多结节和空泡状神经元肿瘤的影像诊断
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摘要
目的总结多结节和空泡状神经元肿瘤(multinodular and vacuolating neuronal tumor,MVNT)的临床、病理及影像学特点,提高对该病的认识。材料与方法分析文献,总结大脑MVNT的临床、病理及影像学特点。检索北京协和医院放射科数据库,回顾性分析具有MVNT典型影像特征的病例特点。结果截至2018年1月,英文文献共报道87例MVNT患者,其中31例为病理证实,56例具有典型影像表现诊断为MVNT。发病年龄平均41岁(6～71岁),女性略多见。临床上以癫痫/发作性疾病、头痛或其他非特异性症状就诊。病理上表现为皮层下白质内多发的结节状病灶,伴有空泡状结构,符合未成熟神经元细胞的表型特征。影像表现可分为典型特征和非典型特征两大类,前者很好地反映了大体病理特点,表现为大脑半球灰白质交界区多发簇状分布的小结节,或表现为斑片状病变伴周围多发簇状结节,T2WI及FLAIR上病变为高信号,无占位效应,一般无强化。回顾分析我院近3年约10万例头颅磁共振图像,发现12例具有MVNT的典型影像特征。结论 MVNT是一组具有典型病理和影像特征的良性病变,提高对该病的影像学表现的认识,大部分MVNT无需病理即可根据其典型影像特征加以诊断。
Objective: To summarize the clinical, pathologic and imaging characteristics of multinodular and vacuolating neuronal tumors(MVNT) and improve the knowledge about this disease. Materials and Methods: Relevant English literature was reviewed and features of the reported MVNT cases were summarized. Accordingly, the cases with typical imaging findings of MVNT in local database were searched and their clinical and imaging featuers were analyzed. Results: Until January 2018, there are 87 MVNT with sufficient data had been reported in English literature. Among them, 31 cases were histologically proved. The mean age at presentation was 41(6—71) years old. A female predominance was observed. The clinical presentation included seizures/suspected seizure(1 episode), headache and nonspecific symptoms. Histologically, the MVNT consisted of multinodular tissue, with varying degrees of matrix vacuolization which indicated a dysplastic early neuronal phenotype. The tumors were located principally within the deep cortical ribbon and superficial subcortical white matter. Imaging findings corresponded well with the histologic features. Typical imaging features consisted of a cluster of variably sized nodular lesions located in the subcortical ribbon and superficial subcortical white matter following the gyral contour. They appeared hyperintense on T2 WI and FLAIR images, and typically do not enhance or cause mass effect.There are 12 cases of MVNT with typical features identified among about 100 thousand cases in our database. Conclusions: MVNT is a benign lesion with typical histologic and imaging features. With improving knowledge of the features, most of the MVNTs may be diagnosed with neuroimaging without the need of biopsy.
Objective: To summarize the clinical, pathologic and imaging characteristics of multinodular and vacuolating neuronal tumors(MVNT) and improve the knowledge about this disease. Materials and Methods: Relevant English literature was reviewed and features of the reported MVNT cases were summarized. Accordingly, the cases with typical imaging findings of MVNT in local database were searched and their clinical and imaging featuers were analyzed. Results: Until January 2018, there are 87 MVNT with sufficient data had been reported in English literature. Among them, 31 cases were histologically proved. The mean age at presentation was 41(6—71) years old. A female predominance was observed. The clinical presentation included seizures/suspected seizure(1 episode), headache and nonspecific symptoms. Histologically, the MVNT consisted of multinodular tissue, with varying degrees of matrix vacuolization which indicated a dysplastic early neuronal phenotype. The tumors were located principally within the deep cortical ribbon and superficial subcortical white matter. Imaging findings corresponded well with the histologic features. Typical imaging features consisted of a cluster of variably sized nodular lesions located in the subcortical ribbon and superficial subcortical white matter following the gyral contour. They appeared hyperintense on T2 WI and FLAIR images, and typically do not enhance or cause mass effect.There are 12 cases of MVNT with typical features identified among about 100 thousand cases in our database. Conclusions: MVNT is a benign lesion with typical histologic and imaging features. With improving knowledge of the features, most of the MVNTs may be diagnosed with neuroimaging without the need of biopsy.
引文
[1]Huse JT, Edgar M, Halliday J, et al. Multinodular and vacuolating neuronal tumors of the cerebrum:10 cases of a distinctive seizureassociated lesion. Brain Pathol, 2013, 23(5):515-524.
[2]BodiI,CurranO,SelwayR,etal.Twocasesofmultinodularand vacuolating neuronal tumour. Acta Neuropathol Commun, 2014, 20(2):7.
[3]Fukushima S, Yoshida A, Narita Y, et al. Multinodular and vacuolating neuronaltumorofthecerebrum.BrainTumorPathol,2015,32(2):131-136.
[4]Nagaishi M, Yokoo H, Nobusawa S, et al. Localized overexpression of alpha-internexin within nodules in multinodular and vacuolating neuronal tumors. Neuropathology, 2015, 35(6):561-568.
[5]Yamaguchi M, Komori T, Nakata Y, et al. Multinodular and vacuolating neuronal tumor affecting amygdala and hippocampus:A quasi-tumor?.Pathol Int, 2016, 66(1):34-41.
[6]Cathcart SJ, Klug JR, Helvey JT, et al. Multinodular and vacuolating neuronaltumor:Arareseizure-associatedentity. AmJSurgPathol,2017, 41(7):1005-1010.
