Serafin Zbigniew, Machnikowska-Sokołowska Magdalena, Zawada Elżbieta, Bekiesińska-Figatowska Monika, Hałabuda Agata, Bladowska Marta, Wasilewski Grzegorz, Barańska Dobromiła, Szczurowska Agata, Karwacki Marek, Garus Katarzyna, Styczyński Jan
Faculty of Medical Sciences, Bydgoszcz University of Science and Technology, Bydgoszcz, Poland.
Department of Diagnostic Imaging, Radiology and Nuclear Medicine, Faculty of Medical Science in Katowice, Medical University of Silesia in Katowice, Poland.
Pol J Radiol. 2025 Jun 3;90:e286-e291. doi: 10.5114/pjr/203005. eCollection 2025.
Neurofibromatosis type 1 (NF1) is a genetically determined disorder with a complex clinical picture, including disorders of many systems and organs, that predisposes to the development of various cancers. Approximately 10-30% of patients with NF1 develop plexiform neurofibromas (PN), non-malignant tumours growing along the nerves, tending to infiltrate nerves and nerve plexuses, and may grow into internal organs, the spinal canal, and middle ear, deforming bones and various regions of the body. Magnetic resonance imaging (MRI) plays a key role in the management of individuals during the course of PN, as a screening tool at baseline, for surveillance (in individuals with known PN), to evaluate treatment response, and for preoperative assessment for surgical planning. The practical recommendations presented in this article aim to standardise the protocol for MRI examination of PN in the course of NF1 for the purpose of treatment in a clinical program in Poland. The protocol, covering volumetric MRI examination, imaging schedule, determination of the target change, the technique for measuring the volume of lesions, and suspected malignant transformation, is intended to be the minimum set of MRI sequences and views that must be included in every examination of these patients.
1型神经纤维瘤病（NF1）是一种由基因决定的疾病，临床表现复杂，累及多个系统和器官，易引发多种癌症。约10%-30%的NF1患者会发展为丛状神经纤维瘤（PN），这是一种沿神经生长的非恶性肿瘤，倾向于浸润神经和神经丛，还可能侵入内部器官、椎管和中耳，导致身体骨骼和各个部位变形。磁共振成像（MRI）在PN病程中对患者的管理起着关键作用，可作为基线筛查工具、用于监测（已知患有PN的患者）、评估治疗反应以及进行术前评估以制定手术方案。本文提出的实用建议旨在规范波兰临床项目中NF1病程中PN的MRI检查方案，以用于治疗。该方案涵盖了MRI容积检查、成像时间表、目标变化的确定、病变体积测量技术以及疑似恶性转化等内容，旨在成为每次对这些患者进行检查时必须包含的最少MRI序列和视图组合。
