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病例报告
MRI诊断眶内视神经鞘非典型脑膜瘤一例
宋晓涵 王丽君 于伟凡

Cite this article as: Song XH, Wang LJ, YU WF. MRI diagnosis of atypical optic nerve sheath meningioma in orbit: One case report[J]. Chin J Magn Reson Imaging, 2022, 13(9): 106-107.本文引用格式:宋晓涵, 王丽君, 于伟凡. MRI诊断眶内视神经鞘非典型脑膜瘤一例[J]. 磁共振成像, 2022, 13(9): 106-107. DOI:10.12015/issn.1674-8034.2022.09.022.


[摘要] 本研究为回顾性研究,经大连医科大学附属第一医院伦理委员会批准,免除受试者知情同意,批准文号:PJ-KS-KY-2022-89。
[关键词] 眶内脑膜瘤;视神经鞘脑膜瘤;非典型脑膜瘤;磁共振成像
[Keywords] intra-orbital meningioma;optic nerve sheath meningioma;atypical meningioma;magnetic resonance imaging

宋晓涵    王丽君 *   于伟凡   

大连医科大学附属第一医院放射科,大连 116011

*王丽君,E-mail:wanglj345@163.com

作者利益冲突声明:全部作者均声明无利益冲突。


收稿日期:2022-02-22
接受日期:2022-09-07
中图分类号:R445.2  R739.45 
文献标识码:B
DOI: 10.12015/issn.1674-8034.2022.09.022
本文引用格式:宋晓涵, 王丽君, 于伟凡. MRI诊断眶内视神经鞘非典型脑膜瘤一例[J]. 磁共振成像, 2022, 13(9): 106-107. DOI:10.12015/issn.1674-8034.2022.09.022

       本研究为回顾性研究,经大连医科大学附属第一医院伦理委员会批准,免除受试者知情同意,批准文号:PJ-KS-KY-2022-89。

       患者女,39岁,无明显诱因出现右侧眼球突出伴视力渐进性下降2年,近半年眼球突出加重,视力下降明显。专科检查:右眼无光感,右眼球突出眼眶约2.5 cm,眼睑闭合不全,结膜充血水肿,暴露性角膜炎,晶状体混浊。MRI检查:右眼眶肌锥内间隙约4 cm×3 cm×2 cm的肿物,长轴与视神经平行,形态欠规整,呈分叶状,信号大部分较均匀,T1WI呈等灰质信号(图1A),T2WI呈稍高信号(图1B),增强扫描肿瘤呈显著强化(图1C1F1G),其内隐约可见视神经(图1C),轮廓模糊,扩散加权成像(diffusion weighted imaging, DWI)呈高信号(图1D),表观扩散系数(apparent diffusion coefficient, ADC)值降低(图1E),眼球后壁视神经乳头明显增大,右侧眼外肌受压变扁、移位,上直肌局部与肿物紧密接触、显示不清(图1G)。影像诊断:右侧眼眶内占位,视神经及眼球均受累,考虑低度恶性肿瘤。

       手术和病理:术中见肿瘤位于眼眶内,包绕视神经生长,色乳白、质韧、呈串珠样改变、囊膜包裹。肉眼见肿瘤组织浸润视神经和巩膜。镜下见椭圆形及短梭形肿瘤细胞,排列成片状及束状,局部可见同心圆状旋涡结构,细胞大小一致,胞浆嗜酸,可见核沟,核分裂象不易见,间质血管增生明显(图1H)。免疫组化:Caldesmon(+),CK(-),EMA(+),Ki-67(+10%),P53(野生型),PR(+),S-100(-),SMA(-),SOX-10(-)。病理诊断:非典型脑膜瘤,侵犯视神经及眼球巩膜。

