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oai:recercat.cat:11351/108842025-10-24T10:44:11Zcom_2072_378070com_2072_378040col_2072_378092

2025-10-24T10:44:11Z urn:hdl:11351/10884 A Clear Cell Sarcoma Case: A Diagnostic and Treatment Challenge, with a Promising Response to Trabectedin Galera, Mar Arregui, Marta Paniagua-González, Miguel Alvarez, Ana González Crisostomo, Raphael Angelo Díazgranados, Adriana ALVAREZ, ROSA Medicaments antineoplàstics - Ús terapèutic Tumors de parts toves - Tractament DISEASES::Neoplasms::Neoplasms by Histologic Type::Neoplasms, Connective and Soft Tissue::Neoplasms, Connective Tissue::Sarcoma, Clear Cell Other subheadings::Other subheadings::Other subheadings::/drug therapy CHEMICALS AND DRUGS::Chemical Actions and Uses::Pharmacologic Actions::Therapeutic Uses::Antineoplastic Agents ENFERMEDADES::neoplasias::neoplasias por tipo histológico::neoplasias de tejido conjuntivo y de tejidos blandos::neoplasias de tejido conjuntivo::sarcoma de células claras Otros calificadores::Otros calificadores::Otros calificadores::/farmacoterapia COMPUESTOS QUÍMICOS Y DROGAS::acciones y usos químicos::acciones farmacológicas::usos terapéuticos::antineoplásicos Clear cell sarcoma; Radiotherapy; Trabectedin Sarcoma de cèl·lules clares; Radioteràpia; Trabectedina Sarcoma de células claras; Radioterapia; Trabectedina Introduction: Clear cell sarcoma (CCS) is a rare and aggressive soft tissue sarcoma. CCS is characterized by the translocation t(12;22) (q13;q12), involving the fusion of EWSR1 and ATF1 genes, and less frequently the fusion gene EWSR1-CREB1. Usually, CCSs are considered poorly responsive to conventional chemotherapy. However, trabectedin has shown activity against translocation-related sarcomas. Furthermore, preclinical results suggest that trabectedin is a promising antitumor agent for CCS, potentially inducing melanocytic differentiation. Case Presentation: We report the case of a challenging anatomopathological diagnosis in a patient with an aggressive metastatic CCS. Following the diagnosis of CCS, the patient experienced a clinical and radiological tumor response to trabectedin after four lines of treatment. Conclusion: This is a novel report of CCS treated with trabectedin that resulted in a partial response and suggests the need for further research on trabectedin as a therapeutic option for CCS. This research was funded by PharmaMar, S.A. 2025-10-24T10:44:11Z 2025-10-24T10:44:11Z 2024-01-24T08:16:53Z 2024-01-24T08:16:53Z 2023 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion http://hdl.handle.net/11351/10884 Case Reports in Oncology;16(1) https://doi.org/10.1159/000534935 Attribution-NonCommercial 4.0 International http://creativecommons.org/licenses/by-nc/4.0/ info:eu-repo/semantics/openAccess Karger Scientia