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颗粒细胞星形细胞瘤

Granular cell astrocytoma.

作者信息

Shi Yan, Morgenstern Nora

机构信息

Department of Pathology, Long Island Jewish Medical Center, New Hyde Park, NY 11040, USA.

出版信息

Arch Pathol Lab Med. 2008 Dec;132(12):1946-50. doi: 10.5858/132.12.1946.

Abstract

Granular cell astrocytoma (GCA) is a rare type of malignant brain tumor with distinct morphologic features and aggressive clinical behavior. Almost all GCAs occur in the cerebral hemispheres. It is characterized by a prominent component of bland-looking granular cells. The tumor cells are usually positive for glial fibrillary acidic protein, S100, CD68, and epithelial membrane antigen. The most important differential diagnoses include a number of reactive lesions such as cerebral infarction, multiple sclerosis, and progressive multifocal leukoencephalopathy. Electron microscopic study reveals that the granules of GCA correspond to an increased number of intracytoplasmic lysosomes. The histogenesis of GCA is still unclear, but most people believe it originates from astrocytes. Loss of 9p and 10q were identified in almost all cases of GCA, but they are not specific for this tumor. Surgical excision plus postoperative chemotherapy or radiotherapy is the choice for most patients with GCA.

摘要

颗粒细胞星形细胞瘤(GCA)是一种罕见的恶性脑肿瘤,具有独特的形态学特征和侵袭性临床行为。几乎所有的GCA都发生在大脑半球。其特征是有大量外观平淡的颗粒细胞。肿瘤细胞通常对胶质纤维酸性蛋白、S100、CD68和上皮膜抗原呈阳性。最重要的鉴别诊断包括一些反应性病变,如脑梗死、多发性硬化症和进行性多灶性白质脑病。电子显微镜研究显示,GCA的颗粒对应于胞质内溶酶体数量的增加。GCA的组织发生仍不清楚,但大多数人认为它起源于星形胶质细胞。几乎所有GCA病例都发现有9p和10q缺失,但它们并非该肿瘤所特有。手术切除加术后化疗或放疗是大多数GCA患者的选择。

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