[7]Nunes RH, Hsu CC, da Rocha AJ, et al. Multinodular and vacuolating neuronal tumor of the cerebrum:A new"leave me alone"lesion with a characteristic imaging pattern. AJNR Am J Neuroradiol, 2017, 38(10):1899-1904.
[8]Badat N, Savatovsky J, Charbonneau F, et al. Multinodular vacuolating and neuronal tumor of the cerebrum. Neurology, 2017, 89(3):304-305.
[9]AlsufayanR, Alcaide-LeonP,deTillyLN,etal.Naturalhistory oflesionswiththeMRimagingappearanceofmultinodularand vacuolating neuronal tumor. Neuroradiology, 2017, 59(9):873-883.
[10]Thom M, Liu J, Bongaarts A, et al. Multinodular and vacuolating neuronal tumors in epilepsy:dysplasia or neoplasia?. Brain Pathol, 2018,28(2):155-171.
[11]MontéAMC, D'Arco F, De Cocker LJL. Multinodular and vacuolating neuronaltumorinanadolescentwithklinefeltersyndrome.Neuroradiology, 2017, 59(12):1187-1188.
[12]Gonzalez-QuaranteLH,Ruiz-JuretschkeF,SolaVendrellE,etal.Multinodular and vacuolating neuronal tumor of the cerebrum:A rare entity. New case and review of the literature. Neurocirugia(Astur),2018, 29(1):44-55.
[13]Kapucu I, Jhaveri MD, Kocak M, et al. Multinodular and vacuolating neuronal tumor of the cerebrum:A benign nonaggressive cerebral lesion.Eur Neurol, 2018, 79(1-2):74-75.
[14]Rudie JD, Rauschecker AM, Nabavizadeh SA, et al. Neuroimaging of dilated perivascular spaces:From benign and pathologic causes to mimics. J Neuroimaging, 2018, 28(2):139-149.
[15]Lecler A, Chauvet D, Biassette HA, et al. Multiparametric imaging improves confidence in the diagnosis of multinodular and vacuolating neuronal tumor of the cerebrum. AJNR Am J Neuroradiol, 2018, 39(2):E32-E33.
[2]BodiI,CurranO,SelwayR,etal.Twocasesofmultinodularand vacuolating neuronal tumour. Acta Neuropathol Commun, 2014, 20(2):7.
[3]Fukushima S, Yoshida A, Narita Y, et al. Multinodular and vacuolating neuronaltumorofthecerebrum.BrainTumorPathol,2015,32(2):131-136.
[4]Nagaishi M, Yokoo H, Nobusawa S, et al. Localized overexpression of alpha-internexin within nodules in multinodular and vacuolating neuronal tumors. Neuropathology, 2015, 35(6):561-568.
[5]Yamaguchi M, Komori T, Nakata Y, et al. Multinodular and vacuolating neuronal tumor affecting amygdala and hippocampus:A quasi-tumor?.Pathol Int, 2016, 66(1):34-41.
[6]Cathcart SJ, Klug JR, Helvey JT, et al. Multinodular and vacuolating neuronaltumor:Arareseizure-associatedentity. AmJSurgPathol,2017, 41(7):1005-1010.
[7]Nunes RH, Hsu CC, da Rocha AJ, et al. Multinodular and vacuolating neuronal tumor of the cerebrum:A new"leave me alone"lesion with a characteristic imaging pattern. AJNR Am J Neuroradiol, 2017, 38(10):1899-1904.
[8]Badat N, Savatovsky J, Charbonneau F, et al. Multinodular vacuolating and neuronal tumor of the cerebrum. Neurology, 2017, 89(3):304-305.
[9]AlsufayanR, Alcaide-LeonP,deTillyLN,etal.Naturalhistory oflesionswiththeMRimagingappearanceofmultinodularand vacuolating neuronal tumor. Neuroradiology, 2017, 59(9):873-883.
[10]Thom M, Liu J, Bongaarts A, et al. Multinodular and vacuolating neuronal tumors in epilepsy:dysplasia or neoplasia?. Brain Pathol, 2018,28(2):155-171.
[11]MontéAMC, D'Arco F, De Cocker LJL. Multinodular and vacuolating neuronaltumorinanadolescentwithklinefeltersyndrome.Neuroradiology, 2017, 59(12):1187-1188.
[12]Gonzalez-QuaranteLH,Ruiz-JuretschkeF,SolaVendrellE,etal.Multinodular and vacuolating neuronal tumor of the cerebrum:A rare entity. New case and review of the literature. Neurocirugia(Astur),2018, 29(1):44-55.
[13]Kapucu I, Jhaveri MD, Kocak M, et al. Multinodular and vacuolating neuronal tumor of the cerebrum:A benign nonaggressive cerebral lesion.Eur Neurol, 2018, 79(1-2):74-75.
[14]Rudie JD, Rauschecker AM, Nabavizadeh SA, et al. Neuroimaging of dilated perivascular spaces:From benign and pathologic causes to mimics. J Neuroimaging, 2018, 28(2):139-149.
[15]Lecler A, Chauvet D, Biassette HA, et al. Multiparametric imaging improves confidence in the diagnosis of multinodular and vacuolating neuronal tumor of the cerebrum. AJNR Am J Neuroradiol, 2018, 39(2):E32-E33.