儿科头部和部症状与多种内分泌瘤综合征相关
T Jellins1, M Hill2, J D Prager3
1University of Colorado School of Medicine, Aurora, CO, USA.
International journal of pediatric otorhinolaryngology
|August 21, 2023
概括
患有多重内分泌新陈代谢 (MEN) 综合征的儿科患者通常需要头部和部手术. 早期的外科干预可能是必要的男性类型2 (MEN2) 患者,即使那些风险较低,以管理骨髓性甲状腺癌.
科学领域:
- 儿科内分泌学 儿科内分泌学
- 手术瘤学手术瘤学
- 遗传学 是一个遗传学.
背景情况:
- 多重内分泌新陈代谢 (MEN) 综合征是一种遗传性疾病,使儿童易患内分泌瘤.
- 头部和部内分泌瘤在儿科男性群体中是一个重大问题.
研究的目的:
- 在儿科头部病例中调查 MEN 1 型 (MEN1) 和 MEN 2 型 (MEN2) 的特定瘤表现.
- 根据当前的指导方针,评估手术干预的时间.
主要方法:
- 在单一机构对儿科MEN患者 (2005-2022) 的回顾性审查.
- 对遗传确认,临床表现,外科手术和组织病理学发现的分析.
主要成果:
- 甲状腺恶性瘤,主要是髓性甲状腺癌 (MTC),在36%的甲状腺切除样本中被发现,通常是隐藏的,并且比ATA指南预测的更早发生.
- 虽然MEN2患者比MEN1患者更早接受手术,但年龄大于目前的ATA指南所建议的年龄.
- 在接受中央部剖析的MEN2B患者中,有50%的宫淋巴结转移被发现.
结论:
- 在这个队列中,近60%的儿科MEN患者需要头部和部手术.
- 目前的MEN1指南似乎是合适的,但儿童MEN2患者可能会从MTC的早期手术中受益,无论风险类别如何.
- 多学科的方法和对早期手术需求的认识对于儿科MEN2管理至关重要.
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