Clinical Trials to Increase Treatment Options in the Progressive Setting

Author(s):

The panel reviews ongoing clinical trials aimed at expanding treatment options for progressive pediatric low-grade gliomas (pLGG), with a specific focus on the PNOC021, PBTC-055, and NF111 studies, discussing their objectives, design features, and potential impact on future treatment strategies.

EP: 1.Overview of pLGG presentation and categorization

EP: 2.Molecular Characterization of pLGG

EP: 3.Less Common Molecular Alterations in pLGG and Testing Recommendations

EP: 4.Standard of care for frontline treatment of non-V600E-specific pLGG tumors

EP: 5.Ongoing clinical trials for frontline treatment of non-V600E-specific pLGG tumors

EP: 6.Current Treatment of Patients with NF1 pLGG

EP: 7.Standard of Care Pptions Specific for Frontline Treatment of BRAF V600E-Mutated pLGG

EP: 8.Treatment Considerations for Progressive pLGG

EP: 9.Radiation and Targeted Therapies for Progressive pLGG

EP: 10.The Role of Tovorafenib in Treatment of Progressive pLGG

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EP: 11.Clinical Trials to Increase Treatment Options in the Progressive Setting

EP: 12.Clinical scenario 1 - BRAFKIAA-1549 fusion

EP: 13.Alternatives and Considerations for Clinical Scenario 1

EP: 14.Clinical Scenario 2 - BRAFV600E

EP: 15.Potential Adverse Events With Targeted Therapies

EP: 16.Clinical Insights: Looking Forward for pLGG

Please briefly describe the various treatment options that might be considered in R/R pLGG
- Clinical trial enrollment
  - PNOC021: Phase I Trametinib + Everolimus in NF1 LGG/HGG (NCT04485559)
  - NF111: Phase II Poly-ICLC in NF1 LGG (NCT04544007)
  - PBTC-055: Phase I/II trial of Dabrafenib, Trametinib, and Hydroxychloroquine (HCQ) for BRAF V600E-mutant or Trametinib and HCQ for BRAF fusion/duplication or NF1 (NCT04201457)