Fig. 3
New cell cultures and patient-derived xenograft model of EPS with chemical space characterization. a PCB490 biopsy sample divided into distinct regions to create different primary tumor cell cultures for study. b Western blot demonstrating loss of INI1 in multiple primary tumor sites and in published EPS cell lines. c Histology of surgical biopsy of PCB490. d Immunohistochemical staining of PCB490 for INI1 shows absence in tumor cells (black arrow) but presence in co-mingled non-cancerous cells. e Histology of PCB490 patient-derived xenograft. f INI1 absence (black arrow) in immunohistochemical staining of PCB490 patient-derived xenograft. g Drug Screen V3 results from primary EPS cell cultures, published EPS cell lines, and a normal myoblast cell line. The heat values indicate drug sensitivity as IC50 values, scaled between 10 nM (red) and 10 μM (white, representing no IC50 achieved) h Heatmap of Pearson correlation coefficients of 60-agent drug screen results between a normal myoblast cell line (SkMC), three EPS cell lines (ESX, VA-ES-BJ, FU-EPS-1), three sites from PCB490 (PCB490–3, PCB490–4, PCB490–5), and an additional EPS patient-derived culture (PCB495). The heat values correspond to Pearson correlation coefficients between drug sensitivities of different cell models