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当前位置:首页 > 健康养生 > 健康常识 > 双语病例丨大脑多结节及空泡性神经元肿瘤(MVNT)

双语病例丨大脑多结节及空泡性神经元肿瘤(MVNT)

分享人:枫叶112 来源:互联网 时间:2018-04-16 阅读:0

History: A 30-year-old woman presents with vertigo.

病史:30岁女性,眩晕。

A contrast-enhanced MRI scan of the brain was performed. Axial T2-weighted, T2-weighted fluid-attenuated inversion-recovery (FLAIR), and T1-weighted and T1-weighted postcontrast MR images are shown below. 颅脑MR增强扫描:轴位T2WI、T2-FLAIR、T1WI及T1WI增强图像如下所示。


Findings

MRI demonstrates a cluster of well-delineated, variable-sized FLAIR/T2-hyperintense cysts within the left frontal subcortical white matter, without obvious mass effect or postcontrast enhancement.

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。


Differential diagnosis

  • Multinodular and vacuolating neuronal tumor

  • Dysembryoplastic neuroepithelial tumor

  • Focal cortical dysplasia

  • Tumefactive perivascular spaces

  • Pilocytic astrocytoma

  • Ganglioglioma

  • Pleomorphic xanthoastrocytoma


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

  • 多形性黄色星形细胞瘤


Diagnosis: Multinodular and vacuolating neuronal tumor (MVNT)

最后诊断:大脑多结节及空泡性神经元肿瘤 (MVNT)


MVNT


Pathophysiology, epidemiology, and clinical presentation:

Multinodular and vacuolating neuronal tumors are a more recently characterized World Health Organization (WHO) grade 1 central nervous system neoplasm. This rare entity has only been described in several case series. They are characterized as a benign, mixed glial neuronal lesion consisting of nodules of disorganized neuronal cells located near the subcortical white matter. They are associated with long-term seizures in adults in about 30% of patients, but they are most often found incidentally and are considered ''do not touch'' or  ''leave me alone'' lesions if not associated with intractable epilepsy.

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。



Imaging features

  • They are difficult to identify on CT but may exhibit hypoattenuation.

  • On MRI, they are seen as a subcortical cluster of tiny, cystic, nodular lesions with associated T2/FLAIR signal abnormality (and mildly T1 hypointensity).

  • They are usually seen in the deep cortical ribbon and superficial subcortical white matter.

  • They rarely enhance (approximately 3% to 10% of cases) with stippled faint enhancement.

  • They should not exhibit restricted diffusion or abnormal susceptibility.

  • They rarely progress/increase in size.

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

They are slow-growing/indolent, and if not associated with refractory epilepsy, they are considered a “leave me alone” lesion.

They are usually just followed with imaging, but they can be resected if they are associated with intractable epilepsy.

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.com



相关链接及资料:


【WHO分类】中枢神经系统肿瘤(2016版)


新分类认为大脑多结节及空泡性神经元肿瘤病变中的多结节和空泡形成是一种新认识的组织学结构形态,但确定病变在CNS肿瘤疾病分类学中的位置,还待进一步研究。

此肿瘤多见于成人,大部分发生于颞叶。组织学表现为呈小的多结节状,病变位于皮质及皮质与白质交界处,基质不同程度空泡变性。瘤细胞小至大,胞浆丰富,嗜酸性,似神经节细胞,预后好。






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