伴有IDH1突变的顶壁巨细胞胶质母细胞瘤1例报告

H. Bolly, A. Adam, H. Agustina, A. Faried, M. Arifin
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引用次数: 0

摘要

背景:巨细胞胶质母细胞瘤(GCG)是一种中枢神经系统的原发性胶质肿瘤。它占所有胶质母细胞瘤的不到1%,是罕见的胶质母细胞瘤之一。它与idh野生型胶质母细胞瘤具有相似的临床特征。我们报告一例在肿瘤切除和使用IDH1突变体染色的特异性免疫组织化学进行组织病理学检查后诊断为GCG的病例。病例介绍:一名43岁男性,伴有进行性头痛和左肢体偏瘫。MRI造影示右侧顶枕叶肿块,T1W序列等高信号,中心坏死,造影增强。T2W序列显示肿块内有高信号。采用俯卧位开颅切除肿瘤,全部切除。组织病理学检查及免疫组化染色显示胶质原纤维酸蛋白(Glial Fibrillary Acid Protein, GFAP)阳性,增殖指数高,Ki-67染色> 10%,IDH1-R132H突变体染色阳性。患者手术切除肿瘤后,持续放疗和化疗,存活38个月。结论:目前的治疗或患者的IDH1突变状态或两者在延长生存时间中的作用仍有待阐明,仍然是一个谜。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Parietal giant cell glioblastoma with IDH1 mutation: A case report
Background: Giant cell glioblastoma (GCG) is a primary glial tumor of the central nervous system. It accounts for < 1% of all glioblastoma and known as one of rare glioblastoma. It is correspondence a similar clinical feature to IDH-wildtype glioblastoma. We present a case with the diagnosis of GCG following tumor resection and histopathology examination using specific immunohistochemistry of IDH1 mutant staining. Case presentation: A 43-year-old male with progressive headache and left extremity hemiparesis. MRI with gadolinium contrast showed a mass at the right parieto-occipital lobes with the characteristic of iso-hyperintense signal on T1W sequence, central necrosis and enhancement of the gadolinium contrast. The T2W sequence showed a hyperintense signal in the mass. Craniotomy tumor removal was performed with prone position and total removal was achieved. Histopathology finding and the immunohistochemistry staining showed results of Glial Fibrillary Acid Protein (GFAP) positive, highly proliferation index which > 10% of Ki-67 staining and positive for IDH1-R132H mutant staining. The patient survived for 38 months since the tumor resection surgery and continuing the treatment with radiotherapy and chemotherapy. Conclusion: The role of the present therapy or IDH1 mutation status of the patient or both of them in prolonged survival time still has to be elucidated and remained a mystery.
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