History:A30-year-oldwomanpresentswithvertigo.
病史:30岁女性,眩晕。
Acontrast-enhancedMRIscanofthebrainwasperformed.AxialT2-weighted,T2-weightedfluid-attenuatedinversion-recovery(FLAIR),andT1-weightedandT1-weightedpostcontrastMRimagesareshownbelow.颅脑MR增强扫描:轴位T2WI、T2-FLAIR、T1WI及T1WI增强图像如下所示。
Findings
MRIdemonstratesaclusterofwell-delineated,variable-sizedFLAIR/T2-hyperintensecystswithintheleftfrontalsubcorticalwhitematter,withoutobviousmasseffectorpostcontrastenhancement.
影像表现:
MRI示左侧额叶皮层下白质内有一簇边界清晰的大小不等的FLAIR/T2高信号的囊肿,无明显占位效应或强化。
Differentialdiagnosis
Multinodularandvacuolatingneuronaltumor
Dysembryoplasticneuroepithelialtumor
Focalcorticaldysplasia
Tumefactiveperivascularspaces
Pilocyticastrocytoma
Ganglioglioma
Pleomorphicxanthoastrocytoma
鉴别诊断:
大脑多结节及空泡性神经元肿瘤
胚胎发育不良性神经上皮肿瘤(DNET)
局灶性皮质发育不良
瘤样血管周围间隙
毛细胞型星形细胞瘤
节细胞胶质瘤
多形性*色星形细胞瘤
Diagnosis:Multinodularandvacuolatingneuronaltumor(MVNT)
最后诊断:大脑多结节及空泡性神经元肿瘤(MVNT)
MVNT
Pathophysiology,epidemiology,andclinicalpresentation:
MultinodularandvacuolatingneuronaltumorsareamorerecentlycharacterizedWorldHealthOrganization(WHO)grade1centralnervoussystemneoplasm.Thisrareentityhasonlybeendescribedinseveralcaseseries.Theyarecharacterizedasabenign,mixedglialneuronallesionconsistingofnodulesofdisorganizedneuronalcellslocatednearthesubcorticalwhitematter.Theyareassociatedwithlong-termseizuresinadultsinabout30%ofpatients,buttheyaremostoftenfoundincidentallyandareconsidered"donottouch"or"leavemealone"lesionsifnotassociatedwithintractableepilepsy.
病理生理学,流行病学和临床表现:
多结节和空泡性神经元肿瘤是最近出现在中枢神经系统肿瘤WHO1级中的,这种罕见的病变仅在几个案例系列中被描述。
主要特征是良性的混合性的胶质神经元病变,位于皮层下白质附近,由紊乱的神经元细胞结节组成。
与大约30%的成年人的长期癫痫发作有关,通常为偶然发现,如果与难治性癫痫无关,无需处理。
Imagingfeatures
TheyaredifficulttoidentifyonCTbutmayexhibithypoattenuation.
OnMRI,theyareseenasasubcorticalclusteroftiny,cystic,nodularlesionswithassociatedT2/FLAIRsignalabnormality(andmildlyT1hypointensity).
Theyareusuallyseeninthedeepcorticalribbonandsuperficialsubcorticalwhitematter.
Theyrarelyenhance(approximately3%to10%ofcases)withstippledfaintenhancement.
Theyshouldnotexhibitrestricteddiffusionorabnormalsusceptibility.
Theyrarelyprogress/increaseinsize.
影像表现:
在CT上很难明确病变,可表现为低密度;
在MRI上表现为T2/FLAIR信号异常(T1轻度低信号)的皮层下簇状微小囊性结节性病变;
多见于深部皮质带和浅表皮层下白质;
多无强化,约3%-10%的病例可见点状微弱增强;
无扩散受限或异常磁敏感性;
病灶大小几乎不会进展/增加。
Treatment
Theyareslow-growing/indolent,andifnotassociatedwithrefractoryepilepsy,theyareconsidereda“leavemealone”lesion.
Theyareusuallyjustfollowedwithimaging,buttheycanberesectediftheyareassociatedwithintractableepilepsy.
治疗:
病灶生长缓慢或无变化,如果不与顽固性癫痫相关,被认为是“别管我”病灶。
通常借助影像进行随访,如果与难治性癫痫相关则予以切除。
以上英文病例来自Auntminnie.