摘要
目的:总结4例McCune-Albright综合征(MAS)合并垂体生长激素-催乳素(GH-PRL)混合腺瘤患者的临床资料,分析其特点,探讨更有效的临床治疗和管理方法。方法:回顾性分析北京协和医院确诊的4例MAS合并垂体生长激素(GH)-催乳素(PRL)混合腺瘤患者的临床表现、生化指标、影像学特点、治疗及转归,并进行文献复习,分析外科手术及药物干预的疗效。结果:临床特点:4例患者中,3例为女性,均7岁前起病。骨纤维异常增殖症(FD)类型均为多骨型,且均累及颅面部。均有明显巨人症/肢端肥大症表现,3例出现明显的视力下降,1例视交叉明显受压。4例患者均无自发或触发泌乳。实验室检查:4例患者GH及胰岛素样生长因子Ⅰ(IGF-Ⅰ)明显升高,GH水平不被高糖抑制,血PRL均大于100 ng/ml,4例患者ALP水平明显升高,血钙、磷水平正常。影像学特点:4例患者垂体瘤均为大腺瘤,病例2、3出现单侧海绵窦包绕,病例4在第一次手术后残余肿瘤再次增大并包绕双侧海绵窦。4例患者头颅CT均显示颅面骨和颅底部多发FD。治疗与转归:4例患者均接受垂体瘤切除手术及静脉双膦酸盐治疗,并根据情况选择长效生长抑素类似物、多巴胺受体激动剂及其他对症治疗。较早诊断并起始治疗的2例患者治疗后达到部分缓解,另2例患者治疗后未缓解。结论:MAS合并GH-PRL混合腺瘤患者,起病年龄更早,更易出现视神经损害。早期诊断并给予合理的手术治疗有助于快速改善生化指标(GH、IGF-Ⅰ和PRL等),同时联合药物(生长抑素类似物和多巴胺受体激动剂)治疗,可以更好地维持生化指标正常或接近正常,最终改善患者的预后。
Objective To investigate the clinical characteristics of four McCune-Albright syndrome(MAS)patients associated with growth hormone(GH)-prolactin(PRL)pituitary adenoma,and explore more effective clinical treatment and management methods.Methods The clinical data of four patients with MAS and GH-PRL pituitary adenoma diagnosed by Peking Union Medical College Hospital were retrospectively analyzed,including clinical manifestations,biochemical parameters,radiographic characteristics,as well as treatment and prognosis.Literature review was also conducted to analyze the efficacy of surgical operations and drug intervention.Results Clinical characteristics:3 females and 1 male,all of whom had onset before the age of 7 years.The types of fibrous dysplasia of bone were all polyosseous and involved craniofacial region.There were obvious gigantism/acromegaly manifestations in all cases,3 cases had evident visual impairment and 1 case with optic chiasm compression.There was no spontaneous or triggered lactation in all 4 patients.Laboratory examination:GH and insulin-like growth factor-Ⅰ(IGF-Ⅰ)levels were significantly increased in 4 patients,GH levels were not inhibited by oral glucose tolerance test(OGTT),PRL levels were over 100 ng/ml,ALP levels were significantly increased in 4 patients,while serum calcium and phosphorus levels were remained normal.Imaging features:All pituitary adenomas in 4 patients were macroadenomas,with unilateral cavernous sinus enclosure in cases 2 and 3,and recurrence of macroadenoma with bilateral cavernous sinus enclosure in case 4 after the first operation.Four cases of skull CT showed multiple FD in craniofacial bone and skull base.Treatment and outcome:All 4 patients underwent pituitary adenoma resection and bisphosphonate therapy,and somatostatin analogues,dopamine receptor agonists and other treatments were selected according to the situation.Two patients with early diagnosis and initial treatment achieved partial remission after treatment,while the other two patients did not.Conclusion
作者
任高飞
朱惠娟
王林杰
阳洪波
卢琳
陈适
赵宇星
李冉
潘慧
姚勇
段炼
Ren Gaofei;Zhu Huijuan;Wang Linjie;Yang Hongbo;Lu Lin;Chen Shi;Zhao Yuxing;Li Ran;Pan Hui;Yao Yong;Duan Lian(Key Laboratory of Endocrinology of National Health Commission,Department of Endocrinology,Peking Union Medical College Hospital,Chinese Academy of Medical Science and Peking Union Medical College,Beijing 100730,China;Department of Neurosurgery,Peking Union Medical College Hospital,Beijing 100730,China)
出处
《中华内分泌代谢杂志》
CAS
CSCD
北大核心
2020年第8期696-702,共7页
Chinese Journal of Endocrinology and Metabolism
基金
国家重点研发计划(2016YFC091501)。
