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 phase 2 trial investigated the pan-fibroblast growth factor receptor inhibitor rogaratinib in 24 patients with advanced succinate dehydrogenase-deficient gastrointestinal stromal tumors (SDH-deficient GIST). The study found an objective response rate of 41.7%, with a median progression-free survival of 31.0 months and a 1-year PFS of 77.4%. Rogaratinib demonstrated manageable toxicities, including hyperphosphatemia, consistent with target engagement. This research highlights a successful targeted cancer therapy for SDH-deficient GIST, predicated on an epigenetic mechanism of oncogene activation, offering a promising new treatment strategy.
10.1038/s41591-026-04376-9
May 18 – May 25, 2026
Dose Reduction of Preoperative Radiotherapy in Myxoid Liposarcoma: The Phase 2 DOREMY Nonrandomized Clinical Trial.
JAMA ONCOL · Q1 JOURNAL - RANK #14/326TOP-TIER
This phase 2 nonrandomized trial (DOREMY) evaluated the efficacy of a reduced preoperative radiotherapy dose of 36 Gy followed by surgical resection in 90 patients with localized myxoid liposarcoma. At a median follow-up of 66.4 months, the study reported a 5-year local recurrence-free survival rate of 97.4% and an overall survival rate of 88.5%. Wound complications occurred in 21% of patients, while late grade 3 toxic effects were minimal at only 3%. These findings demonstrate that dose reduction maintains excellent local control with a favorable toxicity profile, supporting its adoption as a standard treatment option for this specific cancer subtype.
10.1001/jamaoncol.2026.1577
Apr 27 – May 04, 2026
Bayesian sequential learning for prognostication in extremity soft tissue sarcoma (BayeSarc): a retrospective, multicentre cohort study.
LANCET ONCOL · Q1 JOURNAL - RANK #8/326TOP-TIER
This retrospective, multicentre cohort study developed BayeSarc, a Bayesian sequential learning model, to provide continuously updated and more accurate prognostication for overall survival and distant metastasis in 4916 patients with resected extremity soft tissue sarcomas. BayeSarc demonstrated superior discrimination compared to the existing Sarculator tool, with C-indices of 0.801 versus 0.773 for overall survival and 0.738 versus 0.718 for distant metastasis, alongside improved calibration and reduced uncertainty. This continuously updatable, accurate, and precise prognostic tool is highly relevant for cancer management. Its integration into the Sarculator app offers immediate clinical utility, enhancing patient counselling, guiding treatment decisions, and refining trial design for this specific cancer.
10.1016/S1470-2045(26)00067-7
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 trial update reports on three additional years of observation for 53 patients with alveolar soft part sarcoma (ASPS) treated with atezolizumab monotherapy. The median duration of response increased to 37.0 months, with an objective response rate (ORR) of 35.8% and median progression-free survival (mPFS) of 20.8 months. Patients with ASPSCR1::TFE3 type 1 fusion showed higher ORR (43.9%) and mPFS (28.3 months) compared to type 2 (0% ORR, 7.5 months mPFS). These long-term results provide crucial evidence for the efficacy of atezolizumab in treating a specific cancer, ASPS, and suggest that prolonged treatment is beneficial, with drug holidays potentially feasible for some patients.
10.1200/JCO-25-02811