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Case Reports in Oncology
Article . 2023 . Peer-reviewed
License: CC BY NC
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Case Reports in Oncology
Article . 2023
Data sources: DOAJ
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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; +3 Authors

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

Abstract

<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.

Keywords

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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selected citations
These citations are derived from selected sources.
This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Citations provided by BIP!
popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
BIP!Popularity provided by BIP!
influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Influence provided by BIP!
impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
BIP!Impulse provided by BIP!
2
Average
Average
Average
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gold
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