Purpose

Adult Diffuse midline glioma (DMG) is a very rare disease. DMGs are currently treated with radiotherapy and chemotherapy even if only a few retrospective studies assessed the impact on overall survival (OS) of these approaches.

Methods

We carried out an Italian multicentric retrospective study of adult patients with H3K27-altered DMG to assess the effective role of systemic therapy in the treatment landscape of this rare tumor type.

Results

We evaluated 49 patients from 6 Institutions. The median age was 37.3 years (range 20.1–68.3). Most patients received biopsy as primary approach (n = 30, 61.2%) and radiation therapy after surgery (n = 39, 79.6%). 25 (51.0%) of patients received concurrent chemotherapy and 26 (53.1%) patients received adjuvant temozolomide. In univariate analysis, concurrent chemotherapy did not result in OS improvement while adjuvant temozolomide was associated with longer OS (21.2 vs. 9.0 months, HR 0.14, 0.05–0.41, p < 0.001). Multivariate analysis confirmed the role of adjuvant chemotherapy (HR 0.1, 95%CI: 0.03–0.34, p = 0.003). In patients who progressed after radiation and/or chemotherapy the administration of a second-line systemic treatment had a significantly favorable impact on survival (8.0 vs. 3.2 months, HR 0.2, 95%CI 0.1–0.65, p = 0.004).

Conclusion

In our series, adjuvant treatment after radiotherapy can be useful in improving OS of patients with H3K27-altered DMG. When feasible another systemic treatment after treatment progression could be proposed.

中文翻译：

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辅助化疗在 H3K27 改变的弥漫性中线胶质瘤患者中的作用：一项多中心回顾性研究

目的

成人弥漫性中线胶质瘤（DMG）是一种非常罕见的疾病。DMG 目前采用放疗和化疗进行治疗，尽管只有少数回顾性研究评估了这些方法对总生存 (OS) 的影响。

方法

我们对患有 H3K27 改变的 DMG 的成年患者进行了一项意大利多中心回顾性研究，以评估全身治疗在这种罕见肿瘤类型的治疗中的有效作用。

结果

我们评估了来自 6 个机构的 49 名患者。中位年龄为 37.3 岁（范围 20.1-68.3）。大多数患者接受活检作为主要方法 ( n  = 30, 61.2%) 并在手术后接受放射治疗 ( n  = 39, 79.6%)。25 名（51.0%）患者接受同步化疗，26 名（53.1%）患者接受替莫唑胺辅助治疗。在单变量分析中，同步化疗并未导致 OS 改善，而辅助替莫唑胺与更长的 OS 相关（21.2 个月与 9.0 个月，HR 0.14、0.05-0.41，p  < 0.001）。多变量分析证实了辅助化疗的作用（HR 0.1，95%CI：0.03-0.34，p  = 0.003）。对于放疗和/或化疗后病情进展的患者，二线全身治疗对生存具有显着有利的影响（8.0 个月与 3.2 个月，HR 0.2，95%CI 0.1-0.65，p  = 0.004）。

结论

在我们的系列中，放疗后的辅助治疗可有助于改善 H3K27 改变的 DMG 患者的 OS。如果可行，可以在治疗进展后提出另一种全身治疗。