塞鲁美替尼作为丛状神经纤维瘤的靶向治疗：文献综述。

IF 0.9 Q4 OPHTHALMOLOGY

Orbit-The International Journal on Orbital Disorders-Oculoplastic and Lacrimal Surgery Pub Date : 2025-02-28 DOI:10.1080/01676830.2025.2470989

Hammam A Alotaibi, Hunter Kl Yuen, Askar K Alshaibani, Eman A Alibrahim

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引用次数: 0

摘要

1型神经纤维瘤病以常染色体显性方式遗传。1型神经纤维瘤病的大致患病率为1:2500至1:3500，与种族无关。大约50%的1型神经纤维瘤病患者发展为丛状神经纤维瘤，这种病变有恶性转化的倾向。了解丛状神经纤维瘤病的病理生理学和神经纤维蛋白信号转导的含义，导致了针对该途径的靶向治疗的发展和使用。在这篇文章中，我们全面回顾了最近发表的关于使用selumetinib治疗1型神经纤维瘤病患者不能手术的丛状神经纤维瘤的文献。

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Selumetinib use as targeted therapy for plexiform neurofibroma: a comprehensive review of the literature.

Neurofibromatosis type 1 is inherited in an autosomal dominant fashion. The approximate prevalence of neurofibromatosis type 1 is 1:2500 to 1:3500 regardless of ethnic group. Around 50% of neurofibromatosis type 1 patients develop plexiform neurofibroma, a lesion that has a tendency for malignant transformation. Understanding the pathophysiology of plexiform neurofibromatosis and the implication of neurofibromin signal transduction has led to the development and use of targeted therapy on this pathway. In this article, we comprehensively reviewed the recently published literature on the use of selumetinib to treat inoperable plexiform neurofibroma in neurofibromatosis type 1 patients.

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来源期刊

Orbit-The International Journal on Orbital Disorders-Oculoplastic and Lacrimal Surgery OPHTHALMOLOGY-

CiteScore

2.40

自引率

9.10%

发文量

136

期刊介绍： Orbit is the international medium covering developments and results from the variety of medical disciplines that overlap and converge in the field of orbital disorders: ophthalmology, otolaryngology, reconstructive and maxillofacial surgery, medicine and endocrinology, radiology, radiotherapy and oncology, neurology, neuroophthalmology and neurosurgery, pathology and immunology, haematology.