儿童与青少年期颅咽管瘤患者神经内分泌功能受损特点比较研究

Comparative study on the characteristics of neuroendocrine dysfunction in children and adolescents with craniopharyngioma

目的比较鞍区肿瘤原位占位效应（ the mass effects in situ, MEIS）对儿童组与青少年组颅咽管瘤患者神经内分泌功能的影响。方法回顾性分析首都医科大学附属北京天坛医院2009年10月至2014年10月收治的颅咽管瘤患儿227例,按年龄分为儿童组及青少年组（儿童组167例、青少年组60例）,比较2组患儿由于鞍区肿瘤MEIS所致（术前）神经内分泌功能受损特点及受损程度的异同。结果（1）神经内分泌功能受损特点比较：下丘脑功能受损指标中,中枢性尿崩症比例最高（儿童组35.93%,青少年组31.67%）,其次为摄食异常与肥胖（儿童组19.76%,青少年组28.33%）,体温调节异常、睡眠异常与人格认知功能受损三项出现率均小于5%,2组间无统计学显著性意义（P=0.524）。腺垂体-靶腺轴系功能受损指标中,生长激素-胰岛素样生长因子Ⅰ（ IGF-Ⅰ）轴受损比例最高（儿童组64.07%,青少年组50.0%）,其次为青少年组垂体-性腺轴受损（53.33%）,高催乳素血症（儿童组31.14%,青少年组43.33%）,垂体-甲状腺轴受损（儿童组22.16%,青少年组28.33%）,垂体-肾上腺轴受损（儿童组20.36%,青少年组25%）,上述各项腺垂体-靶腺轴系功能受损比例（除垂体-性腺轴外）在2组间差异无统计学意义（P=0.475）。（2）神经内分泌功能受损程度比较：神经内分泌功能正常者儿童组占10.2%,青少年组占8.3%。神经内分泌功能有1~4项异常者儿童组占75.6%,青少年组占73.3%。神经内分泌功能有5项及以上异常者儿童组占14.4%,青少年组占18.4%。2组间神经内分泌功能受损程度比较无统计学意义（Z=-1.63,P=0.103）。结论鞍区肿瘤MEIS对颅咽管瘤儿童组与青少年组患儿神经内分泌功能受损及严重程度的影响无显著差异,提示系统地评价颅咽管瘤患儿下丘脑-垂体-靶腺轴功能对降低术后神经内分泌功能进一步紊乱的风险显得极为重要。

Objective To compare the impact of the mass effects in situ （ MEIS ） of the sella tumor on neuroendocrine function in children and adolescents with craniopharyngioma before surgery. Methods A total of 227 inpatients with craniopharyngioma in Beijing Tiantan Hospital, Capital Medical University, from October 2009 to October 2014, were retrospectively analyzed. These patients were divided into children group （ n = 167 ） and adolescent group（n=60） according to the age at the time of the first diagnosis. The clinical characteristics and damage degrees of neuroendocrine function by MEIS of sella tumor were analyzed and compared between these two groups before surgery. Result （1） Clinical characteristics of neuroendocrine function：Among hypothalamic dysfunctional manifestations, central diabetes insipidus showed the highest percentage（children group 35. 93%vs adolescent group 31. 67%）, followed by the abnormal appetite and obesity （ children group 19. 76% vs adolescent group 28. 33%）. The incidences of abnormal body temperature regulation, sleeping disorder, personality abnormality, and cognitive abnormality all were less than 5%. There were no statistical significant differences in the aforementioned hypothalamic dysfunction parameters between two groups （P=0. 524）. Among pituitary-target glands dysfunction parameters, growth hormonce （ GH ）-insulin-insulin like growth factor Ⅰ（ IGF-Ⅰ） axis dysfunction showed the highest percentage （ children group 64. 07% vs adolescent group 50. 0%）, followed by pituitary-gonad axis dysfunction in adolescent group （53. 33%）, hyperprolactinemia （ children group 31. 14% vs adolescent group 43. 33%）, pituitary-thyroid dysfunction（children group 22. 16%vs adolescent group 28. 33%）, pituitary-adrenal gland dysfunction（children group 20. 36%vs adolescent group 25%）. There were no statistical significant differences＆amp;nbsp;intheseabnormalpituitary-targetglandaxes（exceptpituitary-gonadaxis）betweentwogroups（P=0.475）. （2） Comparison of damage degrees of neuroendocrine dysfunction： The patients with normal neuroendocrine function accounted for 10. 2%in children group and 8. 3%in adolescent group. The patients with 1 to 4 items of neuroendocrine dysfunction accounted for 75. 6%in children group and 73. 3%in adolescent group. The patients with more than 5 items of neuroendocrine dysfunction accounted for 14. 4%in children group and 18. 4%in adolescent group. There were no significant differences between two groups（Z=-1. 63,P=0. 103）. Conclusions There were no significant differences in characteristics and damage degrees of MEIS of the sella tumor on neuroendocrine dysfunction between children and adolescents with craniopharyngioma. It suggests that systematical evaluation on hypothalamus-pituitary-targets axis function is very important for reducing the risks of further neuroendocrine dysfunction in young patients with craniopharyngioma after surgery.