图1  女,39 岁,视神经鞘非典型脑膜瘤。1A:横轴位T1WI 示右侧眼球明显突出,眶内球后肿物呈分叶状,信号较均匀,呈等灰质信号;1B:横轴位T2WI 呈稍高信号;1C:横轴位T1WI 增强示病变明显强化,稍欠均匀,其内隐约可见视神经(箭),轮廓模糊;1D:扩散加权成像(DWI)示肿瘤呈高信号;1E:表观扩散系数(ADC)图显示ADC值降低;1F:冠状位增强示分叶状肿物邻近眼外肌受压变扁;1G:矢状位增强示肿瘤位于右眶肌锥内间隙,上直肌局部与肿物分界不清(箭);1H:病理图(HE ×400)可见椭圆形及短梭形肿瘤细胞,排列成片状及束状,局部可见同心圆状旋涡结构,细胞大小一致,胞浆嗜酸,可见核沟,核分裂象不易见,间质血管增生明显。
Fig. 1  Female, 39-year-old patient with atypical meningioma of the optic nerve sheath. 1A: The right eyeball was obviously protruded on the transverse axial T1WI, and the retrobulbar mass in the orbit was lobulated with uniform iso-gray matter signal; 1B: It was slightly high signal on transverse T2WI; 1C: The transverse contrast enhanced axial T1WI showed that the lesion was significantly enhanced, slightly uneven. The optic nerve (arrow) was faintly visible in the lesion, and the contour was blurred; 1D: The tumor showed hyperintensity on diffusion weighted imaging (DWI); 1E: The apparent diffusion coefficient (ADC) map showed that the ADC value was decreased; 1F: Coronal contrast enhancement MRI showed that the extraocular muscles adjacent to the lobulated mass were compressed and flattened; 1G: Sagittal contrast enhancement MRI showed that the tumor was located in the right intraconal space, and the local boundaries between the superior rectus muscle and the mass were unclear (arrow); 1H: The pathological image (HE ×400) showed oval and short spindle tumor cells arranged in sheets and bundles with concentric circular vortex structure locally. The cells were of the same size, the cytoplasms were eosinophilic, nuclear furrows were visible, mitotic phenomenons were not easily seen, and interstitial vascular hyperplasia was obvious.

讨论

       非典型脑膜瘤在2021年WHO中枢神经系统肿瘤分类归属于WHO 2级,为介于良性脑膜瘤和恶性脑膜瘤的中间类型,侵袭性较强、预后差、术后易复发[1]。颅内非典型脑膜瘤的发病率较低,仅占颅内脑膜瘤总数的5%~7%[2, 3],而发生于眶内视神经鞘的非典型脑膜瘤则更加罕见,目前对视神经鞘非典型脑膜瘤的报道仅见于儿童,多与2型神经纤维瘤病相关[4, 5],而成人型未见相关报道。视神经鞘脑膜瘤是起源于视神经鞘蛛网膜细胞的肿瘤,一般认为其多发生于成年女性[6],与性激素有关,本病例为39岁女性,符合眶内视神经鞘脑膜瘤的好发人群。而据报道[7]颅内非典型脑膜瘤多发生于男性,女性在非典型脑膜瘤人群中相对少见。视神经鞘脑膜瘤在早期一般仅出现一侧性视盘水肿,而晚期才出现典型症状(包括无痛渐进性视力丧失、视盘萎缩和视睫状静脉)[6],患者多因视力下降为主诉就诊,此时病程已达晚期。

       在影像上,良性视神经鞘脑膜瘤多呈等T1等T2信号,增强后视神经周围肿瘤强化明显,中央视神经不强化,出现典型的“双轨征”。而非典型脑膜瘤由于生长不均衡,肿瘤形态不规则,多呈分叶状[7],肿瘤恶性程度较高,侵袭性强,影像表现为肿瘤与邻近正常组织分界不清。本例中肿瘤亦呈不规则分叶状,MRI平扫未见明确视神经,增强扫描其内隐约可见局部弱强化且变形移位的视神经,未见典型的“双轨征”,可能因为肿瘤具有一定侵袭性,视神经受侵所致。手术亦证实视神经被肿块紧密包裹,无法彻底分离,病理学显示其被肿瘤浸润。本例上直肌与肿物之间界限不清,局部亦受到侵犯。视神经鞘非典型脑膜瘤的DWI呈高信号、ADC值降低,与文献报道相仿[5]

       眶内视神经鞘脑膜瘤需与以下疾病进行鉴别:(1)炎性假瘤,表现为眼环增厚、泪腺增大、眼外肌及视神经增粗、眶内肿块等,易累及眶尖脂肪组织,增强扫描强化程度弱于脑膜瘤,且临床上多有反复发作的炎症病史[8];(2)视神经胶质瘤,多发于学龄前儿童,成人少见,部分与神经纤维瘤病伴发,T1WI呈低信号、T2WI呈不均匀高信号,增强扫描呈中度强化,肿块起自视神经,与视神经无分界,无“轨道征”,可同时累及眶内段、管内段视神经和视交叉而表现为“哑铃状”[9];(3)淋巴瘤,多发生在中老年女性,病灶边界不清,呈分叶状,可沿眼眶外上肌锥外间隙向眶内浸润性生长,呈“铸型”生长或“光芒”样改变,呈T1WI低、T2WI稍高信号,信号均匀,ADC图亦见扩散明显受限[10]。(4)绿色瘤,又称“粒细胞性肉瘤”,常见于10岁以下的急性粒细胞白血病患儿,男性多见,表现为单侧或双侧骨膜下和肌锥外间隙内软组织肿块,T1WI和T2WI均呈较均匀的中等信号,增强扫描呈中到明显强化,病情进展迅速[11]

