医学课件颅咽管瘤术后rhGH治疗
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颅咽管瘤治疗方法
颅咽管瘤是一种比较罕见的神经源性肿瘤,它通常发生在颅咽管附近的组织。
对于颅咽管瘤的治疗方法,主要包括下面几种:
1.手术切除:手术切除是治疗颅咽管瘤的主要方法,尤其是对于较大的肿瘤,需要通过手术进行切除。
手术需要在健康的组织和神经系统中进行,因此需要经验丰富的神经外科医生进行,以最小化手术风险。
2.放射治疗:放射治疗是治疗颅咽管瘤的另一种选择,尤其是对于无法切除的肿瘤来说。
放射治疗可以消除肿瘤的细胞,减缓瘤体生长。
3.化学治疗:化学治疗并不是颅咽管瘤治疗的主要方法,但它有时可以与手术或放射治疗结合。
化疗可以通过药物减缓瘤体生长,并控制其扩散。
需要注意的是,颅咽管瘤往往比较难以治疗,因此早期发现并进行治疗非常重要。
治疗前需要进行全面的医学检查,以了解瘤体的位置、大小和生长速度,从而制定最佳治疗方案。
2023生长激素替代治疗安全性的共识解读——颅咽管瘤术后摘要2022年,欧洲内分泌学会更新了《在癌症、颅内肿瘤及垂体瘤幸存者中生长激素替代治疗的安全性:共识声明》,共包含了15条国际关注的安全性问题和态度声明。
在欧洲内分泌学会的支持下,生长激素研究学会邀请了16个国家涵盖10个专业学会的55位专家组成国际专家小组,讨论了癌症和颅内肿瘤患者生长激素替代安全性的问题,并形成国际专家共识。
【关键词】颅咽管瘤;术后;生长激素缺乏症;生长激素替代随着临床上对于儿童和成人生长激素(GH)缺乏症(GHD)的逐步重视,生长激素替代治疗的安全性引起了广泛讨论。
重组人GH(rhGH)最早被批准用于儿童生长激素缺乏症,1996年,rhGH被批准治疗成人生长激素缺乏症,目前临床上积累了大量的应用经验,总体上治疗是安全的。
GHD最常见的原因是儿童特发性生长激素缺乏、成人下丘脑-垂体肿瘤和(或)治疗后引起的生长激素缺乏。
成人下丘脑-垂体部位常见的肿瘤是垂体腺瘤和颅咽管瘤。
颅咽管瘤治疗以手术、放疗为主,治疗后常伴有生长激素缺乏症。
临床医师迫切希望能够改善颅咽管瘤术后患者的GHD,GH替代治疗可以促使儿童GHD和儿童向成人过渡时期的GHD身高增长及骨骼和肌肉成熟,可以使成人GHD在身体成分、运动能力、骨骼、心血管功能等方面获益,可以提高患者的生活质量。
2022年,欧洲内分泌学会更新了《在癌症、颅内肿瘤及垂体瘤幸存者中生长激素替代治疗的安全性:共识声明》,共包含了15条国际关注的安全性问题和态度声明。
本文结合最新共识对颅咽管瘤术后GHD患者使用GH替代治疗的安全性进行综述和讨论。
1 颅咽管瘤的发病率颅咽管瘤是起源于垂体胚胎发育过程中颅颊囊残存鳞状上皮细胞的良性肿瘤,全球每年每100万人中有0.5~2.5个新病例,颅咽管瘤占成人颅内肿瘤的1.2%~4.6%[2]。
颅咽管瘤占儿童颅内肿瘤的5.6%~15.0%,占儿童鞍区肿瘤的54%,是儿童常见的颅内肿瘤。
儿童颅咽管瘤术后重组人生长激素替代治疗疗效及安全性对照研究郑章乾;支涤静;沈水仙;罗飞宏;赵诸慧;陆忠;叶蓉;程若倩;李晓静【摘要】目的观察儿童颅咽管瘤(CP)术后致身材矮小者使用重组人生长激素(rhGH)治疗的疗效及安全性.方法纳入CP术后在复旦大学附属儿科医院内分泌遗传代谢科定期随访的患儿.分为rhGH治疗组和rhGH未治疗组.CP术后1~3个月病情稳定后首次随访患儿垂体功能,之后每3个月随访身高、体重、甲状腺功能和生长因子(IGF-1、IGF-BP3),比较两组治疗前后身高变化.每6~12个月随访头颅MRI,观察两组患儿CP复发及继发肿瘤发生情况.结果 CP术后患儿共18例,男、女各9例,均存在生长激素缺乏症(GHD).rhGH治疗组和rhGH未治疗组分别为6和12例,平均手术年龄分别为(10.1±4.2)和(10.1±4.0)岁.16/18例(88.9%)存在垂体功能减低,其中12例(75.0%)伴甲状腺功能减低,9例(56.2%)伴中枢性尿崩症,4例(25.0%)伴性发育延迟,11例(68.8%)伴促肾上腺皮质激素下降.rhGH治疗组中2例单用rhGH治疗,4例同时使用左旋甲状腺素、醋酸去氨加压素和氢化可的松治疗,开始给予rhGH治疗的时间为术后(3.5±2.4)年,平均治疗时间为(2.6±2.2)年,治疗前身高增长速度(HV)为每年(3.1±1.0)cm,身高标准差(HTSDS)为(-2.63±0.93),至本文观察时点HV为每年(12.0± 1.10)cm,HTSDS为(-0.21±1.39),生长因子水平较治疗前明显上升.rhGH未治疗组治疗前HV为每年(3.2±0.9)cm,HTSDS为(-2.44±0.62),至本文观察时点HV为每年(3.8±1 0)cm,HTSDS为(-3.76±0.97),生长因子水平治疗前后差异无统计学意义.两组随访头颅MRI均未见异常.结论儿童CP 术后可出现多种内分泌激素异常,GH替代治疗可明显改善患儿身高,治疗期间未见原肿瘤复发及继发肿瘤发生.%Objective To observe the safety and effectiveness of recombinant human growth hormone( rhGH ) replacementin postoperative craniopharyngioma ( CP ) children with short stature. Methods Children with pre-diagnosed CP and attending followed-up after surgery were recruited in Children' s Hospital of Fudan University. A retrospective case-control study was conducted. Children were divided into rhGH treatment group and control group. They received first follow - up with 1 to 3 months after surgery and the pituitary hormone functions