Jan 2026: A Real-World Analysis of Outcomes in CIC-Rearranged Sarcomas

CIC-rearranged sarcomas (CRS) are rare tumors predominantly affecting young adults. Despite distinct biology, CRS are often treated like Ewing sarcomas with multi-agent chemotherapy, surgery, and radiotherapy, although the benefits of each remain unclear. We report the impact of treatment on response and survival.
Among 27 patients (median age 21.5 years; range 8–83), 14 (52%) had localized and 12 (44%) metastatic disease (1 unknown). In the localized group, 6 (43%) received chemotherapy (Response: 2 complete, CR; 1 partial, PR; 1 mixed, MR; 1 stable, SD; 1 progressive disease, PD); 5 (36%) died—3 (21%) had received chemotherapy. Among metastatic patients, 10 (83%) received This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited. © 2025 The Author(s). Cancer Medicine published by John Wiley & Sons Ltd. 2 of 10 Cancer Medicine, 2026 chemotherapy (1 CR, 5 PR, 2 MR, 1 PD); 9 died (one without chemotherapy). Local control was achieved in 12/14 (86%) localized and 7/12 (56%) metastatic patients. Only surgical resection was associated with improved OS (HR 0.13; p<0.01) and EFS (HR 0.26; p<0.01), particularly with R0 resection (EFS HR 0.23; p=0.02). At median follow-up of 18months, 2-year EFS was 56% vs. 8% (p=0.02) and OS was 67% vs. 23% (p=0.02) for localized vs. metastatic disease. Conclusion: Complete resection is critical in CRS. Chemotherapy benefit remains uncertain, underscoring the need for improved local control and novel, biology-driven therapies.