磁共振成像在成人眼眶黄色肉芽肿病的诊断价值.pdf

成人眼眶黄色肉芽肿病（AOXGD）是一组罕见的病因和发病机制不明的非朗格汉斯组织细胞增生病。常发生于眼睑皮肤及皮下组织、眶内等组织，可累及泪腺，部分亚型伴有骨、心脏、肺和淋巴组织等全身组织或器官的病变 1。该病缺乏特征性的临床表现，多以眼睑肿胀就诊 2-3，既往报道多以临床经验诊疗报道为主 4-6，影像学诊断方面的文献以个案报道居多 5,7，且多为形态学的描述，没有系统的归纳总结影像特征，且未见对其功能成像的报道，如动态增强曲线、弥散加权成像（DWI）、平均表观扩散系数（ADC）等。本文分析经手术证实的11例AOXGD的影像学资料，总结其磁共振图Value of magnetic resonance imaging in patients with adult orbital xanthogranulomatousdiseaseGUO Jiuhong,WANG Xiaoyan,TAO XiaofengDepartment of Radiology,Shanghai Ninth Peoples Hospital,Medicine School of Shanghai Jiaotong University,Shanghai 200011,China摘要：目的 分析成人眼眶黄色肉芽肿病的磁共振表现，提高对该病的认识。方法回顾性分析11例患者的MR图像，分析MR图像病变部位、双侧/单侧、形态、边缘、眶内结构、T1WI、T2WI信号特点、DWI特点、ADC及强化方式。结果 11例眼眶黄色肉芽肿经手术病理证实，分别是成人起病的黄色肉芽肿（n=6）、渐进性坏死性黄色肉芽肿（n=4）和Erdheim-Chester病（n=1）。成人起病的黄色肉芽肿6例，3例累及单侧眼睑，1例累及右侧鼻泪管，1例累及双侧眼睑和泪腺，1例累及右侧眼睑、翼腭窝和左侧颞肌；渐进性坏死性黄色肉芽肿4例，2例累及左侧眼睑，2例累及双侧眼睑、鼻部、额颞部；Erdheim-chester病1例，双侧眶内多发病变并累及垂体、心包、肾脏。4例边界清晰，7例范围弥漫，边界不清。10例T1WI低、T2WI及压脂高信号，1例T1WI低、T2WI及压脂低信号。弥散加权成像低信号（n=7）；平均表观扩散系数值为1.1810-3mm2/s（n=7）。6例行动态增强检查，时间-信号曲线（TIC）均表现为持续上升型（I型）。结论 眼眶黄色肉芽肿病多有眼睑肿胀，无破溃，累及眼睑及皮下组织、眶前部、眶后部和泪腺，可累及眼外肌肌腱，未见累及视神经，累及眶内病变未见骨壁破坏。MRI可以通过显示化学位移伪影来辅助此病的诊断。关键词：磁共振成像；黄色肉芽肿病；眼眶；弥散加权成像；动态增强磁共振图像Abstract:Objective To analyze the MRI features of adult orbital xanthogranuloma and improve recognition of the disease.Methods The MRI images of 11 patients were retrospectively analyzed.We analyzed the location,unilateral or bilateralinvolvement,shape,margins,orbital structures,T1WI and T2WI signal characteristics,diffusion-weighted imagingcharacteristics,apparent diffusion coefficient and enhancement patterns.Results Eleven cases of orbital xanthogranulomawere confirmed by surgical pathology,including 6 cases of adult-onset xanthogranuloma,4 cases of necrobioticxanthogranuloma,and 1 case of Erdheim-Chester disease.Of the 6 cases of adult-onset xanthogranuloma,3 involved unilateraleyelids,1 involved the right nasolacrimal duct,1 involved bilateral eyelids and lacrimal glands and 1 involved right eyelid,pterygopalatine fossa and left temporal muscle.Of the 4 cases of necrobiotic xanthogranuloma,2 involved the left eyelid,and 2involved bilateral eyelids,nasal and temporal regions.The