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[多形性黄色星形细胞瘤]

[Pleomorphic xanthoastrocytoma].

作者信息

Vajtai I, Varga Z

机构信息

Neuropathologiai Intézet, Universitätsspital Zürich.

出版信息

Orv Hetil. 1997 Apr 20;138(16):991-7.

PMID:9190551
Abstract

Pleomorphic xantho-astrocytoma (PXA) is a relatively rare brain tumor of adolescents and young adults characterized by its superficial location with frequent involvement of the meninges, and its slow growth despite features of histological atypia. The authors present a retrospective immunohistochemical analysis of 8 surgically treated cases in order to determine the expression of glial and neuronal markers, and to assess the proliferating cell fraction. The study population comprised 1 female and 7 male patients with a mean age of 26.7 years, most tumors being located in one of the temporal lobes. Epilepsy predominated as a presenting symptom. Five cases were assigned WHO graded II, while the diagnosis of anaplasia (WHO grade III) was established in three, based either on elevated mitotic counts or the presence of necrosis. Immunostaining with the proliferation marker MIB-1 was present in 2.05% of cells in the former groups, while 4.66% showed labeling in the latter. Postoperative follow-up averaged 6.7 years, with only one recurrence of an anaplastic tumor. All tumors expressed some amount of glial fibrillary acidic protein and were shown to elaborate a characteristic pericellular reticulin network. There was focal reactivity for alpha-1-antitrypsin, but neither the monocyte-macrophage associated antigen CD68 nor lysozym could be detected in neoplastic cells. In 7 cases, scattered individual tumor cells exhibited synaptophysin positivity. The authors review problems and prognostic issues of the histologic diagnosis of anaplasia occurring in some 20% of the cases. A possible dysontogenic origin of PXA and its nosologic relationship to the so-called desmoplastic neuroepithelial tumors of infancy are discussed. This is the first study of pleomorphic xanthoastrocytoma in the Hungarian literature.

摘要

多形性黄色星形细胞瘤(PXA)是一种相对罕见的青少年和年轻成人脑肿瘤,其特征是位于浅表,常累及脑膜,尽管具有组织学异型性特征,但生长缓慢。作者对8例手术治疗病例进行了回顾性免疫组化分析,以确定胶质和神经元标志物的表达,并评估增殖细胞分数。研究人群包括1名女性和7名男性患者,平均年龄26.7岁,大多数肿瘤位于颞叶之一。癫痫是主要的首发症状。5例被归为世界卫生组织(WHO)Ⅱ级,3例根据有丝分裂计数升高或存在坏死被诊断为间变(WHOⅢ级)。在前一组中,增殖标志物MIB-1免疫染色在2.05%的细胞中出现,而后一组中有4.66%显示有标记。术后平均随访6.7年,仅1例间变性肿瘤复发。所有肿瘤均表达一定量的胶质纤维酸性蛋白,并显示形成特征性的细胞周围网状纤维网络。α1抗胰蛋白酶有局灶反应性,但在肿瘤细胞中未检测到单核细胞-巨噬细胞相关抗原CD68和溶菌酶。7例中,散在的单个肿瘤细胞显示突触素阳性。作者回顾了约20%病例中出现的间变组织学诊断的问题和预后问题。讨论了PXA可能的发育异常起源及其与婴儿期所谓促纤维增生性神经上皮肿瘤的疾病分类关系。这是匈牙利文献中关于多形性黄色星形细胞瘤的第一项研究。

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