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以脑出血为表现且被误诊为硬脑膜动静脉瘘的成星形细胞瘤:一种罕见实体的病例回顾

Astroblastoma presenting with intracerebral hemorrhage misdiagnosed as dural arteriovenous fistula: review of a rare entity.

作者信息

Alaraj Ali, Chan Michael, Oh Stephen, Michals Edward, Valyi-Nagy Tibor, Hersonsky Tamir

机构信息

Department of Neurosurgery, University of Illinois at Chicago, Chicago, IL 60612, USA.

出版信息

Surg Neurol. 2007 Mar;67(3):308-13. doi: 10.1016/j.surneu.2006.05.050. Epub 2006 Nov 16.

DOI:10.1016/j.surneu.2006.05.050
PMID:17320647
Abstract

BACKGROUND

Astroblastoma is one of the most unusual types of tumors whose histogenesis has been recently clarified. It occurs mainly among children and young adults. It is grossly well demarcated and shows histologically characteristic perivascular pseudorosettes with frequent vascular hyalinization. Immunohistochemically, the tumor cells show diffuse strong positivity for GFAP, S-100 protein, vimentin, as well as neuron-specific enolase and focal positivity for EMA.

CASE DESCRIPTION

Our patient is a 33-year-old gentleman who presented with intraparenchymal hemorrhage in the left temporal lobe. A 4-vessel cerebral angiogram revealed a dural AVF in the left posterior temporal area. The lesion was resected, and pathology showed a GFAP-positive lesion with prominent astroblastoma rosettes. There was abundant hemosiderin consistent with a prior hemorrhage. Because of its high degree of proliferation, the presence of astroblastic pseudorosettes, prominent perivascular hyalinization, regional hyaline changes, and pushing borders with regard to the adjacent brain, the tumor was considered anaplastic.

CONCLUSIONS

Astroblastoma is a rare pure pathologic entity--a distinct form of astrocytic gliomas. The diagnosis of astroblastoma is often difficult because of the astroblastic aspects that can be found in astrocytic tumors, in ependymomas, and in nonneuroepithelial tumors. The natural history seems to be between astrocytomas and glioblastomas. Total resection and radiation therapy seem to be the advisable treatments.

摘要

背景

成星形细胞瘤是最罕见的肿瘤类型之一,其组织发生学最近已得到阐明。它主要发生于儿童和青年成人。大体上界限清楚,组织学上显示具有频繁血管玻璃样变的特征性血管周围假菊形团。免疫组织化学方面,肿瘤细胞对GFAP、S-100蛋白、波形蛋白以及神经元特异性烯醇化酶呈弥漫性强阳性,对EMA呈局灶性阳性。

病例描述

我们的患者是一名33岁男性,表现为左侧颞叶脑实质内出血。四血管脑血管造影显示左侧颞后区硬脑膜动静脉瘘。病变被切除,病理显示为GFAP阳性病变,有成星形细胞瘤菊形团突出。有大量含铁血黄素,与既往出血相符。由于其高度增殖、存在成星形细胞假菊形团、显著的血管周围玻璃样变、局灶性玻璃样变以及与相邻脑组织的推移边界,该肿瘤被认为是间变性的。

结论

成星形细胞瘤是一种罕见的单纯病理实体——星形细胞胶质瘤的一种独特形式。由于在星形细胞肿瘤、室管膜瘤和非神经上皮肿瘤中均可发现成星形细胞成分,成星形细胞瘤的诊断往往很困难。其自然病程似乎介于星形细胞瘤和胶质母细胞瘤之间。全切除和放射治疗似乎是可取的治疗方法。

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