Soft-Tissue and Osteosarcomas
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May 25 – Jun 01, 2026
Fibroblast growth factor receptor inhibition for succinate dehydrogenase-deficient gastrointestinal stromal tumors: a phase 2 trial.
NAT MED · Q1 JOURNAL - RANK #1/195TOP-TIER
This prospective, single-arm phase 2 trial evaluated the pan-FGFR inhibitor rogaratinib in advanced SDH-deficient gastrointestinal stromal tumors, with a primary objective of objective response rate and secondary endpoints of progression-free survival (PFS) and safety; exploratory serial biopsies assessed FGF3/FGF4, FGFRs, whole-exome sequencing, and PK/PD. Twenty-four patients were treated; 10 achieved partial responses for an ORR of 41.7%. Median PFS was 31.0 months (95% CI 20.2–not reached) and 1-year PFS was 77.4% (95% CI 61.7–97.1). Toxicities were manageable (hyperphosphatemia, fatigue, diarrhea), phosphorus elevations indicated FGFR1 target engagement, and sequencing confirmed SDHx alterations, supporting FGFR inhibition as an effective targeted therapy in this epigenetically driven GIST subtype (NCT04595747).
10.1038/s41591-026-04376-9
May 11 – May 18, 2026
Dose Reduction of Preoperative Radiotherapy in Myxoid Liposarcoma: The Phase 2 DOREMY Nonrandomized Clinical Trial.
JAMA ONCOL · Q1 JOURNAL - RANK #14/326TOP-TIER
The DOREMY trial is a phase 2 nonrandomized prospective clinical trial evaluating a reduced dose of preoperative radiotherapy (36 Gy in 2-Gy fractions) for myxoid liposarcoma (MLS). It enrolled 90 adult patients from nine centers in Europe and the US, who were followed for a median of 66.4 months. The 5-year outcomes demonstrated a local recurrence-free survival of 97.4%, progression-free survival of 81.0%, and disease-specific survival of 89.5%, with wound complications in 21% of patients and late grade 2/3 toxic effects in 18%. The study concludes that dose-reduced preoperative radiotherapy provides excellent local control with a favorable toxicity profile, making it an appropriate treatment option for MLS patients in the absence of a phase 3 trial for this rare cancer.
10.1001/jamaoncol.2026.1577
Targeting CD3L1-NRP2 disarms myeloid-driven tumor immune evasion.
EMBO MOL MED · Q1 JOURNAL - RANK #21/195
This ongoing prospective clinical trial investigates the efficacy of anti-CD3L1, a monoclonal antibody, in advanced solid tumors to overcome tumor immune evasion. A mechanistic approach combined preclinical models, companion animal studies, and a human trial to analyze CD3L1’s role in T-cell suppression and macrophage polarization. The therapy surprisingly activated TAMs, with neuropilin-2 identified as a critical receptor mediating immunosuppressive M2 to anti-tumor M1 repolarization, leading to notable tumor inhibition. Clinical data confirmed robust TME remodeling and underscore anti-CD3L1’s potential as a novel immunotherapy.
10.1038/s44321-026-00451-3
May 04 – May 11, 2026
Phase 2 study of palbociclib plus retifanlimab in patients with advanced dedifferentiated liposarcoma.
J IMMUNOTHER CANCER · Q1 JOURNAL - RANK #12/183TOP-TIER
This prospective, single-arm, phase 2 trial aimed to investigate the efficacy of palbociclib plus retifanlimab in patients with advanced dedifferentiated liposarcoma. It included 30 patients with metastatic or unresectable DDLPS, receiving palbociclib (125 mg orally, days 1-21) and retifanlimab (500 mg IV every 4 weeks). The combination yielded an ORR of 20% (95% CI 8% to 39%) and a median PFS of 4.8 months with generally manageable toxicity. Copy number alteration burden and JUN amplification emerged as potential biomarkers of resistance, while the combination showed deep and durable responses in a subset of patients.