were measured. Body height, body weight, IGF-1, IGF-BP3 and thyroid function were measured every 3 months and the change in body height after treatment was compared between two groups. Head magnetic resonance( MR ) was carried out every 6 to 12 months to observe the difference of the tumor recurrence and second malignant neoplasm between two groups. Results Eighteen postoperative CP children ( 9 girls and 9 boys ) were diagnosed as growth hormone deficiency ( GHD ) and recruited. There were 6 patients and 12 patients in rhGH treatment group and the control group, respectively. The average age at surgery was ( 10. 1 ±4. 2 ) years and ( 10. 1 ±4. 0 ) years in treatment group and the control group, respectively. 16 out of 18 patients ( 88. 9% ) were with multiple pituitary hormone deficiency. Among the 16 patients, 12 of them ( 75% ) also had hypothyroidism, 9 of them ( 56. 2% ) had central diabetes insipidus, 4 of them ( 25% ) were with delayed puberty and 11 of them ( 68. 8% ) had adrenal insufficiency. In rhGH treatment group, 2 patients were treated by rhGH only, 4 patients were treated by rhGH combined with L-thyroxine, cortisone and Minirin. The average time of 6 patients starting rhGH replacement was ( 3. 5 ± 2.4 ) years after surgery and the averagedura tion of treatment was ( 2. 6 ± 2. 2 ) years. Before treatment, the height velocity ( HV ) was (3.1 ±1.0) cm per year, body height standard deviation( HTSDS ) was ( - 2. 63 ± 0. 93 ), at the end of observation of this study, the HV was ( 12.0 ±1. 1 ) cm pe r year, HTSDS was ( -0.21 ±1.39), serum IGF-1 and IGF-BP3 levels were significantly elevated. In control group, before treatment, the HV was ( 3. 2 ±0. 9 ) cm per year, HTSDS was ( - 2.44 ± 0.62). At the end of observation of this study, the HV was 3. 8 ± 1.0 cm per year, HTSDS was ( -3.76±0.97), serum IGF-1 and IGF-BP3 levels were not significantly changed. Recurrent tumor or second malignant neoplasm was not found by head MR in both groups. Conclusions Multiple pituitary hormone deficiency could occur in postoperative craniopharyngioma children and rhGH replacement could improve body height. No recurrent tumor or second malignant neoplasm was found during treatment.【期刊名称】《中国循证儿科杂志》【年(卷),期】2013(008)003【总页数】4页(P201-204)【关键词】颅咽管瘤;重组人生长激素;安全性【作者】郑章乾;支涤静;沈水仙;罗飞宏;赵诸慧;陆忠;叶蓉;程若倩;李晓静【作者单位】复旦大学附属儿科医院内分泌遗传代谢科,上海,201102;复旦大学附属儿科医院内分泌遗传代谢科,上海,201102;复旦大学附属儿科医院内分泌遗传代谢科,上海,201102;复旦大学附属儿科医院内分泌遗传代谢科,上海,201102;复旦大学附属儿科医院内分泌遗传代谢科,上海,201102;复旦大学附属儿科医院内分泌遗传代谢科,上海,201102;复旦大学附属儿科医院内分泌遗传代谢科,上海,201102;复旦大学附属儿科医院内分泌遗传代谢科,上海,201102;复旦大学附属儿科医院内分泌遗传代谢科,上海,201102【正文语种】中文儿童最常见的颅内肿瘤有颅咽管瘤( craniopharyngioma,CP) 和垂体腺瘤等,手术切除和伽马刀治疗是目前治疗颅内肿瘤的首选方法[1]。