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小脑胚胎发育不良性神经上皮肿瘤的复杂形态变异型

Complex form variant of dysembryoplastic neuroepithelial tumor of the cerebellum.

作者信息

Vaquero Jesús, Saldaña Cristobal, Coca Santiago, Zurita Mercedes

机构信息

Neurosurgical Service, Hospital Puerta de Hierro-Majadahonda, 28222 Madrid, Spain ; Neuroscience Research Unit, Hospital Puerta de Hierro-Majadahonda, 28222 Madrid, Spain.

出版信息

Case Rep Pathol. 2012;2012:718651. doi: 10.1155/2012/718651. Epub 2012 Sep 5.

Abstract

Dysembryoplastic neuroepithelial tumor (DNT) is a benign neoplasm with typical supratentorial location, but the possibility of these rare tumors can also be located in the posterior fossa must be taken into account. We report a 21-year-old woman that suffered gait instability, headache, and diplopia. On CT-scan, an intraparenchymatous cerebellar tumor was disclosed. It was isodense, showing light enhancement after contrast administration. On MRI (T1-weighted image) the tumor was isointense, showing inhomogeneous hyperintensity after-gadolinium administration. On T2-weighted MRI, the tumor was inhomogenously hyperintense. At surgery, a solid and hypervascularized tumor was completely removed. Two years after surgery, the patient is symptom-free. Pathological study showed coexistence of areas of pilocytic astrocytoma with areas in which small rounded oligodendrocyte-like cells (OLC), with strong synaptophysin expression were identified. These neurocytic areas showed an eosinophilic matrix forming microcysts, and cells with aspect of "floating neurons" were occasionally identified. A complex form variant of DNT was diagnosed. Our case suggests that in presence of a cerebellar tumor with features of pilocytic astrocytoma, the possibility of a complex form variant of DNT should be considered.

摘要

胚胎发育不良性神经上皮肿瘤(DNT)是一种典型位于幕上的良性肿瘤,但也必须考虑到这些罕见肿瘤位于后颅窝的可能性。我们报告一名21岁女性,她出现步态不稳、头痛和复视。CT扫描显示脑实质内小脑肿瘤。其密度与周围脑组织相同,增强扫描后呈轻度强化。在MRI(T1加权像)上,肿瘤信号与周围脑组织相同,注射钆剂后呈不均匀高信号。在T2加权MRI上,肿瘤呈不均匀高信号。手术中,一个实性且血管丰富的肿瘤被完全切除。术后两年,患者无症状。病理研究显示,肿瘤存在毛细胞型星形细胞瘤区域,同时还存在一些区域,其中可见小的圆形少突胶质细胞样细胞(OLC),突触素表达强烈。这些神经细胞区域显示嗜酸性基质形成微囊肿,偶尔还可发现具有“漂浮神经元”外观的细胞。诊断为DNT的复杂形式变体。我们的病例表明,当存在具有毛细胞型星形细胞瘤特征的小脑肿瘤时,应考虑DNT复杂形式变体的可能性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/476e/3440858/a257c170788a/CRIM.PATHOLOGY2012-718651.001.jpg

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