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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.
Effective targeted treatment is unavailable for most sarcomas and doxorubicin and ifosfamide-which have been used to treat soft-tissue sarcoma for more than 30 years-still have an important role. Whether doxorubicin alone or the combination of doxorubicin and ifosfamide should be used routinely is still controversial. We assessed whether dose intensification of doxorubicin with ifosfamide improves survival of patients with advanced soft-tissue sarcoma compared with doxorubicin alone.
This multicenter study, to our knowledge, is the first phase III trial to compare trabectedin versus dacarbazine in patients with advanced liposarcoma or leiomyosarcoma after prior therapy with an anthracycline and at least one additional systemic regimen.
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