McCune-Albright综合征合并垂体腺瘤的临床分析  

Clinical analysis of McCune-Albright syndrome presented with pituitary adenoma

作　　者：李智[1] 钱海燕[1] Raynald Liu 刘丕楠[1] 

机构地区：[1]首都医科大学附属北京天坛医院神经外科,100050

出　　处：《中华神经外科杂志》2017年第7期703-707,共5页Chinese Journal of Neurosurgery

摘　　要：目的探讨McCune-Albright综合征（MAS）合并垂体腺瘤的临床特点、治疗方法及预后。方法回顾性分析2004年4月至2016年11月首都医科大学附属北京天坛医院神经外科收治的9例MAS合并垂体腺瘤患者的临床资料。9例患者中，男4例，女5例；平均年龄为42岁（31～59岁）。4例血生长激素水平升高，4例血催乳素水平升高，1例血甲状腺素水平异常。8例行经蝶窦入路切除垂体腺瘤（4例应用神经内镜手术，其中2例采用术中导航辅助；4例应用显微镜手术），1例行开颅手术切除垂体腺瘤。随访时间为6～151个月。结果肿瘤全切除7例，近全切除1例，部分切除1例。8例患者术后内分泌异常情况明显缓解，1例术后生长激素水平仍偏高，1例术后出现新发垂体功能低下。术前出现进行性视力下降的6例患者术后视力明显改善。随访期内8例病情稳定，无一例出现垂体腺瘤复发，颅骨纤维异常增殖情况稳定无进展；1例患者因心脏病死亡。结论MAS的临床表现形式多样，临床易误诊和漏诊，其合并的垂体腺瘤和内分泌功能异常可加重病情，早期诊断并积极治疗内分泌功能异常、制定个体化的诊疗策略可提高疗效，改善患者的预后。Objective To investigate the clinical features, surgical treatment and follow-up results of McCune-Albright syndrome （MAS） presented with pituitary adenoma. Methods Nine patients with MAS and pituitary adenoma were admitted to Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University from April 2004 to November 2016 and the data were retrospectively reviewed. Among them, there were 4 males and 5 females. The average age was 42 years old with a range from 31 to 59 years old. Four patients had high level of blood growth hormone （GH）, 4 patients had high level of prolactin （PRL） and 1 patient had abnormal level of free thyroxine （FT4）. Transnasal resection of the pituitary adenoma was performed in 8 patients （endoscopy surgery in 4 cases, neuronavigation in 2 cases, and microsurgical resection in 4 cases）, and craniotomy was conducted in 1 patient. The follow-up lasted 6 - 151 months. At the follow-up, MRI and CT examinations were performed. Results Seven patients received gross total resection, 1 patient had subtotal resection and partial resection was achieved in 1 patient. Postoperatively, 8 patients reported significant improvement on their endocrine function, the GH level remained high in 1 case, and 1 patient developed newly-onset pituitary hypofunction. A total of 6 patients with progressive decrease of visual acuity before surgery had significant improvement postoperatively. During the follow-up, 8 patients＇conditions were stable. No tumor recurrence or progression of the fibrous dysplasia were observed. One patient died of heart disease. Conclusions The clinical presentation of MAS may vary greatly and lead to misdiagnosis or ignorance. Co-existing pituitary tumor and endocrine dysfunction could exacerbate the patient＇s conditions. Early diagnosis and effective treatment of abnormal endocrine function, and individualized therapy regimen could help improve the clinical outcomes.

关 键 词：MCCUNE-ALBRIGHT综合征 骨纤维发育不良 垂体肿瘤 神经外科手术 

分 类 号：R736.4[医药卫生—肿瘤]