Clinicopathologic features of small cell glioblastomas

Clinicopathologic features of small cell glioblastomas
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DOI:
10.1007/s11060-015-2038-0
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发表时间:
2016-04-01
影响因子:
3.9
通讯作者:
Kimura, Hirohiko
Kimura, Hirohiko
中科院分区:
医学2区
文献类型:
--
作者:
Takeuchi, Hiroaki;Kitai, Ryuhei;Kimura, Hirohiko

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小细胞胶质母细胞瘤(SCGBM)是胶质母细胞瘤的一种变异型,其主要特征是小而单形的胶质细胞群。本研究的目的是研究SCGBM的临床、神经影像、病理和遗传学特征。对14例SCGBM患者的临床病理和遗传学特征进行分析。MRI将所有病例分为多灶型和孤立型,观察肿瘤微血管增生、瘤内坏死和血管周围淋巴细胞聚集的程度。免疫组织化学方法检测IDH1突变(IDH1R132H),荧光原位杂交法检测1p19q共缺失。患者年龄从23岁到92岁(中位数:71岁),其中3名女性,11名男性。患者总生存期为7~23个月,平均11个月。多灶型9例(%)。病理检查显示,SCGBM的微血管增生、坏死和淋巴细胞浸润有限。免疫组织化学显示,所有患者肿瘤细胞IDH1R132H均为阴性。FISH分析显示,在有信息的患者中,无SCGBM存在1p/19q共缺失。我们的研究表明,老年起病和多灶性病变是SCGBM的特点,与免疫反应降低、肿瘤细胞浸润性和预后不良有关。
Small cell glioblastoma (SCGBM) is a variant of glioblastomas characterized by a predominant population of small and monomorphic glial cells. The aim of the present study was to investigate clinical, neuroimaging, pathologic, and genetic features of SCGBM. The clinicopathologic and genetic features were evaluated in 14 patients with SCGBM. All cases were divided into multifocal and solitary type by MRI, and extent of microvascular proliferation, intratumoral necrosis, and perivascular lymphocytic accumulation were investigated. IDH1 mutations by immunohistochemistry (IDH1 R132H) and 1p 19q codeletion by fluorescence in situ hybridization were detected. Patients ranged from 23 to 92 years of age (median: 71 years), with three females and eleven males. The overall survival time of the patients ranged from 7 to 23 months (mean: 11 months). Nine patients (64 %) were the multifocal type. Pathologic study revealed that the microvascular proliferation, necrosis, and lymphocytic infiltration were limited in SCGBM. Immunohistochemically, tumor cells were negative for IDH1 R132H in all patients. FISH analysis demonstrated that no SCGBM had 1p/19q codeletion in informative patients. Our investigation suggested that an elderly onset and multifocal lesions were characteristics of SCGBM associated with degradation of the immune response, infiltrative feature of tumor cells, and an unfavorable prognosis.