围青春期垂体性巨人症患者的临床特点  

Clinical characteristics of patients with pituitary gigantism during puberty

作　　者：杨莹莹 罗云云 陈适 王林杰 姚勇[2] 邓侃[2] 朱惠娟 潘慧 YANG Ying-ying;LUO Yun-yun;CHEN Shi;WANG Lin-jie;YAO Yong;DENG Kan;ZHU Hui-juan;PAN Hui(Department of Endocrinology,Key Laboratory of Endocrinology of National Health Commission,Peking Union Medical College Hospital,CAMS&PUMC,Beijing 100730,China;Department of Neurosurgery,Peking Union Medical College Hospital,CAMS&PUMC,Beijing 100730,China)

机构地区：[1]中国医学科学院,北京协和医学院,北京协和医院内分泌科,国家卫生健康委员会内分泌重点实验室,北京100730 [2]中国医学科学院,北京协和医学院,北京协和医院神经外科,北京100730

出　　处：《基础医学与临床》2020年第9期1247-1251,共5页Basic and Clinical Medicine

基　　金：国家重点研发计划(2016YFC091501)。

摘　　要：目的比较不同起病年龄的垂体性巨人症患者临床特点。方法回顾性总结1990—2018年于北京协和医院诊断并随访的垂体性巨人症患者的临床资料。根据起病时间将患者分为起病年龄10~15岁及其他起病年龄组,比较两组的临床特点。结果28例垂体性巨人症患者,其中22名男性,中位病程为11.0年(7.0,13.8)。78.5%的患者首诊原因为身高增长过快,就诊时的平均身高为(190.2±17.5)cm,平均身高标准差分数(Ht SDS)为+4.5±1.6。92.9%的患者就诊时垂体磁共振成像(MRI)即提示肿瘤直径大于1 cm,其中42.9%大于3 cm。起病年龄10~15岁组的年均肿瘤最大直径为(2.9±1.3)cm,显著高于其他年龄组的(1.9±0.8)cm(P<0.05)。此外,年龄10~15岁组垂体巨大腺瘤的比例为62.5%,明显高于其他起病年龄组的16.7%(P<0.05)。长期随访数据显示:起病年龄10~15岁组的联合手术+药物+放疗的患者比例为31.2%,其他起病年龄组的采用联合疗法比例仅为8.3%。两组患者在长期随访治愈比例上的差异无统计学意义。结论围青春期的垂体性巨人症患者具有更大的腺瘤,更易发展为垂体巨大腺瘤,建议临床关注此类人群,必要时采用更积极的治疗手段。Objective To compare the clinical characteristics between pituitary gigantism patients with different age of onset.Methods This study retrospectively summarized the clinical data of patients diagnosed with pituitary gigantism in Peking Union Medical College Hospital from 1990 to 2018.Patients were divided into two groups according to the age of disease onset:patients with the age range of onset between 10~15 years old and the rest of patients.Results A total of 28 patients with pituitary gigantism were included in the study.Among them,22 patients were male,with a median duration of 11.0 years(7.0,13.8).78.5%of the patients had a rapid growth in height at the first consultation,with an average height of(190.2±17.5)cm and an average height SDS(Ht SDS)of+4.5±1.6 at the time of diagnosis.The pituitary MRI of 92.9%of patients demonstrated pituitary macroadenomas,of which 42.9%were pituitary giant adenomas.This study showed that the maximum diameter of the tumor in group with the age of onset between 10 to 15 years was significantly higher than that of control group(the age of onset between 10 to 15 years old:2.9±1.3 cm vs.control group:(1.9±0.8)cm,P<0.05),the proportion of pituitary giant adenoma was also significantly higher than control group(the age of onset between 10 to 15 years old:62.5%vs control group:16.7%,P<0.05).Long-term follow-up data showed that the proportion of combined treatment(surgery&medication&radiotherapy)in group with onset of age between 10 to 15 years old was higher than that of control group(the age of onset between 10 to 15 years old:31.2%vs control group:8.3%).However,the cure rate between two groups in the long-term follow-up did not reach statistical significance.Conclusions This study shows that pituitary gigantism patients during puberty are prone to have larger adenoma and are more likely to develop into a giant pituitary adenoma.It is recommended to focus on this population in clinical practice and more aggressive treatment should be considered if necessary.

关 键 词：垂体性巨人症 垂体生长激素腺瘤 垂体巨大腺瘤 

分 类 号：R584.11[医药卫生—内分泌]