Background

Optic Pathway Gliomas (OPG) are the most common brain tumor in Neurofibromatosis 1 patients (NF1). They are found along the optic pathway and may involve the optic nerves, chiasm, retro-chiasmatic structures, and the optic radiations. NF1 associate OPG (NF1-OPG) have variable presentation, disease course and response to treatment. The optimal management is patient-specific and should be tailored by a multidisciplinary team. Age, sex, histology, and molecular markers may be important factors in the individualized decision-making process. Chemotherapy is the first-line treatment in cases of progressive tumors, and visual preservation is the main goal of treatment.

Purpose

In this paper we will review the disease, practical management, and recent advances of NF1-OPG.

中文翻译：

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神经纤维瘤病1相关的视神经胶质瘤。

背景

视神经胶质瘤（OPG）是神经纤维瘤病1（NF1）患者中最常见的脑肿瘤。它们是沿着视神经通路发现的，可能涉及视神经，chi骨，后chi骨结构和视辐射。NF1相关性OPG（NF1-OPG）具有可变的表现，疾病进程和对治疗的反应。最佳管理是针对患者的，应由多学科团队量身定制。年龄，性别，组织学和分子标记可能是个性化决策过程中的重要因素。化学疗法是进行性肿瘤病例的一线治疗，视觉保护是治疗的主要目标。

目的

在本文中，我们将回顾NF1-OPG的疾病，实用治疗方法和最新进展。