摘要目的:探讨垂体性巨人症的临床特征和长期随访结果。方法:回顾性分析1990年1月至2018年12月31日于中国医学科学院北京协和医院诊治、术后随访时间≥1年的垂体性巨人症患者，共24例。其中采用单纯单次手术9例、手术联合药物3例、手术联合放疗4例、手术联合药物和放疗7例、仅药物治疗1例。分析患者的基本信息、实验室和影像学资料。根据随访资料将患者分为治愈组和非治愈组，比较两组患者的差异。结果:24例患者中，20例(83.3%)为男性；中位起病年龄为13( P25， P75：10，14)岁，中位诊断年龄为17.0( P25， P75：14.0，21.0)岁，中位病程为4.0( P25， P75：2.0，7.0)年。19例(79.2%)患者的首发症状为身高的快速增长。24例均为垂体腺瘤，其中21例(87.5%)为垂体大腺瘤。24例患者的中位随访时间为3.0( P25， P75：2.0，5.7)年，其中治愈组14例(58.3%)，未治愈组10例(41.7%)。与治愈组比较，未治愈组的中位起病年龄晚[分别为11( P25， P75：7，13)岁、14( P25， P75：13，15)岁]，诊断时中位生长激素水平高[分别为15.2( P25， P75：10.0，22.5) μg/L、58.8( P25， P75：20.8, 165.9)μg/L],肿瘤最大直径大[分别为(2.2 ±1.2)cm、(3.6±0.8)cm]、采用手术＋药物＋放射治疗的比例高(分别为1/14和6/10)，差异均有统计学意义(均 P<0.05)。 结论:初步研究显示，垂体性巨人症具有男性和垂体大腺瘤所占比率高、和垂体大腺瘤易延误诊断的特点；随访资料显示其治疗达标率低，建议临床上关注生长过快的儿童青少年，实现早诊断、早治疗。

abstractsObjective:To explore the clinical characteristics and long-term follow-up results of pituitary gigantism.Methods:We performed a retrospective study on 24 patients with pituitary gigantism who were diagnosed and treated at Chinese Academy of Medical Science and Peking Union Medical College, Peking Union Medical College Hospital from January 1990 to December 2018 and underwent follow-up lasting for at least 1 year. Among them, merely one surgical operation was performed in 9 cases, operation combined with medication in 3, operation combined with radiotherapy in 4, operation combined with drugs and radiotherapy in 7, and merely medication in 1. We analyzed the patient’s basic information, laboratory and imaging data. The patients were divided into the cured group and the non-cured group according to follow-up data, and the differences between the two groups were analyzed.Results:Of the 24 patients, 20 (83.3%) were males, the median age of onset was 13( P25, P75: 10, 14) years, the median age of diagnosis was 17.0( P25, P75: 14.0, 21.0) years, and the median duration of disease was 4.0 ( P25, P75: 2.0, 7.0) years. The first symptom of 19 patients (79.2%) was rapid growth in height. Twenty-four cases were pituitary adenomas, of which 21 (87.5%) were pituitary macroadenomas. The median follow-up time for 24 patients was 3.0 ( P25, P75: 2.0, 5.7) years, of which 14 were in the cured group (58.3%) and 10 in the non-cured group (41.7%). Compared with the cured group, the non-cured group had older median age of onset [14 ( P25, P75: 13, 15) years vs. 11 ( P25, P75: 7, 13) years] old, higher median growth hormone level at diagnosis [58.8 ( P25, P75: 20.8, 165.9) μg/L vs. 15.2 ( P25, P75): 10.0, 22.5 μg/L], larger maximal diameter of the tumor (3.6 ± 0.8 cm vs. 2.2 ± 1.2 cm), higher proportion of surgical treatment combined with medication and radiotherapy (6/10 vs. 1/14, P=0.009), and the differences were statistically significant (all P<0.05). Conclusion:Preliminary studies show that pituitary gigantism has the characteristics including male predominance, relatively high incidence of pituitary macroadenomas and high chance of delayed diagnosis. The follow-up data show that the cure rate is low, and it is recommended to pay clinical attention to children and adolescents who grow too fast for the purpose of early diagnosis and treatment.