Precision medicine approaches for the management of Ewing sarcoma: current perspectives

Figures & data

Table 1 Current TET–ETS fusion pairings identified in Ewing sarcoma

Translocation	Fusion	Frequency in ESFT
t(11;22)(q24;q12)	EWSR1–FLI1	~85%–90% of cases
t(21;22)(q22;q12)	EWSR1–ERG	~10% of cases
t(7;22)(p22;q12)	EWSR1–ETV1	Rare
t(17;22)(q12;q12)	EWSR1–ETV4	Rare
t(2;22)(q35;q12)	EWSR1–FEV	Rare
t(16;21)(p11;q22)	FUS–ERG	Rare
t(2;16)(q35;p11)	FUS–FEV	Rare

Abbreviation: ESFT, Ewing sarcoma family of tumors.

Table 2 Current clinical trials for advanced Ewing sarcoma

NCT no.	Patient population	Phase	Treatment	Mechanism of action
NCT02306161	Newly diagnosed, metastatic disease	III	Standard chemotherapy ± ganitumab	IGF-1R mAb
NCT03495921	Recurrence after one prior therapy	III	Temozolomide and irinotecan ± vigil	bi-shRNA(furin) and GM-CSF augmented autologous tumor cell immunotherapy
NCT01858168	Recurrence after >1 prior therapy	I	Olaparib and temozolomide ± irinotecan	PARP inhibitor
NCT02736565	Relapsed or refractory disease	I	pbi-shRNA^™ EWS/FLI1 Type 1 LPX	Functional plasmid DNA construct that targets EWS/FLI1 mRNA
NCT03514407	Relapsed or refractory disease	Ib	INCB059872	LSD1 inhibitor
NCT03600649	Relapsed or refractory disease	I	SP-2577 (seclidemstat)	LSD1 inhibitor
NCT02657005	Relapsed or refractory disease	I	TK216	ETS-family transcription inhibitor

Abbreviations: GM-CSF, granulocyte-macrophage colony-stimulating factor; IGF-1R, insulin-like growth factor one receptor; mAb, monoclonal antibody.