Sistema de Salut de Catalunya
Institut Català de la Salut
[Galera M, Álvarez R, Arregui M] Medical Oncology Department, Hospital Universitario Gregorio Marañón, Madrid, Spain. [Paniagua M] Radiology Department, Hospital Universitario Gregorio Marañón, Madrid, Spain. [Álvarez A] Radiation Oncology Department, Hospital Universitario Gregorio Marañón, Madrid, Spain. [González Crisostomo RA] De La Salle Medical and Health Sciences Institute, Governor, Damariñas, Philippines. [Díazgranados A] Servei d’Anatomia Patològica, Vall d’Hebron Hospital Universitari, Barcelona, Spain
Vall d'Hebron Barcelona Hospital Campus
2024-01-24T08:16:53Z
2024-01-24T08:16:53Z
2023
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.
Article
Published version
English
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
Karger
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/
Articles científics - HVH [3439]
