Table 1.

Compounds shown pre-clinically to target telomere maintenance mechanisms, to be prioritised for evaluation in neuroblastoma

Drug	Target	Pre-clinical Data	Clinical Trials
6-thio-dG	Telomerase	Pre-clinical efficacy in TERT activated neuroblastoma models	No
Tetra-Pt (bpy)	ALT	In-vitro and in-vivo activity in U2OS ALT osteosarcoma model	No
CX-5461	ALT	In-vitro activity in U2OS and SAOS2 ALT osteosarcoma cell lines	On-going adult phase I clinical trial (NCT0271997)
Pifithrin-α	ALT	In-vitro and in-vivo activity in U2OS ALT osteosarcoma model	No
Trabectedin	ALT	In-vitro activity in a panel of ALT cell lines (sarcoma, breast cancer and melanoma)	-FDA approval for certain soft tissue sarcomas [68] -Paediatric sarcoma phase II data [69] -On-going paediatric clinical trials (NCT04067115)