Sarcoma Research Updates

Among 1,613 patients with localized rhabdomyosarcoma treated across 126 European centres (2005–2016), diagnostic work-up and treatment quality indicators were broadly comparable across high-, medium-, and low-volume centres, though high-volume centres more often performed central pathology review and molecular testing and medium-volume centres used FDG-PET/CT more frequently. Risk-group misclassification occurred more often in low-volume centres, but five-year event-free and overall survival did not differ significantly between centre-volume groups, including high-risk subgroups.

This review of epigenetic targeting in solid tumours highlights advances in inhibitors of EZH2, PRMT5, and BET proteins. EZH2 inhibition has achieved the first clinical approval in SMARCB1‑deficient epithelioid sarcoma; PRMT5 inhibitors exploit synthetic lethality in MTAP‑deleted tumours with early activity and improved tolerability in next‑generation compounds; and BET inhibitors show biological promise but limited efficacy and toxicity as monotherapy, suggesting potential utility in combination regimens such as in glioblastoma.

A multidisciplinary panel used a modified Delphi process informed by systematic review and expert opinion to produce perioperative care recommendations specific to primary retroperitoneal sarcoma (RPS) surgery. Forty-two recommendations across preoperative, intraoperative, and postoperative domains were proposed; 33 reached consensus (16 strong, 17 weak) and 9 did not meet the predefined threshold, with most guidance supported by indirect evidence from major abdominal and oncologic surgery. These represent the first consensus-based, multidisciplinary perioperative guidelines tailored to primary RPS surgery and the authors call for prospective validation and international collaboration to refine implementation.