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55岁及以上人群中的异柠檬酸脱氢酶突变型星形细胞瘤:与年轻人相比的生存差异

Isocitrate dehydrogenase-mutant astrocytoma in persons aged 55 years and older: Survival differences versus the young.

作者信息

Siddiq Zainab, Gilani Ahmed, Ung Timothy H, Kleinschmidt-DeMasters Bette K

机构信息

Colorado School of Public Health, University of Colorado, Aurora, CO, United States.

Department of Pathology, University of Colorado Anschutz Medical Campus, Aurora, CO, United States.

出版信息

J Neuropathol Exp Neurol. 2025 Jul 1;84(7):611-616. doi: 10.1093/jnen/nlaf024.

Abstract

Isocitrate dehydrogenase (IDH)-mutant astrocytomas show a peak incidence in young and middle-aged adults and have relatively favorable outcomes. In patients with these tumors ≥55 years at diagnosis, clinical, histopathologic, and prognostic characteristics are less clear. Here, we compared histopathological, immunohistochemical, molecular, and overall survival of 34 patients aged ≥55 years with a group of 84 patients aged 19-54 years; all had IDH mutant astrocytomas. The older cohort had 14 World Health Organization (WHO) grade 2, 7 WHO grade 3, and 13 WHO grade 4 tumors versus 24, 32, and 28 WHO grade 2, 3, and 4 tumors in the younger group. Comparing equal-grade tumors in both cohorts, Kaplan-Meyer survival analysis revealed that patients ≥55 years of age showed worse prognosis despite receiving comparable treatment regimens (Stupp protocol). Roughly equal numbers of noncanonical IDH mutations were seen in both groups (11.76% in ≥55 vs 19.04% in <55). Older patients were more likely to show retention of nuclear protein alpha-thalassemia and mental retardation X-linked syndrome (ATRX) and/or absence of strong P53 staining by immunohistochemistry. Although patients ≥55 years of age with astrocytomas, IDH-mutant, had worse overall survival, many, particularly those with low-grade tumors, had 5 years or greater survival. Employing parallel treatment regimens with chemotherapy, radiation, and maximum safe resection may improve survival of older patients with IDH mutant gliomas.

摘要

异柠檬酸脱氢酶(IDH)突变型星形细胞瘤在中青年成人中发病率最高,且预后相对较好。对于诊断时年龄≥55岁的这些肿瘤患者,其临床、组织病理学和预后特征尚不清楚。在此,我们比较了34例年龄≥55岁患者与84例年龄在19 - 54岁患者的组织病理学、免疫组化、分子特征及总生存期;所有患者均患有IDH突变型星形细胞瘤。老年队列中有14例世界卫生组织(WHO)2级、7例WHO 3级和13例WHO 4级肿瘤,而年轻组分别有24例、32例和28例WHO 2级、3级和4级肿瘤。对两个队列中相同级别的肿瘤进行比较,Kaplan - Meyer生存分析显示,尽管接受了相似的治疗方案(Stupp方案),但年龄≥55岁的患者预后更差。两组中观察到的非典型IDH突变数量大致相等(≥55岁组为11.76%,<55岁组为19.04%)。老年患者更有可能通过免疫组化显示核蛋白α地中海贫血和智力发育迟缓X连锁综合征(ATRX)保留和/或缺乏强烈的P53染色。尽管年龄≥55岁的IDH突变型星形细胞瘤患者总生存期较差,但许多患者,尤其是低级别肿瘤患者,生存期达5年或更长。采用化疗、放疗和最大安全切除的平行治疗方案可能会改善老年IDH突变型胶质瘤患者的生存期。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ca65/12191623/a755677a60c2/nlaf024f1.jpg

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