976 名幕上胶质母细胞瘤 IDH 野生型患者的长期幸存者。
Long-term survivors in 976 supratentorial glioblastoma, IDH-wildtype patients.
发表日期:2024 Aug 30
作者:
Oumaima Aboubakr, Alessandro Moiraghi, Angela Elia, Arnault Tauziede-Espariat, Alexandre Roux, Arthur Leclerc, Martin Planet, Aziz Bedioui, Giorgia Antonia Simboli, Frédéric Dhermain, Eduardo Parraga, Chiara Benevello, Houssem Fathallah, Jun Muto, Fabrice Chrétien, Edouard Dezamis, Catherine Oppenheim, Pascale Varlet, Marc Zanello, Johan Pallud
来源:
JOURNAL OF NEUROSURGERY
摘要:
胶质母细胞瘤,异柠檬酸脱氢酶(IDH)野生型是最具侵袭性的胶质瘤,预后较差。作者探讨了携带胶质母细胞瘤 IDH 野生型的患者的生存率和与长期生存相关的因素。在一项观察性、回顾性、单中心研究中,作者检查了 976 名新诊断为幕上胶质母细胞瘤 IDH 的成人的医疗记录- 2000 年 1 月至 2021 年 1 月期间的野生型。他们分析了与 2 年和 5 年生存相关的临床、影像和治疗相关因素。中位总生存期为 11.2 个月(2005 年之后纳入的患者为 12.2 个月,引入标准联合放化疗)。中位无进展生存期为 9.4 个月(2005 年之后纳入的患者为 10.0 个月)。总体而言,17.6%的患者达到了2年总生存期,2.2%的患者达到了5年总生存期。此外,6.6% 的患者存活 2 年无进展,1.1% 的患者存活 5 年无进展。与 2 年和 5 年生存率一致相关的两个因素是采用标准联合放化疗的一线肿瘤治疗和甲基化 O6-甲基鸟嘌呤-DNA 甲基转移酶启动子。与 2 年或 5 年生存率显着相关的其他因素包括诊断时年龄 ≤ 60 岁、诊断时头痛或颅内压升高体征、对比增强的皮质接触、初始成像时对比剂增强未穿过中线、总或次全肿瘤切除术,以及复发时的二线肿瘤治疗。在 21 例 5 年生存者中,18 例被确认为胶质母细胞瘤,IDH 野生型,5 年生存者中有 7 例(38.9%)有额外的基因改变:3 例有 FGFR 突变或融合,3 例有 FGFR 突变或融合,3 例有 FGFR 突变或融合,3 例有 FGFR 突变或融合,3 例有PIK3CA突变,1例PTPN11突变,1例PMS2突变,存在体质错配修复缺陷综合征。 IDH野生型胶质母细胞瘤患者的五年总生存率极低。较长生存期的预测因素主要是治疗因素,这强调了在可行的情况下完整的肿瘤治疗计划的重要性。胶质母细胞瘤、IDH 野生型 5 年幸存者可以筛查复发情况下的可行靶点。
Glioblastoma, isocitrate dehydrogenase (IDH)-wildtype is the most aggressive glioma with poor outcomes. The authors explored survival rates and factors associated with long-term survival in patients harboring a glioblastoma, IDH-wildtype.In an observational, retrospective, single-center study, the authors examined the medical records of 976 adults newly diagnosed with supratentorial glioblastomas, IDH-wildtype between January 2000 and January 2021. They analyzed clinical-, imaging-, and treatment-related factors associated with 2-year and 5-year survival.The median overall survival was 11.2 months (12.2 months for patients included after 2005 and the introduction of standard combined chemoradiotherapy). The median progression-free survival was 9.4 months (10.0 months for patients included after 2005). Overall, 17.6% of patients reached a 2-year overall survival, while 2.2% of patients reached a 5-year overall survival. Furthermore, 6.6% of patients survived 2 years without progression, while 1.1% of patients survived 5 years without progression. Two factors that were consistently associated with 2-year and 5-year survival were first-line oncological treatment with standard combined chemoradiotherapy and methylated O6-methylguanine-DNA methyltransferase promoter. Other factors that were significantly associated with 2-year or 5-year survival were age at diagnosis ≤ 60 years, headaches or signs of raised intracranial pressure at diagnosis, cortical contact of contrast enhancement, no contrast enhancement crossing the midline on initial imaging, total or subtotal tumor resection, and a second line of oncological treatment at recurrence. Within 21 cases of 5-year survival, 18 were confirmed to be glioblastomas, IDH-wildtype, and 7 of the 5-year survivors (38.9%) had additional genetic alterations: 3 cases had an FGFR mutation or fusion, 3 cases had a PIK3CA mutation, 1 case had a PTPN11 mutation, and 1 case had a PMS2 mutation in the context of constitutional mismatch repair deficiency syndrome.Five-year overall survival in patients with glioblastoma, IDH-wildtype is extremely low. Predictors of a longer survival are mostly treatment factors, emphasizing the importance of a complete oncological treatment plan, when achievable. Glioblastoma, IDH-wildtype 5-year survivors could be screened for actionable targets in case of recurrence.