IDH-野生型胶质母细胞瘤长期存活病例

IF 1.1 4区 医学 Q4 CLINICAL NEUROLOGY
Lauren M Webb, Bryan J Neth, Aditya Raghunathan, Patricia T Greipp, Cristiane M Ida, Ivan D Carabenciov, Michael W Ruff
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引用次数: 0

摘要

简介胶质母细胞瘤是一种致命的原发性中枢神经系统肿瘤。尽管对胶质母细胞瘤病理生理学的认识有所提高,治疗方法也有所进步,但IDH-野生型胶质母细胞瘤患者的中位总生存期仍为确诊后14至21个月:我们报告了一例 48 岁女性的病例,她因局灶性癫痫发作就诊,脑磁共振成像检查发现其右侧额叶肿块。她接受了大体全切除术,组织学诊断为胶质母细胞瘤。她接受了放疗和6个周期的卡莫司汀(BCNU)治疗。17个月后,她出现左侧偏瘫。她接受了一个周期的甲氯雷他敏、长春新碱(ONCOVIN)、丙卡巴嗪和泼尼松(MOPP)治疗。随后的监测成像显示了治疗反应。在确诊胶质母细胞瘤 27 年后,她出现了癫痫状态,并死于呼吸衰竭。尸检的神经病理学结果显示,她的病变与治疗有关,但没有复发性胶质母细胞瘤的证据。在她最初确诊后30多年进行的基因组检测显示,根据2021年世界卫生组织的标准,她的基因组特征诊断为IDH-野生型胶质母细胞瘤:该患者是已知存活时间最长的IDH-野生型胶质母细胞瘤患者之一,切除时病理证实为胶质母细胞瘤,且在最后一次治疗26年后无残留疾病证据。她具有与胶质母细胞瘤长期存活相关的多种因素,包括女性、年轻、Karnofsky评分高和多模式治疗。这个病例表明,胶质母细胞瘤确诊后是有可能长期存活的,而且很可能是由个人、肿瘤和治疗因素共同促成的。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A Case of Long-Term Survival After Glioblastoma, IDH-Wild Type.

Introduction: Glioblastoma is a uniformly lethal primary central nervous system neoplasm. Despite the increased understanding of its pathophysiology and treatment advancements, median overall survival for patients with glioblastoma, IDH-wild type remains 14 to 21 months from diagnosis.

Case report: We present the case of a 48-year-old female who presented with a focal seizure and was found to have a right frontal lobe mass on the brain magnetic resonance imaging. She underwent gross total resection and received a histological diagnosis of glioblastoma. She received radiotherapy and 6 cycles of carmustine (BCNU). Seventeen months later, she developed left hemiparesis. Imaging was concerning for tumor progression, and she was treated with 1 cycle of mechlorethamine, vincristine (oncovin), procarbazine, and prednisone (MOPP). Subsequent surveillance imaging demonstrated a therapeutic response. Twenty-seven years after her glioblastoma diagnosis, she developed status epilepticus and died from respiratory failure. Neuropathology on autopsy demonstrated extensive treatment-related changes but no evidence of recurrent glioblastoma. Genomic testing performed over 30 years after her original diagnosis revealed a profile diagnostic of glioblastoma, IDH-wild type per 2021 World Health Organization criteria.

Conclusions: This patient is one of the longest-known survivors of glioblastoma, IDH-wild type, with pathologic confirmation of glioblastoma at the time of her resection and no evidence of residual disease 26 years after her last treatment. She presented with multiple factors associated with long-term glioblastoma survivorship, including female sex, young age, high Karnofsky score, and multimodal therapy. This case shows that long-term survival after glioblastoma diagnosis is possible and likely mediated through a combination of individual, tumor, and treatment factors.

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来源期刊
Neurologist
Neurologist 医学-临床神经学
CiteScore
1.90
自引率
0.00%
发文量
151
审稿时长
2 months
期刊介绍: The Neurologist publishes articles on topics of current interest to physicians treating patients with neurological diseases. The core of the journal is review articles focusing on clinically relevant issues. The journal also publishes case reports or case series which review the literature and put observations in perspective, as well as letters to the editor. Special features include the popular "10 Most Commonly Asked Questions" and the "Patient and Family Fact Sheet," a handy tear-out page that can be copied to hand out to patients and their caregivers.
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