
<b><i>Introduction:</i></b> 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. <b><i>Case Presentation:</i></b> 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. <b><i>Conclusion:</i></b> 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.
diagnosis, Otros calificadores::Otros calificadores::Otros calificadores::/farmacoterapia, Medicaments antineoplàstics - Ús terapèutic, Case Report, Tumors de parts toves - Tractament, Case report, Diagnosis, case report, clear cell sarcoma, radiotherapy, RC254-282, Other subheadings::Other subheadings::Other subheadings::/drug therapy, treatment, Radiotherapy, Neoplasms. Tumors. Oncology. Including cancer and carcinogens, Treatment, COMPUESTOS QUÍMICOS Y DROGAS::acciones y usos químicos::acciones farmacológicas::usos terapéuticos::antineoplásicos, trabectedin, ENFERMEDADES::neoplasias::neoplasias por tipo histológico::neoplasias de tejido conjuntivo y de tejidos blandos::neoplasias de tejido conjuntivo::sarcoma de células claras, DISEASES::Neoplasms::Neoplasms by Histologic Type::Neoplasms, Connective and Soft Tissue::Neoplasms, Connective Tissue::Sarcoma, Clear Cell, Clear cell sarcoma, Trabectedin, CHEMICALS AND DRUGS::Chemical Actions and Uses::Pharmacologic Actions::Therapeutic Uses::Antineoplastic Agents
diagnosis, Otros calificadores::Otros calificadores::Otros calificadores::/farmacoterapia, Medicaments antineoplàstics - Ús terapèutic, Case Report, Tumors de parts toves - Tractament, Case report, Diagnosis, case report, clear cell sarcoma, radiotherapy, RC254-282, Other subheadings::Other subheadings::Other subheadings::/drug therapy, treatment, Radiotherapy, Neoplasms. Tumors. Oncology. Including cancer and carcinogens, Treatment, COMPUESTOS QUÍMICOS Y DROGAS::acciones y usos químicos::acciones farmacológicas::usos terapéuticos::antineoplásicos, trabectedin, ENFERMEDADES::neoplasias::neoplasias por tipo histológico::neoplasias de tejido conjuntivo y de tejidos blandos::neoplasias de tejido conjuntivo::sarcoma de células claras, DISEASES::Neoplasms::Neoplasms by Histologic Type::Neoplasms, Connective and Soft Tissue::Neoplasms, Connective Tissue::Sarcoma, Clear Cell, Clear cell sarcoma, Trabectedin, CHEMICALS AND DRUGS::Chemical Actions and Uses::Pharmacologic Actions::Therapeutic Uses::Antineoplastic Agents
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