颅咽管瘤的垂体内分泌功能管理

Management of pituitary endocrine function in craniopharyngioma

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，为罕见疾病，发病率约0．13／10万人年，占儿童颅内肿瘤的5％-10％。由于颅咽管瘤的位置靠近鞍区重要结构，如下丘脑、视交叉等，常常给外科治疗带来一系列难题；同时治疗引起一系列并发症如尿崩症、下丘脑性肥胖、腺垂体功能减退症等，给患者术后的生活质量带来了很大的影响。因此，颅咽管瘤已被视为一种慢性疾病，应对患者的神经内分泌系统进行长期临床监测、评估及多学科参与的综合治疗以改善患者的生活质量。

Craniopharyngioma is a kind of benign tumor of the relict squamous epithelial cells originated from the pituitary gland of cranial cheek pouch in the process of embryonic development. With an overall incidence of 0.13 cases per 100 000 person-years, they may represent up to 5% -10% of intracranial tumors in children. There is always debate regarding the appropriate treatment for craniopharyngiomas which often present symptomatically given their proximity to critical brain structures, and pose significant surgical challenges. And the complications caused by the treatment such as diabetes insipidus, hypothalamic obesity, and hypopituitarism, that throw out a challenge to the postoperative management and the quality of the patients. Craniopharyngioma should be recognized as a chronic disease requiring constant monitoring along with the clinical and multidisciplinary treatment in order to provide optimal care of surviving patients.