case of Erdheim-Chester disease involved multiple bilateral orbitallesions and also affected the pituitary gland,pericardium,and kidneys.The lesions of 4 cases had clear boundaries,whilethose of 7 cases had diffuse boundaries.Ten cases showed low signal intensity on T1WI,high signal intensity on T2WI and fat-suppressed imaging,and 1 case showed low signal intensity on T1WI,T2WI and fat-suppressed imaging.Diffusion-weightedimaging showed low signal intensity(n=7).The value of apparent diffusion coefficient was 1.1810-3mm2/s(n=7).Dynamiccontrast-enhanced MRI showed continuous rising patterns(type I)in all 6 cases.Conclusion Orbital xanthogranulomatypically presents as eyelid swelling without ulceration,involving eyelids and subcutaneous tissue,the anterior and posteriororbit,and lacrimal glands,and may involve extraocular muscle tendons but not optic nerves.No bone destruction wasobserved in the involved orbit.MRI can assist in the diagnosis of this disease by displaying chemical shift artifacts.Keywords:magnetic resonance imaging;xanthogranulomatous disease;orbit;diffusion-weighted imaging;dynamic contrast-enhanced MRI磁共振成像在成人眼眶黄色肉芽肿病的诊断价值磁共振成像在成人眼眶黄色肉芽肿病的诊断价值郭久红，王晓琰，陶晓峰上海交通大学医学院附属第九人民医院放射科，上海 200011收稿日期：2023-03-22基金项目：国家自然科学基金（91859202,82172049）Supported by National Natural Science Foundation of China(91859202,82172049)作者简介：郭久红，硕士，住院医师，E-mail:通信作者：王晓琰，主治医师，E-mail:分子影像学杂志,2023,46(4):609-613doi 10.12122/j.issn.1674-4500.2023.04.06 609像特征，旨在帮助影像科医生提高对本病的认识，为临床诊断和治疗提供重要参考依据，从而减少误诊或不必要的手术治疗。1 资料与方法1.1 一般资料回顾性分析2015年10月2022年10月入院且经手术切除或活检证实的眼眶黄色肉芽肿病患者11例，其中男性4例，女性7例，年龄4166岁，平均52.09岁。病史2月7年，主要临床表现为9例（81.8%）眼睑肿胀渐进增大至触及肿块，1例（9.1%）眼球突出，1例（9.1）累及鼻泪管，出现流泪，无红痛、视力下降等。纳入标准：患者均经病理证实。排除标准：图像质量较差，以及曾有过外院手术或者放化疗病史。临床上将AOXGD分为4个亚型：成人起病的黄色肉芽肿（AOX）、渐进性坏死性黄色肉芽肿（NXG）、Erdheim-Chester病（ECD）和成人起病的眶周黄色肉芽肿合并哮喘（AAPOX）8。本研究已通过医院伦理学审批和患者知情同意。1.2 检查方法所有患者知情同意后，行磁共振成像检查，采用西门子Magnetom Verio 3.0T磁共振扫描仪。患者仰卧位，采用头部12通道相控阵线圈。眼眶扫描序列包括常规平扫、增强及DWI序列，6例行动态增强。主要序列和参数如下：横断位T1WI采用快速自旋回波序列，TR 620 ms，TE 9.2 ms，层厚3 mm，FOV 100 mm100mm；横断位T2WI压脂序列，TR 4000 ms，TE 75 ms；动态增强扫描采用容积式内插值法屏气检查序列，TR 20ms，TE 3.7 ms，层厚1.5 mm，扫描时间为4 min，共采集8个时相；增强后横断位、冠状位、斜矢状位T1WI扫描，TR 433 ms，TE 9.2 ms。