P2, N=52, Active, not recruiting, Memorial Sloan Kettering Cancer Center | Trial completion date: Mar 2026 --> Mar 2027 | Trial primary completion date: Mar 2026 --> Mar 2027
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Trial completion date • Trial primary completion date
Mechanistically, CBX3 binds directly to and SHH and prevents its ubiquitin-mediated degradation, thereby stabilizing the protein. CBX3-SHH subsequently suppresses mitochondrial oxidative metabolism, which in turn inhibits ferroptosis and facilitates EMT - ultimately promoting SS aggressiveness.
Risk factors may include male sex, older age, and previous tarsal trauma. A standardized IHC panel combined with a diagnostic algorithm improved histotyping accuracy and should be adopted in clinical practice.
ATRX mutational status may serve as a potential biomarker for prognosis and therapeutic stratification. Future clinical trials investigating epigenetic therapies could offer novel treatment strategies for ATRX-deficient sarcomas.
Here we provide the basis for further development of trabectedin/irinotecan for patients with ES by the international cooperative groups. ClinicalTrials.gov: NCT04067115 .
The biphasic EPCAM+ SyS has the highest levels of differentiation and lowest levels of the core oncogenic program. Our data complements the traditional classification of SyS and suggests that the monophasic mesenchymal SyS might consist of two molecular subtypes.