儿童下丘脑胶质瘤相关性早熟的临床分析

Hypothalamic glioma-associated precocious puberty in children

目的探讨儿童下丘脑胶质瘤相关性早熟的临床特点、治疗方法及随访效果。方法选取首都医科大学附属北京天坛医院小儿神经外科2009年1月至2013年12月收治的下丘脑胶质瘤病例中出现性早熟的7例患儿为研究对象，对其年龄、性别、病变类型、临床病程、治疗方法和治疗效果等进行回顾性分析。结果本组7例患儿中男4例，女3例，约占同期病例的1．75％（7／400）；就诊时年龄为2～8岁，平均年龄为5．1岁；病变部位为视交叉或下丘脑。例1～5及例7部分切除肿瘤；例6近全切除肿瘤。术后病理结果为WHO Ⅰ级5例，WHO Ⅱ及Ⅲ级各1例；内分泌实验室检查结果、体征及Tanner分期情况均符合中枢性性早熟诊断。本组平均随访40个月。其中4例采用促性腺激素释放激素类似物治疗，有效控制了患儿的性早熟症状，其余3例由于患儿个人原因未治疗，目前仍表现出性早熟的症状。结论除常见的下丘脑错构瘤、生殖细胞肿瘤以外，儿童下丘脑胶质瘤也可以继发性早熟症状，在临床诊治过程中要有足够的重视，术后随访过程中要注意检查并予以相应治疗。

Objective To investigate the clinical features, treatment strategies and follow-up effect of hypothalamic glioma-asseciated precocious puberty in children. Methods Seven children with precocious puberty in the hypothalamic glioma cases admitted to the Department of Pediatric Neurosurgery, Beijing Tiantan Hospital, Capital Medical University from January 2009 to December 2013 were selected as the research objects. Their age, gender, lesion types, clinical course, treatment methods, and efficacy were analyzed retrospectively. Results There were 3 girls and 4 boys in this group of children, accounting for about 1.75% （7/400） of the all cases during the same time period. Their ages at presentation ranged from 2 to 8 years （ mean age 5.1 years）. The lesion site was chiasm or hypothalamus. The tumors of patients 1 to 5 and 7 were resected partially, and the tumor of patient 6 was resected subtotally. The pathological results after procedure were 5 cases with WHO grade Ⅰ , 1 with WHO grade Ⅱ, and 1 with WHO grade Ⅲ. The endocrine laboratory results, signs, and Tanner stage were in accordance with the diagnosis criteria of central precocious puberty. The mean follow-up time was dO months in this group. Four of them were treated with gonadotropin-releasing hormone analogues. It effectively controlled symptoms of precocious puberty in children. The remaining 3 children still have the symptoms of precocious puberty now, because of personal reasons they did not receive treatment. Conclusions In addition to hypothalamic hamartomas and germ cell tumors, hypothalamic gliomas in children may also be secondary to the symptoms of precocious puberty. In the process of clinical diagnosis and treatment, enough attention should be paid to. In the postoperative follow-up process, examination should be paid to and be treated accordingly.