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A Clear Cell Sarcoma Case: A Diagnostic and Treatment Challenge, with a Promising Response to Trabectedin

Authors :
Mar Galera
Rosa Álvarez
Marta Arregui
Miguel Paniagua
Ana Álvarez
Raphael Angelo González Crisostomo
Adriana Díazgranados
Natalia Gutiérrez
Antonio Calles
Carolina Agra
Source :
Case Reports in Oncology, Vol 16, Iss 1, Pp 1542-1550 (2023)
Publication Year :
2023
Publisher :
Karger Publishers, 2023.

Abstract

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.

Details

Language :
English
ISSN :
16626575
Volume :
16
Issue :
1
Database :
Directory of Open Access Journals
Journal :
Case Reports in Oncology
Publication Type :
Academic Journal
Accession number :
edsdoj.19c3b8f342434bff92972852e9aed44e
Document Type :
article
Full Text :
https://doi.org/10.1159/000534935