神经纤维瘤病的MRI影像学特点分析

Analysis of MRI features of neurofibromatosis

目的探讨神经纤维瘤病(NF)的MRI影像学特点。方法回顾性分析2018年1月至2022年3月于北京房山区良乡医院确诊的39例NF患者的临床及影像学资料,其中NFⅠ型7例,NFⅡ型32例,总结两者的临床表现和MRI影像特点。结果NFⅠ型患者中,4例MRI表现为多发不明原因亮点、双侧视神经胶质瘤,2例MRI表现为眶周丛状神经纤维瘤,1例MRI表现为外周神经分布区多发丛状神经纤维瘤;NFⅡ型患者中,MRI主要以听神经瘤多见,共22例(17例为双侧,5例为单侧),并伴发三叉神经鞘瘤11例,脑膜瘤15例,脊膜瘤2例,眼眶血管瘤1例,椎管内外神经源性肿瘤22例。结论MRI对鉴别NFⅠ型及NFⅡ型具有重要诊断价值。NFⅠ型主要表现为多发不明原因亮点、视神经胶质瘤及多发丛状神经纤维瘤;NFⅡ型主要表现为双侧听神经瘤,并多伴发三叉神经鞘瘤、脑膜瘤、脊膜瘤、神经源性肿瘤等。

Objective To explore MRI manifestations of neurofibromatosis(NF).Methods The clinical and imaging data of 39 patients diagnosed with NF in Liangxiang Hospital of Fangshan District,Beijing from January 2018 to March 2022 were retrospectively analyzed,including 7 patients with NFⅠ and 32 patients with NFⅡ.The clinical manifestations and MRI imaging characteristics of both patients were summarized.Results In patients with NFⅠ,the MRI showed 4 cases with significant numbers of so-called unidentified bright objects and bilateral optic gliomas;2 cases with plexiform neurofibroma located in para-orbital soft tissue;1 case with plexiform neurofibroma located in peripheral nerve distribution area.In patients with NFⅡ,the MRI showed 22 cases with acoustic neuroma(bilateral in 17 cases,unilateral in 5 cases);11 cases with trigeminal schwannomas;15 cases with multiple meningiomas;2 cases with spinal meningioma;1 case with orbital hemangioma;22 cases with neurogenic tumors.Conclusion MRI has important diagnostic value in distinguishing NFⅠ from NFⅡ,the distinctive features of NFⅠ in MRI are significant numbers of unidentified bright objects,with optic gliomas,and multiple plexiform neurofibroma,NFⅡ is characterized by bilateral acoustic neuromas,with trigeminal schwannomas,multiple meningiomas,spinal meningioma and neurogenic tumors.