与VHL相关的神经内分泌新生体的新进展
Marina Tsoli1, Maria Panagaki2, Elisavet Tasouli2
1Neuroendocrine Tumour Unit, ENETS Centre of Excellence, 1st Department of Propaedeutic and Internal Medicine, Laiko Hospital, National and Kapodistrian University of Athens, Agiou Thoma 17, Athens, 11527, Greece. martso.mt@gmail.com.
Current oncology reports
|January 5, 2025
概括
·希佩尔-林多 (VHL) 综合征可以导致神经内分泌新生瘤 (NEN),特别是胰腺NEN和染细胞瘤. 管理包括监视,对较大的瘤进行手术,以及新兴的治疗方法,如贝尔祖提凡.
科学领域:
- 神经内分泌学神经内分泌学
- 在瘤学瘤学.
- 遗传学 遗传学 是一个
背景情况:
- ·希佩尔-林道综合征 (VHL) 是一种遗传性疾病,使个体易患各种瘤.
- 神经内分泌瘤 (NENs) 是VHL患者的一个重大问题.
研究的目的:
- 审查目前对VHL综合征中NEN的流行病学,诊断和管理的理解.
- 巩固关于VHL患者胰腺NEN (vPNEN) 和乳红细胞瘤的知识.
主要方法:
- 对VHL相关的NEN的流行病学数据,诊断标准和治疗策略的文献综述.
- 分析当前的治疗指南和新兴的治疗选择.
主要成果:
- 胰腺NENs (vPNENs) 在8-17%的VHL患者中发生,通常是多焦点和非功能性;对于瘤>3厘米或快速生长的瘤,显示手术切除.
- 在VHL类型2中的血红细胞瘤经常是双边的,noradrenergic和复发的;[131I]-MIBG和sunitinib是既定的治疗方法,而贝尔祖提凡显示出希望.
- 作为HIF-2α抑制剂的Belzutifan是一种潜在的治疗方法,用于VHL相关的NEN转移性疾病.
结论:
- 终身监测和多学科管理对于NEN患者VHL患者的最佳结果至关重要.
- 早期检测和量身定制的治疗策略对于管理vPNEN和染细胞瘤至关重要.
- 像贝尔祖蒂芬这样的新兴疗法为管理先进或转移性VHL相关的NEN提供了新的希望.
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