Small cell astrocytoma: An aggressive variant that is clinicopathologically and genetically distinct from anaplastic oligodendroglioma
Small cell astrocytoma: An aggressive variant that is clinicopathologically and genetically distinct from anaplastic oligodendroglioma
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DOI：
10.1002/cncr.20625
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发表时间：
2004-11-15
期刊：
影响因子：
6.2
通讯作者：
Burger, PC
中科院分区：
文献类型：
--
作者：
Perry, A;Aldape, KD;Burger, PC
背景。小细胞胶质母细胞瘤 (GBM) 是一种具有单形性、看似平淡的细胞核的变异体，经常被误诊为间变性少突胶质细胞瘤。为了阐明其临床病理学和遗传特征，作者研究了 71 名成年患者（中位年龄 57 岁），其中 22 名患者是从 229 例 GBM（10%）中鉴定出来的，这些患者先前已通过表皮生长因子受体 (EGFR)/EGFR-vIII 变异免疫组织化学进行了表征。还通过荧光原位杂交分析肿瘤的 1p、19q、10q 和 EGFR 拷贝数。 结果。放射学上，37% 未选择分级的肿瘤显示出最小程度的增强或没有增强。同样，33% 的肿瘤在组织学上没有内皮增生或坏死，根据世界卫生组织的标准，仅符合间变性星形细胞瘤（III 级）的标准。然而，此类肿瘤进展迅速，死亡率与 IV 级肿瘤没有区别。随访至死亡的 37 名患者的中位生存期为 11 个月。少突胶质细胞瘤样组织学包括铁丝状脉管系统（86%）、晕圈（73%）、神经周围卫星细胞增多症（58%）和微钙化（45%），但缺乏充满粘蛋白的微囊空间。小细胞星形细胞瘤没有 1p/19q 共缺失，而 69% 和 97% 的小细胞星形细胞瘤分别存在 EGFR 扩增和 10q 缺失。肿瘤表达 EGFR 和 EGFR-vIII 的情况比非小细胞 GBM 更常见（83% vs. 35% [P
BACKGROUND. Small cell glioblastoma (GBM) is a variant with monomorphous, deceptively bland nuclei that often is misdiagnosed as anaplastic oligodendroglioma.METHODS. To elucidate its clinicopathologic and genetic features, the authors studied 71 adult patients (median age, 57 years), including 22 patients who were identified from a set of 229 GBMs (10%) that had been characterized previously by epidermal growth factor receptor (EGFR)/EGFR-vIII variant immunohistochemistry. Tumors also were analyzed by fluorescence in situ hybridization for 1p, 19q, 10q, and EGFR copy numbers.RESULTS. Radiologically, 37% of tumors that were not selected for grade showed minimal to no enhancement. Similarly, 33% of tumors had no endothelial hyperplasia or necrosis histologically, qualifying only as anaplastic astrocytoma (Grade III) using World Health Organization criteria. Nevertheless, such tumors progressed rapidly, with mortality rates that were indistinguishable from their Grade IV counterparts. The median survival for 37 patients who were followed until death was 11 months. Oligodendroglioma-like histology included chicken-wire vasculature (86%), haloes (73%), perineuronal satellitosis (58%), and microcalcifications (45%), although mucin-filled rnicrocystic spaces were lacking. No small cell astrocytomas had 1p/19q codeletions, whereas EGFR amplification and 10q deletions were present in 69% and 97% of small cell astrocytomas, respectively. The tumors expressed EGFR and EGFR-vIII more commonly than nonsmall cell GBMs (83% vs. 35% [P