采用高压注射器经上臂静脉注射，注射速率2.5 mL/s，剂量14 mL。DWI采用b值1000 s/mm2。1.3 图像分析和数据处理所有图像传到西门子工作站进行图像后处理，选择显示病变最佳层面，将感兴趣区置于病变强化最明显区域，生成时间信号强度曲线（TIC）。TIC分型：I型为持续上升型，信号逐渐增强；II型为上升平台型，信号强化先增加到峰值后不变；III型为上升流出型，信号强度上升到峰值后下降超过10%。由2位工作8年以上且长期从事头颈部疾病诊断有经验的影像学医师分别完成MR图像评估分析，评估包括：单侧还是双侧病变、病变部位、信号、形态、边界、泪腺眼外肌等眼眶附属结构等。以正常大脑灰质信号强度为标准，判断眼眶病变T1WI、T2WI及压脂、DWI信号特点，信号强度低于脑灰质信号定义为低信号，反之则为高信号。2 结果经病理证实的11例AOXGD患者共有3种分型，分别是AOX（n=6）、NXG（n=4）和ECD（n=1）。AOX 6例中，3例累及单侧眼睑，1例累及右侧鼻泪管，1例累及双侧眼睑和泪腺，1例累及右侧眼睑、翼腭窝和左侧颞肌（图1）。NXG 4例中，2例累及左侧眼睑，2例累及双侧眼睑、鼻部、额颞部。ECD 1例双侧眶内外多发病变伴多系统累及，累及垂体、心包、肾脏等（图2）。所有病变MRI表现为：10例T1WI低、T2WI及压脂高信号，1例T1WI低、T2WI及压脂低信号；4例边界清晰，7例边界不清；7例行DWI检查，均为低信号，ADC范围（1.011.50）10-3mm2/s，平均为1.1810-3mm2/s；6例行动态增强检查，TIC表现为持续上升型（I型）。临床资料和MRI表现（表1）。SubtypeAOX（n=6）NXG（n=4）ECD（n=1）Age(years)33-6641-5843Chief complaintFive cases of eyelidswelling ranging from3 months to 3 years,and one case of righteye tearing for morethan 3 years.Eyelid swelling formore than 3 months to7 years.Bilateral eyelidswelling withprotrusion of theeyeballs for more than2 months.Lesion locationThree cases involved unilateraleyelids,one case involved theright nasolacrimal duct,one caseinvolved both eyelids and lacrimalgland,and one case involved theright eyelid,pterygopalatine fossa,and left temporal muscle.Two cases involving the lefteyelid,and two cases involvingboth eyelids,nose,forehead andtemporal region.Bilateral lesions in the anteriorand posterior orbit and medial andlateral rectus muscles.MRI findingsT1WI showed low signal,T2WI showed highsignal,with unclear boundaries in four cases andclear boundaries in two cases.The lesions hadirregular shapes,diffuse and unrestricteddistribution,and homogeneous enhancement.T1WI showed low signal,T2WI showed highsignal.Three cases had unclear boundaries,and onecase had clear boundaries.The shape was irregular,with diffuse and unrestricted enhancement.T1WI showed low signal intensity,T2WI imagesshowed low signal intensity,and T2WI fat-suppressed images show low signal intensity.Theborder was clear,the shape was irregular,thediffusion was unrestricted,and with homogeneousenhancement.AOX:Adult-onset xanthogranuloma;NXG:Necrobiotic xanthogranuloma;ECD:Erdheim-Chester disease.