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青少年胼胝体部毛黏液样星形细胞瘤伴原发性出血

Pilomyxoid astrocytoma of the corpus callosum presenting with primary haemorrhage in an adolescent.

作者信息

Longo Maria, Adams Perez Juliano, Oliveira Francine, Antunes Apio, Vedolin Leonardo, Duarte Juliana Avila

机构信息

Pathology, HCPA, POA, Brazil.

Neurosurgery, HCPA, POA, Brazil.

出版信息

BJR Case Rep. 2017 Jan 7;3(2):20150020. doi: 10.1259/bjrcr.20150020. eCollection 2017.

Abstract

A 17-year-old male patient with history of intraventricular haemorrhage in 2007 underwent a brain MRI scan in 2013 owing to headache. Brain MRI scan showed an expansive lesion adjacent to the left lateral ventricle infiltrating the anterior portion of the corpus callosum. After surgery, pathology confirmed a pilomyxoid astrocytoma (PMA), an aggressive subtype of astrocytoma that occurs predominantly in the hypothalamic-chiasmatic region. On imaging, PMA presents as a tumour isointense on , hyperintense on that enhanced heterogeneously with contrast. The signal is higher than pilocytic astrocytoma, which indicates the presence of myxoid matrix. These findings on MRI scan have a direct correlation with a specific pathological finding-monomorphic proliferation of piloid cells in a mucopolysaccharide-rich matrix. These characteristics associated with the absence of Rosenthal fibres or eosinophilic granules indicated the diagnosis of PMA. To our knowledge, this is the first case report of PMA affecting the corpus callosum in an adolescent.

摘要

一名17岁男性患者,2007年有脑室内出血病史,2013年因头痛接受脑部MRI扫描。脑部MRI扫描显示左侧脑室旁有一膨胀性病变,侵犯胼胝体前部。手术后,病理证实为毛细胞黏液样星形细胞瘤(PMA),这是一种主要发生在下丘脑-视交叉区域的侵袭性星形细胞瘤亚型。在影像学上,PMA在T1加权像上呈等密度肿瘤,在T2加权像上呈高密度,增强扫描呈不均匀强化。T2加权像信号高于毛细胞型星形细胞瘤,提示存在黏液样基质。MRI扫描的这些表现与特定的病理发现——在富含黏多糖的基质中毛细胞样细胞的单形性增殖直接相关。这些特征与无Rosenthal纤维或嗜酸性颗粒相关,提示PMA的诊断。据我们所知,这是青少年中PMA累及胼胝体的首例病例报告。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ffc9/6159255/48e77797df7f/bjrcr.20150020.g001.jpg

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