bims-myxlip 2023-12-17 papers

Issue of 2023–12–17
two papers selected by
Laura Mannarino, Humanitas Research

Case Rep Oncol. 2023 Jan-Dec;16(1):16(1): 1542-1550

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

Mar Galera, Rosa Álvarez, Marta Arregui, Miguel Paniagua, Ana Álvarez, Raphael Angelo González Crisostomo, Adriana Díazgranados, Natalia Gutiérrez, Antonio Calles, Carolina Agra.

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.

Keywords: Case report; Clear cell sarcoma; Diagnosis; Radiotherapy; Trabectedin; Treatment

DOI: https://doi.org/10.1159/000534935

Rare Tumors. 2023 ;15 20363613231212380

An extremely rare case of recurrent pleomorphic myxoidliposarcoma with response to eribulin chemotherapy - A case report.

Raghavendra Rao, Sameer Rastogi, Divya Kashyap, Shamim A Shamim, Adarsh Barwad.

Pleomorphic myxoid liposarcoma (PML) is a newly recognized entity with aggressive clinical behavior and a tendency to recur. It has histological features of both myxoid and pleomorphic liposarcoma and lacks the molecular and structural chromosomal abnormalities associated with myxoid and pleomorphic liposarcoma. The data about their response to chemotherapy is quite sparse. We report a case of incidentally detected pleomorphic myxoid liposarcoma of the mediastinum in a 32-year-old gentleman. After resection and adjuvant chemotherapy with doxorubicin and ifosfamide, there was no evidence of residual disease at the end of treatment. During a routine follow-up 5 months later, he was found to have a recurrence of the disease with histological confirmation. He received a trabectedin given its activity in myxoid liposarcoma. However, he had toxicities and progression leading to its discontinuation. Subsequently, eribulin was started as the next line of therapy. After 4 cycles of chemotherapy, response assessment was suggestive of partial response, which is still maintained after 7 cycles of eribulin. This is the first report of this entity responding to a newer chemotherapy regimen.

Keywords: Pleomorphic myxoid liposarcoma; eribulin; trabectedin

DOI: https://doi.org/10.1177/20363613231212380