背景:H3K27M突变的神经胶质瘤在2016年WHO分类中首次被描述为新的4级实体。目前的研究集中在儿童和年轻人的典型外观上,越来越需要更好地了解预后因素和手术对成人的影响。这里,我们报道了一项针对成人这一实体的多中心研究.
方法:我们纳入了2016年至2022年诊断的18岁以上患者的分子确认的H3K27M突变神经胶质瘤病例。临床,放射学,并对手术特点进行分析。进行单变量和多变量分析以确定预后因素。
结果:在70名平均年龄为36.1岁的患者中,中位总生存期(OS)为13.6±14个月.14.3%的患者实现了大体全切除,而30%的患者进行了次全切除,54.3%进行了活检。位于端脑/间脑/髓脑的肿瘤与OS较差相关,而中脑/中脑的位置显示出明显更长的OS(8.7vs.25.0个月,p=0.007)。术前Karnofsky性能评分(KPS)<80显示操作系统降低(4.2vs.18个月,p=0.02)。此外,ATRX损耗,占25.7%,与操作系统的增加独立相关(31与8.3个月,p=0.0029)。值得注意的是,接受切除术的患者与活检相比没有生存获益(12vs.11个月,p=0.4006)。
结论:本研究在一项大型多中心研究中描述了成年期H3K27M突变神经胶质瘤的手术特征。我们的数据显示ATRX状态,位置和KPS显著影响H3K27M突变神经胶质瘤的OS。重要的是,我们的数据集表明,与活检相比,切除并不具有生存优势.
BACKGROUND: H3 K27M-mutated gliomas were first described as a new grade 4 entity in the 2016 World Health Organization classification. Current studies have focused on its typical appearance in children and young adults, increasing the need to better understand the prognostic factors and impact of surgery on adults. Here, we report a multicentric
study of this entity in adults.
METHODS: We included molecularly confirmed H3 K27M-mutated glioma cases in patients ≥ 18 years diagnosed between 2016 and 2022. Clinical, radiological, and surgical features were analyzed. Univariate and multivariate analyses were performed to identify prognostic factors.
RESULTS: Among 70 patients with a mean age of 36.1 years, the median overall survival (OS) was 13.6 ± 14 months. Gross-total resection was achieved in 14.3% of patients, whereas 30% had a subtotal resection and 54.3% a biopsy. Tumors located in telencephalon/diencephalon/myelencephalon were associated with a poorer OS, while a location in the mesencephalon/metencephalon showed a significantly longer OS (8.7 vs. 25.0 months, P = .007). Preoperative Karnofsky-Performance Score (KPS) ≤ 80 showed a reduced OS (4.2 vs. 18 months, P = .02). Furthermore, ATRX loss, found in 25.7%, was independently associated with an increased OS (31 vs. 8.3 months, P = .0029). Notably, patients undergoing resection showed no survival benefit over biopsy (12 vs. 11 months, P = .4006).
CONCLUSIONS: The present
study describes surgical features of H3 K27M-mutated glioma in adulthood in a large multicentric
study. Our data reveal that ATRX status, location and KPS significantly impact OS in H3 K27M-mutated glioma. Importantly, our dataset indicates that resection does not offer a survival advantage over biopsy.