多骨性纤维发育不良
巨人症
麦库恩-奥尔布赖特综合征
纤维发育不良
医学
颅骨
性早熟
磁共振成像
解剖
颅面
蝶骨
垂体腺瘤
放射科
腺瘤
病理
内科学
精神科
激素
作者
Guive Sharifi,Farzaneh Sarvghadi,Mohammad Farhadi,Maryam Jalessi
出处
期刊:Journal of neurological surgery
[Thieme Medical Publishers (Germany)]
日期:2013-01-10
卷期号:74 (S 01): e140-e144
被引量:3
标识
DOI:10.1055/s-0032-1330957
摘要
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.
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