Table 1 Promising Tumor Microenvironment and Antigen Targets in Pediatric Sarcomas

Target

Approach

Citations

Tumor microenvironment

Tumor-associated macrophages (TAMs)

Stratification, depletion

[6, 11]

Immune checkpoints

Checkpoint inhibitors, stratification

[17, 22,23,24,25,26,27,28,29,30]

Regulatory T cells

Chemotherapy (preconditioning found to promote anti-tumor immunity, possibly through reduction of regulatory immune populations)

[7, 86]

Sarcoma antigens

Neoantigens

Checkpoint inhibitors, vaccines, combination with PARPi

[35, 39, 68, 70]

Fusion-derived NeoAgs (e.g. EWSR1-FLI1)

Cancer vaccines, mithramycin, LSD1, lurbinectedin

[35, 44, 46, 47, 50, 78]

Cancer testis antigens (e.g. NY-ESO-1, MAGE family)

Cancer vaccines, tgTCR-T cells, CAR-T

[52, 45, 49, 79]

Gangliosides (GD2/GD3)

Immunotoxins, CAR-T, CAR-NK

[53, 56, 57, 89, 90]

HER2

CAR-T

[55, 61, 87]

CD99

possibly CAR-T

[62]

CD248

possibly CAR-T

[12, 42, 43]

IGF1R

Ganitumab

[64,65,66]

uPAR/EGFR

Bispecific immunotoxin

[67]