Sarcomas

The FDA has accepted for priority review the BLA seeking the approval of afamitresgene autoleucel for the treatment of advanced synovial sarcoma.

R. Lor Randall, MD, FACS, discusses the early efficacy seen with vimseltinib monotherapy and pexidartinib plus surgery in tenosynovial giant cell tumors.

The European Medicines Agency has granted orphan drug designation to pimicotinib for use in patients with inoperable tenosynovial giant cell tumor.

R. Lor Randall, MD, FACS, emphasizes the overall potential for investigating the utility of engineered bone marrow in patients with osteosarcoma.

The NCCN has updated its guidelines for soft tissue sarcoma to include nirogacestat as a systemic therapy option for patients with desmoid tumors.

Research has continued to focus on developing novel therapies and bringing new treatments to the sarcoma treatment paradigm.

The FDA has granted an orphan drug designation to AL102 as a potential therapeutic option for patients with desmoid tumors.

Vimseltinib elicited a statistically significant and clinically meaningful improvement in 25-week overall response rate vs placebo in patients with tenosynovial giant cell tumor that was not amenable to surgery.

Meredith McKean, MD, MPH, discusses the efficacy and safety of treatment with the anti-TGIT agent etigilimab and the PD-1 inhibitor nivolumab in patients with solid tumors and PD-L1-low disease.

R. Lor Randall, MD discusses the primary results of the PARITY study and their implications for the use of antibiotics for patients with lower extremity bone tumors undergoing surgery and highlighted the findings from the secondary analysis of the study on the effect of surgical duration on risk of infection

Damon R. Reed, MD, discusses the genetic differences that distinguish Ewing sarcoma from osteosarcoma, as well as unmet needs in patients with these diseases.

R. Lor Randall, MD, FACS, discusses the process of organizing the symposium, key surgical and non-surgical advancements as well as socioeconomic disparities identified in these papers, and the importance of this information for both surgical and medical oncologists.

Breelyn Wilky, MD, discusses the rationale for investigating the addition of doxorubicin to zalifrelimab and balstilimab in patients with advanced/metastatic soft tissue sarcoma, expands on results from the phase 2 trial, and emphasizes the need for continued research for the potential role of immunotherapy in this patient population.

Brian A. Van Tine, MD, PhD, discusses findings from the overall survival analysis of the phase 2 SPEARHEAD-1 trial in patients with advanced synovial sarcoma.

R. Lor Randall, MD, FACS, explains the difference between noncancerous chondroblastoma and other bone neoplasms, details the typical diagnosis and treatment of this rare condition, and emphasizes the importance of pediatric and medical oncologists being able to identify it in clinic.

The PD-1 inhibitor pembrolizumab displayed activity with a manageable safety profile in patients with certain rare and ultra-raresarcoma histotypes.

Treatment with camsirubicin led to a reduction in tumor size in patients with advanced soft tissue sarcoma.

The FDA has approved the therascreen PDGFRA RGQ PCR kit for use as a companion diagnostic, co-developed by Qiagen and Blueprint Medicines, to assist in the identification of patients with gastrointestinal stromal tumors who may be candidates to receive avapritinib.

R. Lor Randall, MD, FACS, provides details about the 2024 Birmingham Orthopedic Oncology Meeting and its main topic of discussion, explains the rationale for its inception, and emphasizes the importance of this meeting for orthopedic oncologists treating chondrosarcoma.

Jonathan C. Trent, MD, PhD, discusses data from 2 phase 2 trials of immunotherapy in soft tissue sarcoma, as well as their clinical significance.

Theseus Pharmaceuticals has announced that the development of THE-630 as a potential therapeutic option for patients with gastrointestinal stromal tumor has been terminated, and enrollment to the ongoing phase 1/2 trial evaluating the agent’s safety and efficacy in this population has been discontinued.

Andrew S. Brohl, MD, discusses the safety and immunologic responses with intratumoral administration of Idx-Hu2.0 in patients with advanced Merkel cell carcinoma and cutaneous squamous cell carcinoma.

Breelyn A. Wilky, MD, discusses the rationale for investigating the combination of zalifrelimab, balstilimab, and doxorubicin in patients with advanced/metastatic soft tissue sarcoma, and early results from a phase 2 trial.

Damon R. Reed, MD, discusses a phase 2 study of fam-trastuzumab deruxtecan-nxki in adolescent and young adult patients with recurrent HER2-positive osteosarcoma.

R. Lor Randall, MD, FACS, discusses the use of neoadjuvant pazopanib in patients with non-rhabdomyosarcoma soft tissue sarcoma, expanded on findings from the subgroup analysis of ARST1321, and detailed the implications of these findings.

R. Lor Randall, MD, FACS, discusses findings from a study of one-stage synovectomy in patients with diffuse-type tenosynovial giant cell tumor of the knee.

R. Lor Randall, MD, FACS, discusses the standard treatment approach for patients with diffuse-type tenosynovial giant cell tumor, the results of the retrospective cohort study evaluating 1- vs 2-stage synovectomies in these patients, and the importance of having additional treatment options for this patient population.

A full dose of lurbinectedin plus a low dose of doxorubicin was clinically active and tolerable in patients with advanced or metastatic soft tissue sarcomas, supporting its continued investigation in those with leiomyosarcoma.

Brian A. Van Tine, MD, PhD, discusses the rationale for launching a randomized phase 2 trial investigating cabozantinib combined with nivolumab and ipilimumab vs cabozantinib alone in metastatic soft tissue sarcoma and discusses key findings from the trial.

Bernd Kasper, MD, PhD, professor, Universitätsmedizin Mannheim, Mannheim, discusses the effect of nirogacestat on tumor volume and T2 hyperintensity in patients with desmoid tumors.