10.1136/jitc-2025-014346
Apr 27 – May 04, 2026
Neoadjuvant chemotherapy for soft-tissue sarcoma of the extremities: A post-hoc Sarculator-based risk analysis of the EORTC 62961-ESHO 95 randomized trial.
CANCER-AM CANCER SOC · Q1 JOURNAL - RANK #68/326
This study assessed the survival benefits of neoadjuvant chemotherapy (NAC) combined with regional hyperthermia (RHT) in patients with extremity soft tissue sarcoma (ESTS) based on risk predictions from the Sarculator nomogram. Conducted as a post-hoc analysis of the EORTC 62961-ESHO 95 randomized trial (135 patients analyzed, median follow-up of 136 months), results showed an absolute 5-year overall survival (OS) improvement of 15.6% (95% CI: 0.0%-31.4%) with a hazard ratio of 0.67 (95% CI: 0.39-1.17, p=0.081) for NAC+RHT versus NAC alone. Higher-risk patients derived greater benefits from NAC+RHT, though interaction analyses between risk score and treatment were not significant (p=0.495). Findings validated the previously reported survival advantage of combining RHT with NAC in ESTS but suggest individualized treatment decisions shouldn’t rely solely on risk estimates.
10.1002/cncr.70427
Apr 20 – Apr 27, 2026
A Phase 1 dose-escalation study to evaluate safety, pharmacokinetics, and pharmacodynamics of OSE-279, an anti-PD-1 monoclonal antibody in patients with advanced solid tumours.
EUR J CANCER · Q1 JOURNAL - RANK #45/326
This phase 1, open-label, dose-escalation trial evaluated the safety, pharmacokinetics, pharmacodynamics, and antitumor activity of OSE-279, an anti-PD-1 monoclonal antibody, in 20 patients with advanced solid tumors. Patients received intravenous OSE-279 at 100 mg q3w, 300 mg q3w, or 600 mg q6w. Key findings included two recommended phase 2 doses (300 mg q3w, 600 mg q6w), linear PK with >80% receptor occupancy, and durable responses: 1 complete response, 4 partial responses, and 7 stable disease, with response duration ranging from 6.8 to 18.4 months. The authors concluded OSE-279 monotherapy is well tolerated and shows durable antitumor activity, with the trial continuing in combination with a therapeutic cancer vaccine.
10.1016/j.ejca.2026.116729
Apr 13 – Apr 20, 2026
Atezolizumab for Alveolar Soft Part Sarcoma: A Clinical Trial Update.
J CLIN ONCOL · Q1 JOURNAL - RANK #6/326TOP-TIER
This phase II prospective clinical trial evaluated long-term outcomes of atezolizumab in 53 patients with metastatic alveolar soft part sarcoma, providing an additional three years of follow-up to earlier results. Patients received single-agent atezolizumab; genomic subtyping of ASPSCR1::TFE3 fusion was performed, and a subset underwent protocol-defined drug holidays or subsequent combination therapy with bevacizumab upon progression. Overall objective response rate was 35.8 % (95 % CI 23.1-50.2 %), median duration of response reached 37.0 months, and median progression-free survival was 20.8 months; type 1 fusion cases showed higher ORR (43.9 %) and longer mPFS (28.3 months) than type 2 (0 % ORR, mPFS 7.5 months; HR 3.2). Investigators conclude that prolonged atezolizumab therapy yields durable benefit in ASPS and that monitored drug holidays may be feasible, although adding bevacizumab after progression showed no objective responses.
10.1200/JCO-25-02811
First-in-Human Study of CEB-01: Novel Loco-Regional SN-38-Releasing Membrane to Prevent Local Recurrence in Retroperitoneal Sarcomas.
