzaneh Sarvghadi
3   Research Institute for Endocrine Sciences, Taleghani Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Islamic Republic of Iran
,
Mohammad Farhadi
2   Endoscopic Pituitary and Skull Base Surgery Unit, ENT Head and Neck Research Center and Department, Hazrat Rasoul Akram Hospital, Tehran University of Medical Sciences, Tehran, Islamic Republic of Iran
› Author Affiliations
Further Information

Publication History

15 January 2012

23 August 2012

Publication Date:
10 January 2013 (online)

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Abstract

McCune-Albright syndrome (MAS) is an uncommon polyostotic manifestation of fibrous dysplasia in association with at least one endocrinopathy that is mostly associated with precocious puberty and hyperpigmented skin macules named café-au-lait spots. We present an atypical manifestation of McCune-Albright syndrome in a 19-year-old man with the uncommon association of polyostotic fibrous dysplasia and gigantism in the absence of café-au-lait spots and precocious puberty. He presented with a height increase to 202 cm in the previous 3 years, which had become more progressive in the few months prior. Physical examination revealed only a mild facial asymmetry; however, a computed tomography (CT) scan discovered vast areas of voluminous bones with ground-glass density and thickening involving the craniofacial bones and skull base. Magnetic resonance imaging (MRI) found a right stalk shift of the pituitary with a 20 mm pituitary adenoma. We describe the diagnostic and endoscopic endonasal transsphenoidal approach for excision of the tumor.

Keywords

McCune-Albright syndrome - neuroendoscopy - pituitary adenoma
 

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