Soft-Tissue and Osteosarcomas
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Oct 05 – Oct 12, 2026
RINGSIDE Phase II Study of Varegacestat for Treatment of Desmoid Tumors: A Randomized Dose-Finding Clinical Trial.
J CLIN ONCOL · Q1 JOURNAL - RANK #6/326TOP-TIER
The RINGSIDE Phase II trial evaluated varegacestat for aggressive desmoid tumors, a challenging connective tissue neoplasm. This randomized, dose-finding study showed the 1.2-mg once-daily dose achieved a median tumor volume reduction of -51.91% at week 16 and a confirmed objective response rate (ORR) of 35.7%. In an open-label extension, this dose further improved to a median best tumor volume change of -85.88% and an ORR of 57.1%, with a manageable safety profile. Varegacestat offers a promising new oral treatment option for desmoid tumors, a condition often managed by cancer specialists, and the 1.2-mg dose was selected for Phase III.
10.1200/JCO-26-00725
Sep 14 – Sep 21, 2026
Circulating Tumor DNA Profiling Defines Risk Classification in Patients With Ewing Sarcoma: A Report From the Children’s Oncology Group and the LEOPARD Study.
J CLIN ONCOL · Q1 JOURNAL - RANK #6/326TOP-TIER
This study prospectively validated circulating tumor DNA (ctDNA) as a prognostic factor and developed clinical-molecular risk groups in patients with Ewing sarcoma (EWS). Utilizing ultralow-pass whole-genome and hybrid capture panel sequencing on 395 patients, elevated baseline ctDNA was associated with inferior outcomes in localized (HR=2.36, p=.032) and metastatic disease (HR=2.15, p=.001). The research identified novel low-risk groups and differentiated patients into low-, intermediate-, and high-risk categories by integrating ctDNA burden with clinical-molecular features. These findings offer a crucial tool for risk stratification in EWS, directly impacting treatment planning and patient management in oncology.
10.1200/JCO-26-00285
Sep 07 – Sep 14, 2026
Management of Soft Tissue and Visceral Leiomyosarcomas.
JAMA ONCOL · Q1 JOURNAL - RANK #14/326TOP-TIER
This international consensus review synthesizes evidence on diagnosing and managing soft tissue and visceral leiomyosarcomas, a rare mesenchymal cancer, using literature searches and expert panel input. Complete surgical resection remains the cornerstone for localized disease, while advanced disease benefits from anthracycline- or gemcitabine-based combinations, trabectedin, and tyrosine kinase inhibitors, though optimal sequencing is undefined. Molecular heterogeneity is recognized but not yet integrated into routine care, and prospective perioperative chemotherapy data are lacking. Clinically, the findings support multidisciplinary, site-specific management but highlight persistent uncertainties requiring leiomyosarcoma-specific trials.
10.1001/jamaoncol.2026.3366
Personalized Circulating Tumor DNA Analysis for Predicting Outcomes and Tracking Response to Radiotherapy and Pembrolizumab in Localized Sarcomas: Analysis of the SU2C-SARC032 Trial.
J CLIN ONCOL · Q1 JOURNAL - RANK #6/326TOP-TIER
This phase II randomized trial investigated personalized circulating tumor DNA (ctDNA) analysis to predict outcomes and track response to radiotherapy and pembrolizumab in 106 patients with high-risk, localized soft tissue sarcomas (STS). Personalized ctDNA profiling detected baseline ctDNA in 85% of patients, correlating with tumor size and grade, and levels across the perioperative course were strongly associated with disease-free and overall survival. Patients receiving pembrolizumab, who had significantly better disease-free survival, exhibited a greater decrease in ctDNA levels after neoadjuvant therapy. These findings demonstrate ctDNA’s utility as an independent prognostic and monitoring tool in STS, supporting future ctDNA-guided interventional trials in cancer management.
10.1200/JCO-26-00769