Synovial Sarcoma

P1, N=8, Not yet recruiting, Northwestern University

P1, N=22, Active, not recruiting, University Health Network, Toronto | Trial completion date: Sep 2025 --> Nov 2026 | Trial primary completion date: Sep 2025 --> Nov 2026

Adult and embryonic MSCs exhibited overlapping H2AK119ub and H3K4me3/H3K27me3 (bivalent) histone marks, while SS18::SSX-mediated transformation culminated in the widespread loss of H3K27me3 at these genes and their consequent transcription. Collectively, these studies define a rare MSC context, conducive for SS18::SSX-mediated transformation, and demonstrate that SyS tumorigenesis involves the induction and maintenance of an embryonic-like MSC phenotype.

Further, the advancement of CFT8634 as a monotherapy was inhibited by the emergence of cardiac toxicities. Nevertheless, this first study of an orally bioavailable BRD9 degrader recapitulates preclinical pharmacokinetic and pharmacodynamic observations in a clinical trial setting.

P1, N=44, Active, not recruiting, Seattle Children's Hospital | Recruiting --> Active, not recruiting | Trial primary completion date: Jun 2025 --> Jun 2040

This case highlighted the importance of a multidisciplinary approach in diagnosing and managing a rare head and neck SS. Furthermore, this is the first reported case from Pakistan, emphasizing the need for awareness in atypical locations.

TAK-981 synergizes with cytotoxic chemotherapy through increased DNA damage, leading to tumor regression. Targeting the SUMOylation pathway in SS restores cBAF complexes and blocks the SS18::SSX transcriptome, identifying an unappreciated role of SUMOylation in SS and a subsequent therapeutic vulnerability.

P1/2, N=58, Completed, Epizyme, Inc. | Active, not recruiting --> Completed

The tumor cells exhibited positivity for CKAE1/AE3, TLE-1, and CD99, while displaying negativity for S100. This report contributes to the limited extant regarding this rare malignancy, shedding light on their clinical and diagnostic characteristics.

SOX11 is moderately sensitive but highly specific for IFS/CMN with ETV6::NTRK3 fusion. In the correct context, SOX11 shows utility as a screening adjunct for focused molecular testing.

Our findings demonstrate that hypoxia promotes SS metastasis through activation of HIF-1α and related pathways. Fusion-positive SS cells appear particularly responsive to hypoxic cues, suggesting that targeting hypoxia-induced signaling could be a promising strategy to inhibit metastasis in SS. These results provide mechanistic insight into SS progression and support the integration of hypoxia-targeted therapies into future treatment strategies.

Overall, oncogenic SGA correlated with a worse survival in certain TAS types.