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The role of adjuvant chemotherapy in patients with H3K27 altered diffuse midline gliomas: a multicentric retrospective study

  • 期刊论文

  • 发表日期：

    2024年3月8日

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  • Vincenzo Di Nunno,
  • Giuseppe Lombardi,
  • Matteo Simonelli,
  • Giuseppe Minniti,
  • Angela Mastronuzzi,
  • Valentina Di Ruscio,
  • Martina Corrà,
  • Marta Padovan,
  • Marta Maccari,
  • Mario Caccese,
  • Giorgia Simonetti,
  • Arianna Berlendis,
  • Mariangela Farinotti,
  • Bianca Pollo,
  • Manila Antonelli,
  • Antonio Di Muzio,
  • Angelo Dipasquale,
  • Sofia Asioli,
  • Dario De Biase,
  • Alicia Tosoni,
  • Antonio Silvani,
  • Enrico Franceschi

摘要

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.