Treatment of Plexiform Neurofibromas : Current Perspectives on Surgery and Medical Treatment.

IF 1.4 4区 医学 Q4 CLINICAL NEUROLOGY
Journal of Korean Neurosurgical Society Pub Date : 2025-05-01 Epub Date: 2025-04-30 DOI:10.3340/jkns.2025.0041
Jun Kyu Hwang, Se Hoon Kim, Dong-Seok Kim
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引用次数: 0

Abstract

Plexiform neurofibromas (PNFs), a critical clinical feature of neurofibromatosis type 1, mainly involve several peripheral nerve branches and extend widely, including the skin and bones and the internal organs. Determining the appropriate treatment is difficult. Additionally, they possess the potential to develop into malignant peripheral nerve sheath tumors (MPNSTs), which are linked to an extremely poor prognosis. Active treatment is critical in patients with symptoms or progressive tumor growth, especially in pediatric cases. Surgery remains the standard treatment for managing PNFs and MPNSTs; however, it has often demonstrated insufficient results because of its wide distribution and the frequent involvement of major organs. Selumetinib, a recently approved mitogen-activated protein kinase kinase inhibitor, is gaining traction in treating inoperable PNFs in children.

丛状神经纤维瘤的治疗:外科和内科治疗的最新进展。
丛状神经纤维瘤(Plexiform neurofibromas, PNFs)是1型神经纤维瘤病的一个重要临床特征,主要累及多个周围神经分支,并广泛扩展,包括皮肤、骨骼和内脏器官。确定适当的治疗方法是困难的。此外,它们具有发展成恶性周围神经鞘肿瘤(MPNSTs)的潜力,这与预后极差有关。对于有症状或肿瘤进展性生长的患者,尤其是儿科患者,积极治疗至关重要。手术仍然是治疗pnf和mpnst的标准治疗方法;然而,由于其广泛分布和经常涉及主要器官,它往往显示出不足的效果。Selumetinib是最近批准的一种丝裂原活化蛋白激酶抑制剂,在治疗儿童不能手术的pnf方面获得了越来越多的关注。
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来源期刊
CiteScore
2.90
自引率
6.20%
发文量
109
审稿时长
3-8 weeks
期刊介绍: The Journal of Korean Neurosurgical Society (J Korean Neurosurg Soc) is the official journal of the Korean Neurosurgical Society, and published bimonthly (1st day of January, March, May, July, September, and November). It launched in October 31, 1972 with Volume 1 and Number 1. J Korean Neurosurg Soc aims to allow neurosurgeons from around the world to enrich their knowledge of patient management, education, and clinical or experimental research, and hence their professionalism. This journal publishes Laboratory Investigations, Clinical Articles, Review Articles, Case Reports, Technical Notes, and Letters to the Editor. Our field of interest involves clinical neurosurgery (cerebrovascular disease, neuro-oncology, skull base neurosurgery, spine, pediatric neurosurgery, functional neurosurgery, epilepsy, neuro-trauma, and peripheral nerve disease) and laboratory work in neuroscience.
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