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非典型双侧小脑桥脑角髓母细胞瘤:鉴别诊断、免疫组化特征及影像学表现

Atypical bilateral cerebellopontine angle medulloblastoma: differential diagnosis, immunohistochemical features and radiological presentation.

作者信息

Piña Batista Kelvin M, de Eulate-Beramendi Sayoa Alvarez, Rico María, Rodrigo Victor, Batista Batista Yordy E, Alvarez Reyes Kenia Y

机构信息

Hospital Universitario Central de Asturias, Oviedo, Spain.

Hospital Clinico Lozano Blesa de Zaragoza, Oviedo, Spain.

出版信息

Contemp Oncol (Pozn). 2017;21(3):249-253. doi: 10.5114/wo.2017.69593. Epub 2017 Sep 29.

Abstract

Medulloblastoma (MB) is a highly aggressive soft tissue neoplasm, classified as a primitive neuroectodermal tumor. It is the most common posterior fossa tumor in children, but occurs rarely in adults. MB accounts for approximately 20% of all primary central nervous system (CNS) tumors of childhood, while its incidence is around 1% of adult brain tumors. Most often it occurs in the cerebellum. We report a case of multicentric MB involving the bilateral cerebellopontine angle (CPA) and right cerebellar hemisphere. The tumor showed isointensity on T1/T2-weighted images, and slight hyperintensity on T2-weighted fluid-attenuated inversion-recovery (FLAIR) images. The MB had restricted diffusion on diffusion-weighted images (DWI). It was not easy to make an accurate diagnosis before biopsy. The lesion in our patient presented with atypical MR image features of medulloblastoma. To our knowledge, this is the first case of bilateral CPA MB.

摘要

髓母细胞瘤(MB)是一种侵袭性很强的软组织肿瘤,归类为原始神经外胚层肿瘤。它是儿童中最常见的后颅窝肿瘤,但在成人中很少见。MB约占儿童所有原发性中枢神经系统(CNS)肿瘤的20%,而其发病率约为成人大脑肿瘤的1%。它最常发生于小脑。我们报告一例多中心性MB累及双侧小脑脑桥角(CPA)和右侧小脑半球。该肿瘤在T1/T2加权图像上呈等信号,在T2加权液体衰减反转恢复(FLAIR)图像上呈轻度高信号。MB在扩散加权图像(DWI)上有扩散受限。在活检前很难做出准确诊断。我们患者的病变表现出髓母细胞瘤的非典型磁共振图像特征。据我们所知,这是双侧CPA MB的首例病例。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/510c/5701578/fa285ea788b3/WO-21-30474-g001.jpg

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