Ewing Sarcoma

Early comprehensive genomic profiling was essential for confirming this rare subtype. Despite transient responses to Ewing sarcoma regimens, rapid chemoresistance was observed, emphasizing the need for precise molecular diagnosis and novel therapeutic approaches.

These findings provide new insights into the molecular mechanisms underlying EWS metastasis. The online version contains supplementary material available at 10.1007/s10616-026-01001-y.

Further cell assay experiments confirmed that cells expressing individual fusion genes were more sensitive to the suggested drugs, and the key downstream genes were affected by our drugs. FusionTarget provides a unique foundation for developing therapeutics targeting fusion proteins.

P1, N=25, Recruiting, University of California, Irvine

In contrast, the EWSR1:R565A mutant, which lacks the ability to interact with Aurora B kinase, failed to rescue this phenotype. We propose that the combination of EWSR1-FLI1 expression and loss of EWSR1 contributes to the induction of trisomy 8 through the compromised EWSR1-Aurora B pathway.

The patient received Vincristine, Adriamycin, Cyclophosphamide Ifosfamide, Etoposide (VAC/IE) chemotherapy according to the European Ewing tumour Working Initiative of National Groups (EUROE.W.I.N.G.) protocol and achieved full neurological recovery. This case illustrates the diagnostic and therapeutic challenges posed by Ewing-like sarcomas in the absence of molecular confirmation. Function-preserving subtotal resection combined with multimodal chemotherapy may lead to excellent outcomes and may be preferable to radical resection when the latter carries a high neurological risk.

P=N/A, N=55, Not yet recruiting, Peking University People's Hospital

Hypertrophic conditions foster the proliferation of EwS cells as compared to avascular and mineralized environments and support the migration of highly aggressive EWSR1::FLI1low cell phenotype, contrarily to empty 3D fibrin gel. Overall, the results support the use of the ECO-mimicking microphysiological system as a valuable preclinical model to investigate the role of developmental mechanisms in EwS onset and progression.

A partial penectomy and adjuvant chemotherapy with vincristine, doxorubicin, and cyclophosphamide alternating once every 2 weeks with ifosphamide and etoposide were performed, with no recurrence at one year. This case highlights the importance of initial management of tumors of undetermined type, where complete surgical treatment is key to improving disease-free survival (DFS). To our knowledge, this is the first reported case in France.

P2, N=3, Active, not recruiting, National Cancer Institute (NCI) | Trial completion date: May 2026 --> May 2027

This study reveals a critical in vitro and in vivo discordance and suggests that Mito + Doxo co-administration may paradoxically induce multidrug resistance, abrogating backbone chemotherapy efficacy. These findings urge caution in preclinical combination strategies involving standard-of-care agents and underscore the need for further investigation prior to clinical translation in ES.

P2, N=406, Recruiting, Milton S. Hershey Medical Center | Not yet recruiting --> Recruiting