Dou Wanchen, Lin Ning, Ma Wenbin, Yang Yi, Zhu Huijuan, Sun Juan, Lian Wei, Yang Zhong, Li Wuyi, Wang Renzhi
Department of Neurosurgery, Peking Union Medical College Hospital, Beijing, China.
J Neurosurg. 2008 Jan;108(1):164-9. doi: 10.3171/JNS/2008/108/01/0164.
The McCune-Albright syndrome (MAS) is characterized by a clinical triad of polyostotic fibrous dysplasia, café-au-lait hyperpigmented macules, and hypersecretory endocrinopathies. Acromegaly is an uncommon manifestation of the endocrine disturbance associated with MAS, and the role of surgery in managing these cases has been a topic of debate. The authors present the case of a 35-year-old man with MAS who was also diagnosed with acromegaly, hyperprolactinemia, and pituitary macroadenoma. The patient had an 18-year history of fibrous dysplasia involving the right frontal bone and ribs as well as multiple endocrinopathies, but no cutaneous hyperpigmented macules. An oral glucose tolerance test demonstrated partial suppression of plasma levels of growth hormone (GH). The patient underwent transsphenoidal resection of the pituitary tumor, performed with assistance of neuronavigation, and tolerated the procedure well. After the surgery, both prolactin and GH levels returned to normal. These results suggest that neuronavigation-assisted transsphenoidal surgery can safely remove pituitary adenomas associated with MAS and successfully treat the underlying endocrine abnormalities.
McCune-Albright综合征（MAS）的特征为多骨纤维发育不良、牛奶咖啡斑色素沉着斑和分泌亢进性内分泌病三联征。肢端肥大症是与MAS相关的内分泌紊乱的一种罕见表现，手术在处理这些病例中的作用一直是一个有争议的话题。作者报告了1例35岁患有MAS的男性患者，该患者还被诊断为肢端肥大症、高泌乳素血症和垂体大腺瘤。患者有18年的纤维发育不良病史，累及右额骨和肋骨以及多种内分泌病，但无皮肤色素沉着斑。口服葡萄糖耐量试验显示生长激素（GH）血浆水平部分受抑制。患者在神经导航辅助下接受了垂体肿瘤经蝶窦切除术，手术耐受良好。术后，泌乳素和GH水平均恢复正常。这些结果表明，神经导航辅助经蝶窦手术能够安全地切除与MAS相关的垂体腺瘤，并成功治疗潜在的内分泌异常。
