颅咽管瘤术后致全垂体功能减退症伴肝硬化1例报道并文献复习

One Case of Hypohypopituitarism with Cirrhosis after Craniopharyngioma Was Reported and Reviewed in the Literature

摘要: 下丘脑–垂体是内分泌激素的中枢，下丘脑分泌相应释放激素刺激腺垂体激素合成及分泌增加，腺垂体激素作用于靶腺，由此构成完整的神经内分泌反射轴系。各种全身性疾病、手术、放疗、感染、炎症等病因引起的垂体分泌激素减少，导致靶腺功能减退的一系列临床症状称为垂体功能减退症。若所有激素全部受累及包括神经垂体，则称为全垂体功能减退症。肿瘤因素中垂体瘤、颅咽管瘤均是引起垂体功能减退的常见原因，可以是肿瘤的直接压迫，也可由手术后诱发。颅咽管瘤是由Rathke囊或颅咽管残存的胚胎上皮细胞分化而来，是儿童常见的颅内肿瘤，虽然在细胞学上属良性病变，但由于解剖位置毗邻视神经、垂体、下丘脑及颈动脉等重要结构，故全切手术容易造成患者终身神经内分泌功能障碍。尽管在治疗上使用了激素替代，但神经内分泌的复杂性决定了外援替代不可能完全模拟自身的激素分泌，从而影响患者生活质量。本文报道一例颅咽管瘤术后全垂体功能减退伴肝硬化的病例，并复习相关文献，以提高临床医师对垂体功能减退患者中激素替代治疗的认识。

Abstract: Hypothalamic-pituitary is the center of endocrine hormones. The corresponding secretion stimulates the synthesis and secretion of pituitary hormones. Adenular pituitary hormones act on the target glands, thus forming a complete neuroendocrine reflex axis. A series of clinical symptoms caused by various systemic diseases, surgery, radiotherapy, infection, inflammation and other etiologies, leading to hypotarget adenism are called hypopituitarism. If all hormones are involved, including the nerve pituitary gland, it is called hypohypopituitarism. In tumor fac-tors, pituitary tumor and craniopharyngioma are common causes of hypophyuitary function, which can be the direct compression of tumor or can be induced after surgery. Craniopharyn-gioma is by Rathke capsule or craniharyngeal tube remnant of embryonic epithelial cell differ-entiation, is a common intracranial tumor in children, although on cytology is benign lesions, but due to the anatomical location adjacent to the optic nerve, pituitary, hypothalamus and ca-rotid artery and other important structure, total surgery is easy to cause lifelong neuroendo-crine dysfunction in patients. Despite the therapeutic use of hormone replacement in patients, the neuroendocrine complexity determines that additional replacement treatments cannot completely mimic their own hormone secretion, thereby affecting the patient’s quality of life. We report a case of postoperative total hypopituitarism with cirrhosis after craniopharyngioma, and review the relevant literature to improve clinicians’ awareness of hormone replacement therapy in patients with hypopituitarism.