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弥漫性中枢神经系统原浆型星形细胞瘤。

Diffuse central nervous system protoplasmic astrocytoma.

机构信息

Department of Paediatric Oncology, Alder Hey Childrens' NHS Foundation Trust, Liverpool, UK.

出版信息

Pediatr Blood Cancer. 2010 May;54(5):768-9. doi: 10.1002/pbc.22402.

Abstract

Protoplasmic astrocytoma is an extremely rare form of grade II low grade glioma which usually presents as a discrete mass lesion. We describe a 3-year-old female with diffuse protoplasmic astrocytoma with parenchymal involvement and leptomeningeal spread. This tumour proved extremely difficult to diagnose and followed a progressive course. Three superficial biopsies did not give the diagnosis and this was only confirmed 8 months from presentation from a larger fourth biopsy taken deeper from the cerebellum. To our knowledge this case represents the distinct presentation of protoplasmic astrocytoma presenting as extensive diffuse meningeal disease.

摘要

原浆型星形细胞瘤是一种极其罕见的二级低级别胶质瘤,通常表现为离散的肿块病变。我们描述了一名 3 岁女性患有弥漫性原浆型星形细胞瘤,伴有实质受累和软脑膜播散。该肿瘤的诊断极具挑战性,且呈现进行性病程。3 次浅层活检均未能做出诊断,直到发病 8 个月后,从深部小脑进行的第四次较大活检才最终确诊。据我们所知,本例代表了原浆型星形细胞瘤的独特表现,即广泛弥漫性脑膜疾病。

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