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