Chordoma

P=N/A, N=140, Not yet recruiting, The first affiliated hostipal of nanchang university; The first affiliated hostipal of nanchang university

P2, N=88, Not yet recruiting, Shanghai Proton and Heavy Ion Center; The Clinical Technology Research And Development Center of Shanghai Proton and Heavy Ion Center

P2, N=88, Not yet recruiting, The Clinical Technology Research And Development Center of Shanghai Proton and Heavy Ion Center; The Clinical Technology Research And Development Cent

This study advances understanding of the chordoma immune landscape by integrating spatial, transcriptomic, and TCR data. The findings highlight systemic and local immune dynamics, reveal tumor-specific TCR motifs, and identify potential therapeutic targets. These insights provide a foundation for developing personalized immunotherapies to overcome immune suppression and enhance anti-tumor immunity in chordomas.

Liquid biopsy holds strong potential for transforming bone tumor care. Its clinical adoption depends on further validation through standardized methods and large-scale studies.

P=N/A, N=20, Recruiting, Quironsalud | Trial primary completion date: May 2025 --> May 2027

P1/2, N=46, Recruiting, Memorial Sloan Kettering Cancer Center | N=11 --> 46

We speculated that both TBXT and SMARCE1 might indirectly promote EGFR signalling to drive chordoma cell proliferation and survival, although the direct interaction between TBXT and SMARCE1 is unknown. To our knowledge, this is the first report of a patient with a spinal chordoma after CCM treatment, suggesting that SMARCE1 is a candidate pathological factor in chordoma.

International, multicentric registries are essential for studying rare diseases like primary spine tumors, enabling robust data collection, improved statistical power, and broader applicability of findings across diverse clinical settings. Ongoing prospective data collection through PTRON will further refine evidence-based care for these rare and challenging conditions.

P=N/A, N=100, Recruiting, Italian Sarcoma Group | Trial completion date: Sep 2025 --> Dec 2026 | Trial primary completion date: Sep 2025 --> Dec 2026

This article aims to expand the knowledge about sacrococcygeal chordoma and its pathogenesis. Notably, chordoma can exclusively cause anal swelling, and clinicians should consider differential diagnosis in practice.

Clinical evidence indicates that immune checkpoint inhibitors and targeted vaccines yield limited efficacy as monotherapies, highlighting the immune-excluded phenotype and the scarcity of PD-L1 protein expression as primary obstacles. Future therapeutic breakthroughs will require rational combination strategies, including CAR-T cell therapies targeting novel antigens (e.g., B7-H3) and adoptive T-cell transfer, designed to dismantle stromal barriers and exploit systemic anti-tumor immunity.