表1 11例AOXGD的临床资料和MRI表现Tab.1 Clinical data and MR findings of 11 cases of AOXGD分子影像学杂志,2023,46(4):609-613http:/www.j- 6103 讨论眼眶黄色肉芽肿病是一种罕见的组织细胞增生症。目前认为此病继发于游离组织巨噬细胞的反应性增殖，对于其增殖机制目前还不清楚。组织病理学特征为眶周病变中可见大量泡沫细胞和典型的Touton巨细胞，伴有不同程度的纤维化。各亚型之间的微小区别在于所含不同细胞的数量和聚集程度 9。多数起病缓慢，可有眼球突出，肿胀不适，无发红、无眼痛、无触痛、无球结膜充血水肿、无视力下降等。病变累及鼻泪管，会引起流泪的症状。MRI对软组织分辨力高，可以清晰显示病变大小、部位、形态、范围及与周围组织关系等，尤其是眶内外多发的病变。本文有1例因流泪就诊的患者，多次行泪道冲洗治疗无效，后做MRI增强检查发现病变，可见MRI在眼眶病变诊断的重要价值。目前尚未查阅到AOXGD的特异性影像学的文献，多数为个案报道。AOXGD主要累及眶隔前软组织，ECD除外，其主要累及球后锥内软组织。但是所有病例中视神经未见受累，与文献报道相符合 10，故此病基本不伴有视力障碍。本研究11例患者眶脂体未见T2WI高信号，考虑此病不伴有渗出。有文献指出AOXGD在T2WI图像上，眶内肌锥外病变下缘与邻近眼肌交界处会出现线样低信号影，出现此征象，可以提示此病变 7。本文中有1例病变与邻近外、上直肌边缘见弧形低信号，与报道相符。出现这种征象可能因为病变细胞内含有脂肪，即化学位移伪影，虽然脂肪诱导的化学位移伪影是眼眶MR成像中常见伪影，但是很少出现在肿块下方。ECD属于罕见的亚型，文献报道其发病年龄多为4070岁，男性稍多见 11。本文1例此型患者，为中年男性，与报道相符合。目前认为ECD是一种系统性疾病，发病机制仍未明确，多认为ECD是一种炎性克隆性疾病 12。大多数患者存在多脏器、多系统受累，常可累及骨骼、中枢神经系统、肾、心血管、肺、内分泌、腹膜后、眼眶、皮肤等，眼眶受累的ECD罕见，约占已诊断ECD的25%13-14。本研究案例以眼睑肿胀伴眼球突出就诊，检查发现合并鞍区肿块、肾脏病变和心包积液。此病例T1WI低、T2WI及T2WI压脂低信号，考虑此病纤维化成分较多所致，与报道的ECD亚型的纤维成分较别的亚型偏多 15 相符合。DWI是现阶段较常用的MR序列，通过检测病变组织中水分子扩散运动，观察机体水分子微观运动状态。图1 患者男，59岁，右侧眼睑、翼腭窝和左侧颞肌的AOX的MRI表现Fig.1 MRI findings of a 59-year-old AOX male patient,which involved right eyelid,pterygopalatinefossa,and left temporal muscle.A:T1WI axial image showed the lesions in the right eyelid,pterygopalatine fossa,and left temporal muscle appeared as irregular low signals;B:T2WI axial imageshowed the lesions appeared as irregular high signals;C:T2WI coronal image shows the lesion showeda curved low signal at the edge of the adjacent lateral and superior rectus muscles（）;D:T1WI coronalimage showed that the enhancement was significantly and uniformly enhanced.ABCDhttp:/www.j-分子影像学杂志,2023,46(4):609-613 611CDEFGHIAB图2 患者男，43岁，双侧眶内多发病变并累及垂体、肾脏的ECD MRI表现Fig.2 MRI findings of a 43-year-old ECD male patient,which involved multiple bilateral orbital lesions and also affected thepituitary gland and kidneys.A:T1WI axial image showed multiple lesions with low signal intensity in both orbits,with clearboundaries;B:T2WI axial image showed comparing with the gray matter of the brain,the lesion appeared as a low signal;C,D:T2WI coronal