Synovial Sarcoma

Resection subsequently revealed a biphasic morphology, and molecular testing confirmed the presence of an SS18::SSX fusion, confirming the diagnosis of synovial sarcoma. This case highlights a potential diagnostic pitfall in dermatopathology and stresses the importance of confirmatory molecular testing in cases where both sarcoma and carcinoma are included in the differential diagnosis.

The patient underwent wide surgical excision with negative margins and recovered uneventfully. This case emphasizes the importance of maintaining synovial sarcoma in the differential diagnosis of deep thigh masses in older adults, where imaging and molecular confirmation are essential to avoid misdiagnosis and treatment delay.

Talazoparib combined with ATR inhibitor possesses potential application as a therapeutic option for SS.

P1, N=25, Recruiting, Baylor College of Medicine | Trial completion date: Dec 2040 --> Jun 2043 | Trial primary completion date: Dec 2027 --> Jun 2028

This constituted only the second approval of a cell therapy in a solid tumor following lifileucel in melanoma and demonstrated the potential of cell therapies in sarcomas...However, the broader application of these therapies is hindered by the lack of targetable sarcoma-restricted immunogenic epitopes, spatiotemporal intratumoral heterogeneity, and a profoundly immunosuppressive tumor microenvironment that impedes effector-cell trafficking, expansion and persistence. While cell therapies hold promise for integration into precision medicine approaches for sarcomas, their successful implementation will require careful evaluation of clinical feasibility, logistical considerations and cost-effectiveness to optimize patient outcomes.

Our findings suggest that SS is a glutamine-dependent malignancy and validate ASCT2 as a promising therapeutic target. The ASCT2 inhibitor V9302 demonstrated therapeutic efficacy both in vitro and in vivo, supporting its potential as a therapeutic agent for SS.

P3, N=5, Recruiting, Takara Bio Inc. | Not yet recruiting --> Recruiting

Renal sarcomas presenting in children and adolescents comprise a heterogeneous disease category with unique patient, clinical, and molecular characteristics that complicate standardizing therapeutic strategies beyond CCSK and EWS. None.

However, challenges remain regarding HLA restriction, tumor microenvironment resistance, manufacturing delays and cost. Future efforts should focus on broadening applicability, optimizing combinations, and ensuring equitable access.

Pediatric STS are rare and biologically heterogeneous. Genomic advances have refined risk stratification and uncovered therapeutic targets; further progress relies on international collaboration and trials.

Published single-cell RNA sequencing data were analyzed to validate our finding that BRD9 contributes to SySa EMT regulation. Our study provides novel insights into the multilayered regulation of key EMT players by SS18::SSX and BRD9 in SySa, thereby defining tumor phenotype and (potentially) prognosis.

Disruption of Arid1a or Arid1b (both CBAF-specific) retains synovial sarcoma character, while Smarcb1 (PBAF- and CBAF-specific) or Pbrm1 (PBAF-specific) disruption does not, although all accelerate SS18::SSX-driven tumorigenesis in mice. Thus, the synovial sarcomagenesis mechanism involves SS18::SSX reprogramming transcription positively through GBAF redistribution to activate polycomb-targeted developmental genes, and negatively by loss of normal CBAF localization and function.