ANN SURG ONCOL · Q1 JOURNAL - RANK #39/312
This first-in-human, multicenter phase 1 trial evaluated CEB-01, a novel loco-regional SN-38-releasing membrane implanted after resection, to prevent local recurrence in retroperitoneal sarcoma patients using a 3+3 dose escalation design across six Spanish centers. Fourteen patients (median age 63 years) received implants at three SN-38 dose levels (9, 18, and 36 mg), with the primary objectives being to establish the recommended phase 2 dose (RP2D) and assess safety, efficacy, and pharmacokinetics. No dose-limiting toxicities occurred, the RP2D was set at 18 mg, and with a median follow-up of 9.1 months, all patients at RP2D were recurrence-free and alive, while pharmacokinetics showed substantially lower Cmax (20-70-fold) and longer half-life than intravenous irinotecan. The study concluded that CEB-01 at 18 mg exhibited a tolerable safety profile and preliminary efficacy against postsurgery recurrence, warranting further clinical investigation.
10.1245/s10434-026-19487-3
Trabectedin and low-dose irinotecan to target EWS::FLI1 in Ewing sarcoma: a phase 1/2 trial.
NAT MED · Q1 JOURNAL - RANK #1/195TOP-TIER
This open-label phase 1/2 clinical trial (SARC037; NCT04067115) evaluated trabectedin (1-hour infusion) in combination with low-dose irinotecan in 37 patients with relapsed/refractory Ewing sarcoma, with phase 1 focused on safety/tolerability and recommended phase 2 dose (RP2D), and phase 2 on objective response rate (ORR); secondary endpoints included progression-free survival (PFS), 6-month PFS, duration of response, and 18F-FLT PET avidity. The RP2D was trabectedin 1.0 mg/m^2 on day 1 plus irinotecan 25 mg/m^2 on days 2 and 4 in a 21-day cycle, with manageable toxicities and grade ≥3 events >15% for myelosuppression and alanine aminotransferase elevations. The phase 2 ORR was 33% (39% when including RP2D phase 1 patients), and the 6-month PFS was 48%; transcriptional profiling demonstrated reversal of the EWS::FLI1 transcriptome in tumors from a subset of patients. These findings indicate clinically meaningful activity and target engagement, supporting further development of trabectedin/irinotecan for Ewing sarcoma in international cooperative groups.
10.1038/s41591-026-04340-7
Apr 06 – Apr 13, 2026
A randomized clinical study comparing trabectedin combined with regional hyperthermia with trabectedin in patients with advanced soft tissue sarcoma: HyperTET, a German Interdisciplinary Sarcoma Group trial.
ESMO OPEN · Q1 JOURNAL - RANK #36/326
This randomized, open-label, multicenter trial evaluated the efficacy and safety of trabectedin plus regional hyperthermia (Tr + RHT) versus trabectedin alone (Tr) in adults with advanced soft tissue sarcoma that had progressed after at least one line of anthracycline-based chemotherapy or who were unsuitable for such treatment. A total of 118 patients from five German centers were randomized 1:1 to Tr + RHT (n = 60) or Tr (n = 58) every 3 weeks, with progression-free survival (PFS) in the intention-to-treat population as the primary endpoint; patients received a median of 3 cycles (IQR 2–6.2) in Tr + RHT and 4 cycles (IQR 2–6) in Tr. Median PFS was 3.0 months (95% CI 2.5–5.0) with Tr + RHT versus 3.5 months (95% CI 2.8–5.9) with Tr (stratified HR 0.86, 95% CI 0.57–1.29; P = 0.459), while a post hoc subgroup of patients receiving ≥5 cycles showed improved PFS with Tr + RHT (12.8 months, 95% CI 9.8–21.0) versus Tr (7.8 months, 95% CI 6.0–12.6; HR 0.33, 95% CI 0.13–0.86; P = 0.023). Adverse events were mainly grade 3/4 hematologic, hepatic, and infections, and three treatment-related deaths (5.3%) occurred in the Tr arm; overall, adding RHT did not improve PFS, though exploratory findings in patients receiving ≥5 cycles suggest potential benefit warranting further study.
10.1016/j.esmoop.2026.106921