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第四脑室内有玫瑰花结形成的神经胶质神经元肿瘤,见于老年患者。

Rosette-forming glioneuronal tumor of the fourth ventricle in an elderly patient.

机构信息

Department of Neurosurgery, Carl Gustav Carus University Hospital, Fetscherstr. 74, 01307 Dresden, Germany.

出版信息

J Neurooncol. 2011 Jul;103(3):727-31. doi: 10.1007/s11060-010-0408-1. Epub 2010 Sep 25.

Abstract

Rosette-forming glioneuronal tumor of the fourth ventricle (RGNT) is a rare condition, which previously has been described predominantly in middle-aged patients. There is limited experience with this kind of tumor in the elderly. Clinical, neuroimaging, and histological features of an example in a 70-year-old male who presented initially with vertigo are detailed and compared with published cases. Neuroimaging studies demonstrated a 4-cm cystic lesion in posterior fossa containing a 1-cm contrast-enhancing nodule on its lateral margin. The lesion was confined to the fourth ventricle and initially thought to be a hemangioblastoma until angiography clarified the minimal tumor vascularization. Gross total resection was achieved. Pathological examination showed a rosette-forming low grade tumor with a cell proliferation rate of 2% being consistent with RGNT. The postoperative course was uneventful and clinical symptoms resolved completely. There was no tumor recurrence after 2 years follow-up. We confirm that the rare and only recently characterized tumor entity of RGNT can also be found in elderly patients; furthermore, it can be associated with a benign course. The main differential diagnosis of RGNT resulting from CNS-imaging modalities in elderly patients are pilocytic astrocytoma and hemangioblastoma of the posterior fossa, which after metastasis are the most common primary adult intra-axial posterior fossa tumors. Therefore, a subtle preoperative radiological diagnosis is warranted and surgery should be performed by experienced hands to avoid neurological deterioration.

摘要

第四脑室胶神经元肿瘤(RGNT)是一种罕见的疾病,以前主要在中年患者中描述。在老年人中,这种肿瘤的经验有限。详细介绍了一位 70 岁男性最初表现为眩晕的病例的临床、神经影像学和组织学特征,并与已发表的病例进行了比较。神经影像学研究显示在后颅窝有一个 4cm 的囊性病变,其外侧缘有一个 1cm 的对比增强结节。病变局限于第四脑室,最初被认为是血管母细胞瘤,直到血管造影明确了肿瘤的最小血管化程度。实现了完全肿瘤切除术。病理检查显示为具有 2%细胞增殖率的玫瑰花形成的低级别肿瘤,符合 RGNT。术后过程顺利,临床症状完全缓解。2 年随访后无肿瘤复发。我们证实,罕见且仅在最近才确定的 RGNT 肿瘤实体也可在老年患者中发现;此外,它可能与良性病程相关。老年患者的中枢神经系统影像学检查的主要鉴别诊断是毛细胞星形细胞瘤和后颅窝血管母细胞瘤,它们是最常见的成人原发性脑室内后颅窝肿瘤。因此,需要进行微妙的术前影像学诊断,并且应由经验丰富的医生进行手术,以避免神经恶化。

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