Resch R, Haas H, Schwarz S, Mayr U, Twerdy K, Hüttenberger H
Dtsch Med Wochenschr. 1981 Nov 6;106(45):1502-8. doi: 10.1055/s-2008-1070543.
Craniopharyngioma, primarily a benign tumour, is associated with a variety of serious problems due to tumour localization. Access to the tumour during surgery is difficult and total resection can rarely be achieved. Growth of the remaining tumour continues and symptoms of increased intracranial pressure, further visual field loss and hormonal disturbances reappear. However, conservative operation in conjunction with radical irradiation shows good results as regards tumour regression and survival with satisfactory quality of life. Irradiation with 50 Gy on average was performed in 5 out of 6 children with remaining tumour or tumour recurrence, and satisfactory results were obtained. In all cases tumour size regressed. Endocrine disturbances could be ascertained by the combined LHRH-TRH-insulin test and the clinical picture. In nearly every case there was finally a complete lack of hormones of the pituitary-hypothalamic axis. Substitution of various hormones became necessary in all cases. Meticulous surveillance of children still undergoing developmental maturation is mandatory. All six children are well and lead a nearly normal life.
颅咽管瘤主要是一种良性肿瘤,由于肿瘤的位置关系,会引发各种严重问题。手术中接近肿瘤困难,很少能实现全切。残留肿瘤会继续生长,颅内压升高、视野进一步丧失和激素紊乱等症状会再次出现。然而,保守手术联合根治性放疗在肿瘤消退和生存方面显示出良好效果,生活质量令人满意。6例残留肿瘤或肿瘤复发的儿童中,有5例平均接受了50 Gy的放疗,获得了满意的结果。所有病例肿瘤大小均有缩小。通过联合促黄体生成素释放激素-促甲状腺激素释放激素-胰岛素试验和临床表现可确定内分泌紊乱。几乎在每例病例中,最终垂体-下丘脑轴的激素完全缺乏。所有病例都需要补充各种激素。对仍在发育成熟阶段的儿童进行细致监测是必不可少的。所有6名儿童情况良好,过着几乎正常的生活。