Uncovering therapeutic vulnerabilities in clear cell sarcoma

Clear Cell Sarcoma (CCS) are ultra-rare fusion-translocated soft-tissue tumors occurring mainly in young adults with poor prognosis. Their high metastatic potential and resistance to conventional chemotherapy characterize these tumors. In this project, we aimed to identify transcriptional alterations between primary tumor derived cells and their matched lung metastastis to uncover vulnerabilities in a metastatic setting. For this we established cell lines from a 23 year old female CCS patient. The cell line "MUG Lucifer prim" was estbalished from the primary tumour in the knee and "MUG Lucifer met" from the metastatic lesion in the lung. Furthermore we tested the impact of lurbinectedin, an inibitor of oncogenic transcription, on transcriptional signatures of these cell lines and correlated the results with efficiency of lurbinectedin in vitro and in mouse xenografts.