       视神经鞘非典型脑膜瘤常呈进行性生长且易复发、预后不佳,MRI许多征象与常见良性视神经鞘脑膜瘤相似,但临床呈现较快速生长,视力下降较早且明显,同时MRI表现出分叶征、侵犯视神经等结构,DWI高信号、ADC降低时,可能提示非典型脑膜瘤的诊断。

[1]
Liang YM, Ning B, Hua X, et al. Atypical meningioma: a retrospective analysis of six cases and literature review[J]. Transl Cancer Res, 2021, 10(3): 1509-1518. DOI: 10.21037/tcr-21-375.
[2]
Bulleid LS, James Z, Lammie A, et al. The effect of the revised WHO classification on the incidence of grade Ⅱ meningioma[J]. Br J Neurosurg, 2020, 34(5): 584-586. DOI: 10.1080/02688697.2019.1639616.
[3]
Wiemels J, Wrensch M, Claus EB. Epidemiology and etiology of meningioma[J]. J Neurooncol, 2010, 99(3): 307-314. DOI: 10.1007/s11060-010-0386-3.
[4]
Dolar Bilge A, Yazici B, Güngör AF, et al. An atypical paediatric optic nerve sheath meningioma[J]. Neuroophthalmology, 2020, 44(6): 403-406. DOI: 10.1080/01658107.2020.1757727.
[5]
Nabavizadeh SA, Santi M, Belasco JB, et al. Primary atypical optic nerve sheath meningioma in a child with restricted diffusion on magnetic resonance imaging[J]. J Neuroophthalmol, 2014, 34(2): 173-176. DOI: 10.1097/WNO.0000000000000127.
[6]
苟文, 肖利华, 李月月, 等. 视神经鞘脑膜瘤50例临床分析[J]. 眼科新进展, 2009, 29(4): 284-287. DOI: 10.13389/j.cnki.rao.2009.04.005.
Gou W, Xiao LH, Li YY, et al. Clinical analysis of optic nerve sheath meningiomas in 50 cases[J]. Recent Adv Ophthalmol, 2009, 29(4): 284-287. DOI: 10.13389/j.cnki.rao.2009.04.005.
[7]
郭慧, 张云亭, 张敬, 等. 非典型脑膜瘤MRI表现[J]. 中国临床医学影像杂志, 2009, 20(1): 1-4. DOI: 10.3969/j.issn.1008-1062.2009.01.001.
Guo H, Zhang YT, Zhang J, et al. MR features of atypical meningiomas[J]. J China Clin Med Imaging, 2009, 20(1): 1-4. DOI: 10.3969/j.issn.1008-1062.2009.01.001.
[8]
Yeşiltaş YS, Gündüz AK. Idiopathic orbital inflammation: review of literature and new advances[J]. Middle East Afr J Ophthalmol, 2018, 25(2): 71-80. DOI: 10.4103/meajo.MEAJO_44_18.
[9]
李海燕, 王道芸, 黄莉. 眼眶炎性假瘤多层螺旋CT、磁共振影像学表现及诊断价值研究[J]. 中国CT和MRI杂志, 2021, 19(10): 22-24. DOI: 10.3969/j.issn.1672-5131.2021.10.008.
Li HY, Wang DY, Huang L. Imaging features of the orbital inflammatory pseudotumor in the multi-slice spiral CT and magnetic resonance imaging and its diagnostic value[J]. Chin J CT MRI, 2021, 19(10): 22-24. DOI: 10.3969/j.issn.1672-5131.2021.10.008.
[10]
张蕾, 杨全新, 王建明, 等. 眼眶淋巴瘤的CT和MR影像特点[J]. 现代肿瘤医学, 2019, 27(19): 3495-3499. DOI: 10.3969/j.issn.1672-4992.2019.19.032.
Zhang L, Yang QX, Wang JM, et al. Characteristics of CT and MR imaging of orbital lymphoma[J]. J Mod Oncol, 2019, 27(19): 3495-3499. DOI: 10.3969/j.issn.1672-4992.2019.19.032.
[11]
AlSemari MA, Perrotta M, Russo C, et al. Orbital myeloid sarcoma (chloroma): report of 2 cases and literature review[J/OL]. Am J Ophthalmol Case Rep, 2020, 19 [2022-02-22]. https://doi.org/10.1016/j.ajoc.2020.100806. DOI: 10.1016/j.ajoc.2020.100806.

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