image showed the lesion appeared as a low signal;E:The DWI lesion appeared as a low signal,ADC was1.0110-3mm2/s;F:T1WI coronal image showed the lesion with obvious and uniform enhancement,and encircled the bilateralrectus internus muscle and the right superior rectus muscle；G:TIC showed continuous rising patterns(type I);H:T1WI axialimage showed a lesion with a diameter of approximately 15mm in the sellar region;I:T2WI coronal image showed dilationand hydronephrosis of both renal pelvises,and infiltration shadows around both kidneys.良性眼眶肿瘤患者ADC远高于恶性患者 16。良性病变中水分子弥散障碍较小，肿瘤细胞生长密度较小，细胞外容积较大，故扩散不受限。既往研究眼眶良恶性病变的ADC低于110-3mm2/s 17-18。本文7例行DWI检查，平均ADC为1.1810-3mm2/s，最低的ADC亦有1.0110-3mm2/s，与既往报道相符。本研究未收集到AAPOX类型，此型与全身免疫系统结构紊乱有关，文献报道此分型主要累及眶前组织，患者一般伴有哮喘，其特征在于即使哮喘发展到很严重阶段，但肺部检查也可能为阴性；哮喘可与眼周病变同时发生，亦可以在眼周病变发生几个月后出现，除了哮喘还可伴随有淋巴结肿大、多发性骨髓瘤等 19-22。因为本文搜集的病例多位于眼科就诊，问及相关病史当时无哮喘症状，但是不排除患者眼科就诊后因哮喘去其他医院就诊的情况。眼眶黄色肉芽肿病在影像学上与一些疾病表现相似，应注意鉴别。炎性假瘤：好发中年女性，多有红肿热痛病史，可累及眶前、眶后多种组织结构，眶脂体可受累，范围弥漫，边界不清，信号混杂，I型曲线，一般对激素治疗比较敏感。淋巴瘤：眼眶淋巴瘤多见于中老年，眶前多见，T1WI等、T2WI稍高信号，信号均匀，边界清楚，增强轻中度强化。淋巴瘤大多为II或III型曲线，ADC值明显偏低 23，既往报道约0.610-3mm2/s 24。IgG4-ROD：头颈部病变好发中老年男性，且血清IgG4水平明显升高，且多呈肿块样改变 2。IgG4-ROD累及三叉神经及其分支是它特征性表现，以眶下神经受累多见 25-27。幼年性黄色肉芽肿：该病在病理上与黄色肉芽肿很相似，但一般发生于婴幼儿，且有自限性；可累及头颈部皮肤，还可伴有眼睛自发性前房积血和青光眼等症状 28。分子影像学杂志,2023,46(4):609-613http:/www.j- 612AOXGD的治疗取决于亚型，AOX偶尔具有自限性，ECD有时可引起显著的发病率和死亡率。AOX和AAPOX通常通过切除或皮质类固醇治疗。NBX或ECD患者首选免疫抑制治疗和放疗 5。综上所述，MRI检查是诊断AOXGD重要环节，既可以了解病变范围，又可以评估治疗效果。AOXGD是罕见但具有临床意义的疾病，可发生在眶前、眶后，较少累及眼肌肌腹和视神经。MRI看到眶前、眶后边界不清的T1WI低、T2WI及压脂高信号病变，ECD亚型呈边界清晰的T2WI及压脂呈低信号，形态不规则，弥散不受限，增强呈中度-明显强化，多为I型曲线，ADC约为1.1810-3mm2/s。MRI可以通过显示化学位移伪影来辅助AOXGD的诊断。熟悉加深对此病的认识，可以提高诊断水平，以减少不必要的手术。参考文献：1 Kerstetter J,Wang J.Adult orbital xanthogranulomatous disease J .Dermatol Clin,2015,33(3):457-63.2 王玉川,李 静,林锦镛.成人眼眶黄色肉芽肿病伴泪腺反应性淋巴组织增生的临床病理学特征 J .中华眼科杂志,2022,58(9):682-7.3 王婷婷,林婷婷,刘 勋,等.眼眶成人型黄色肉芽肿的临床分析 J .中华眼科杂志,2019,55(5):381-6.4 Detiger SE,Htte GJ,Verdijk RM,et al.Adult orbital xanthogranu-loma:long-term follow-up of treated cases J .Eye,2022.doi:10.1038/s41433-022-02357-z.5 Maeng MM,Godfrey KJ,Jalaj S,et al.Adult xanthogranulomatousdisease 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