Sharifi Guive, Jalessi Maryam, Sarvghadi Farzaneh, Farhadi Mohammad
Department of Neurosurgery, Loghman Hakim Hospital, Shaheed Beheshti University of Medical Sciences, Tehran, Islamic Republic of Iran.
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.
J Neurol Surg A Cent Eur Neurosurg. 2013 Dec;74 Suppl 1:e140-4. doi: 10.1055/s-0032-1330957. Epub 2013 Jan 10.
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.
McCune-Albright综合征（MAS）是一种罕见的多骨型纤维发育不良表现，伴有至少一种内分泌病，主要与性早熟和名为咖啡牛奶斑的色素沉着皮肤斑有关。我们报告了一名19岁男性McCune-Albright综合征的非典型表现，其罕见地合并了多骨型纤维发育不良和巨人症，且无咖啡牛奶斑和性早熟。他在过去3年中身高增长至202厘米，在之前几个月里增长更为迅速。体格检查仅发现轻度面部不对称；然而，计算机断层扫描（CT）发现广泛区域的骨质膨大，呈磨玻璃密度，增厚累及颅面骨和颅底。磁共振成像（MRI）发现垂体向右移位，伴有一个20毫米的垂体腺瘤。我们描述了诊断方法以及经鼻内镜经蝶窦入路切除肿瘤的过程。
