颅咽管瘤术后下丘脑垂体功能管理的临诊应对  

Approach to the management of hypothalamic pituitary function in patients with craniopharyngioma after operation

作　　者：隋婧[1] 史沛杰 郭辉[1] 何明倩 施秉银[1] Sui Jing;Shi Peijie;Guo Hui;He Mingqian;Shi Bingyin(Department of Endocrinology, the First Affiliated Hospital of Xi′an Jiaotong University, Xi′an 710061, China)

机构地区：[1]西安交通大学第一附属医院内分泌科,710061

出　　处：《中华内分泌代谢杂志》2018年第12期1042-1047,共6页Chinese Journal of Endocrinology and Metabolism

摘　　要：颅咽管瘤是起源于垂体胚胎发育过程中颅颊囊残存的鳞状上皮细胞的良性肿瘤,临床症状主要以视力障碍、颅内压增高及下丘脑垂体内分泌紊乱为主,表现为视力下降、头痛、恶心、生长迟缓、性腺功能低下等。尽管颅咽管瘤被视为一种良性肿瘤,但由于瘤体毗邻下丘脑、垂体柄、视交叉等重要结构,且具有明显复发倾向,同时治疗后可能引起一系列并发症如腺垂体功能减退症、尿崩症、下丘脑性肥胖等,严重影响患者术后的生活质量,也给外科治疗及术后下丘脑垂体功能管理带来了巨大挑战。本文通过对1例临床诊断的颅咽管瘤经2次手术治疗和术后放射治疗后垂体内分泌功能紊乱的临床特点进行分析,结合国内外研究进展探讨及时有效的诊治思路和诊疗方法。Craniopharyngiomas develop from remnant epithelial cells of Rathke′s pouch in the sella/parasella region with low histological grade. Clinical manifestations are related to visual impairment, increased intracranial pressure, and hypothalamic/pituitary deficiencies, including vision loss, headache, nausea, growth retardation, and hypogonadism. Despite benign epithelial tumors, craniopharyngiomas are adjacent to critical brain structures including hypothalamus, pituitary stalk, chiasma opticum and have recurrence tendency. Treatment-related complications including hypothalamic/pituitary deficiencies, diabetes insipidus, and hypothalamic obesity are major risk factors for reduced quality of survival and throw out a great challenge to the surgical treatment and the postoperative management. Clinical features of one case craniopharyngiomas with manifestation of severe hypopituitarism are described with the aim of exploring effective therapies.

关 键 词：颅咽管瘤 垂体功能减退症 尿崩症 下丘脑肥胖 治疗 

分 类 号：R739.4[医药卫生—肿瘤]