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少突胶质细胞瘤和青少年毛细胞型星形细胞瘤表现为同步性原发性脑肿瘤。病例报告及肿瘤的组织学和分子鉴别,并文献复习

Oligodendroglioma and juvenile pilocytic astrocytoma presenting as synchronous primary brain tumors. Case report with histological and molecular differentiation of the tumors and review of the literature.

作者信息

Kan Peter, Gottfried Oren, Blumenthal Deborah T, Townsend Jeannette J, Drozd-Borysiuk Ela, Brothman Arthur R, Jensen Randy L

机构信息

Department of Neurosurgery, University of Utah, Salt Lake City, Utah 84132-2303, USA.

出版信息

J Neurosurg. 2004 Apr;100(4):700-5. doi: 10.3171/jns.2004.100.4.0700.

Abstract

Multiple metastatic brain tumors and multifocal primary brain tumors of a single histological type are well described in the literature. The concurrent presence of multiple primary brain tumors with different histological characteristics, however, is very rare. The authors describe the first known case in which an oligodendroglioma and a juvenile pilocytic astrocytoma (JPA) presented as synchronous primary brain tumors in the same patient. This 43-year-old man presented with a 2-month history of progressive headaches, nausea, and vomiting. Magnetic resonance imaging demonstrated an enhancing heterogeneous right medial cerebellar lesion and a larger calcified, nonenhancing, heterogeneous right frontal lesion with surrounding edema and a mass effect. The results of a metastatic workup were unremarkable. The patient underwent an initial right frontotemporal craniotomy and a subsequent suboccipital craniectomy 2 years later for resection of the posterior fossa lesion. Histological examination revealed the frontal and cerebellar lesions to be an oligodendroglioma and JPA, respectively. A molecular analysis detected a deletion of chromosome 1p36 in the oligodendroglioma, but not in the JPA. After the initial operation, the patient received follow-up care for his oligodendroglioma, but eventually required temozolomide for tumor progression. His condition remains stable both neurologically and according to imaging studies. The authors describe the first known case in which a low-grade oligodendroglioma and a JPA presented as synchronous primary brain tumors. They review the literature on multiple primary brain tumors with different histological characteristics and discuss potential mechanisms for the development of these lesions.

摘要

文献中对单一组织学类型的多发性转移性脑肿瘤和多灶性原发性脑肿瘤已有充分描述。然而,同时存在具有不同组织学特征的多个原发性脑肿瘤的情况非常罕见。作者描述了首例已知病例,一名患者同时出现少突胶质细胞瘤和青少年毛细胞型星形细胞瘤(JPA)这两种同步原发性脑肿瘤。这名43岁男性有2个月渐进性头痛、恶心和呕吐病史。磁共振成像显示右侧小脑内侧有一个强化的不均匀病变,以及一个更大的钙化、无强化、不均匀的右侧额叶病变,伴有周围水肿和占位效应。转移灶检查结果无异常。患者最初接受了右额颞开颅手术,2年后又接受了枕下颅骨切除术以切除后颅窝病变。组织学检查显示额叶和小脑病变分别为少突胶质细胞瘤和JPA。分子分析在少突胶质细胞瘤中检测到1p36染色体缺失,而在JPA中未检测到。初次手术后,患者接受了少突胶质细胞瘤的随访治疗,但最终因肿瘤进展需要使用替莫唑胺。他的神经状况和影像学检查结果均保持稳定。作者描述了首例已知的低级别少突胶质细胞瘤和JPA作为同步原发性脑肿瘤出现的病例。他们回顾了关于具有不同组织学特征的多个原发性脑肿瘤的文献,并讨论了这些病变发生发展的潜在机制。

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