2026-04-17T14:45:48Zhttps://recercat.cat/oai/requestoai:recercat.cat:11351/108842025-10-24T10:44:11Zcom_2072_378070com_2072_378040col_2072_378092
RECERCAT
author
Galera, Mar
author
Arregui, Marta
author
Paniagua-González, Miguel
author
Alvarez, Ana
author
González Crisostomo, Raphael Angelo
author
Díazgranados, Adriana
author
ALVAREZ, ROSA
2025-10-24T10:44:11Z
2025-10-24T10:44:11Z
2024-01-24T08:16:53Z2024-01-24T08:16:53Z2023
http://hdl.handle.net/11351/10884
Clear cell sarcoma; Radiotherapy; TrabectedinSarcoma de cèl·lules clares; Radioteràpia; TrabectedinaSarcoma de células claras; Radioterapia; TrabectedinaIntroduction: 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.
Attribution-NonCommercial 4.0 International http://creativecommons.org/licenses/by-nc/4.0/ info:eu-repo/semantics/openAccess
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
A Clear Cell Sarcoma Case: A Diagnostic and Treatment Challenge, with a Promising Response to Trabectedin
info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion