双语病例间变性星形细胞瘤

朗读老师:医院

翻译老师:医院放射科

审校老师:姜春医院

History:A43-year-oldmanwithnosignificantmedicalhistorywasreferredforthreeweeksofrightfacialweaknessthatprogressedtoincluderightarmandhandweakness,rightlegweakness,andword-findingdifficulty.Priortoreferral,aCTscanintheemergencydepartmentdemonstratedamass-likelesion.

MRbrainandMRspectroscopy(MRS)scanswithdiffusion-tensorimaging(DTI)werealsoperformed.Clickontheimagebelowtoenlarge.

病史:男性,43岁,主诉三个星期前无明显诱因出现右侧面肌无力,后进展为右侧上肢、下肢无力,唤词困难。急诊CT发现颅内肿瘤样病变。

头颅MR、MRS及DTI扫描图像如下

Findingsanddiagnosis

·MRbrain:DiffuseexpansilelesionwithT2/FLAIRhyperintensitywithpatchymultifocalenhancementextendingfromtheleftcoronaradiatathroughtheposteriorlimboftheleftinternalcapsule,leftthalamus,leftcerebralpeduncle,thepons,andleftcerebellarpeduncle,andintothemedullaoblongata.

·MRS:Single-voxelMRspectroscopyofafocalabnormalareaintheleftcerebralpedunclewithechotime(TE)ofmsecdemonstrateselevatedcholinepeaks(3.2ppm)withdepressedNAA(2.0ppm)andcreatine(2.0ppm)peaks.

·DTI:Color-encodedstructuralorientationmapofthefibertractsshowssignificantlossofthenormaldirectionalencodedcoloroftheposteriorlimboftheleftinternalcapsule.Correspondingfractionalanisotropy(FA)mapshowsindistinctnessandreducedsignalintensityofthefibersoftheposteriorlimb.Findingsarehighlysuggestiveoftumoralinfiltrationandlikelydisruptionofthecorticalspinaltract.

影像与诊断

颅脑MR:左侧放射冠、内囊后肢、丘脑、大脑脚、桥脑、小脑脚及延髓见弥漫性膨胀性病变,T2/FLAIR呈高信号,增强呈不均匀多灶性斑片状强化。

MRS:选择左侧大脑脚病变中心位置,回波时间ms,显示胆碱峰升高(3.2ppm),NAA(2.0ppm)和Cr(2.0ppm)峰下降。

DTI(弥散张量成像):彩色纤维束结构成像显示左侧内囊后肢正常的定向编码色显著消失。内囊后肢纤维束在相对应的各向异性分数图上显示模糊、信号强度减低。图像高度提示肿瘤浸润以及皮质脊髓束可能中断。

Differentialdiagnosis

·Glioma

·Cerebralmetastasis

·Tumefactivedemyelination

·Subacutecerebralinfarct

Diagnosis:Glioma-anaplasticastrocytoma

鉴别诊断

胶质瘤

脑内转移瘤

肿瘤样脱髓鞘病变

亚急性脑梗死

诊断:胶质瘤-间变型星形细胞瘤

Pathophysiology

Anaplasticastrocytoma(AA)isamalignant,diffuselyinfiltratingneuroepithelialtumororiginatingfromtheastrocytesofthecentralnervoussystem(CNS).AccordingtotheWorldHealthOrganization(WHO)classificationsystemforglialtumors,itisahigh-gradetumor(gradeIII/IV)characterizedbyhypercellularity,mitoticactivity,andnuclearatypiawithoutnecrosis.Twenty-fivepercentofAAsarisedenovo,while75%resultfromtransformationoflower-gradeastrocytoma.MalignantprogressionofAAmayresultinglioblastomamultiforme(WHOgradeIV/IV).Exposuretoionizingradiation,particularlyatayoungage,aswellasrareMendeliandisordersincludingneurofibromatosistypes1and2,tuberoussclerosis,andLi-Fraumenisyndromearetheonlywell-establishedriskfactorsforthedevelopmentofAA.

病理生理

间变型星形细胞瘤(AA)是一种来源于中枢神经系统星形细胞的神经上皮肿瘤,具有恶性、弥漫浸润特性。根据WHO胶质瘤分型,AA属于高级别肿瘤(III/IV级),具有细胞密集,核分裂活跃,核异型性无坏死的特点。25%的AA属于原发,75%由低级别星形细胞瘤演变而来。AA有恶变为多形性胶质母细胞瘤(IV/IV级)的可能。AA致病的危险因素,目前已经明确的有低龄电离辐射,罕见的遗传性疾病,例如神经纤维瘤病1,2型、结节性硬化、李-佛美尼综合症。

Epidemiology

AAisun

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