Synovial Sarcoma (SYNS)

Overview

Synovial sarcoma is a malignant soft-tissue sarcoma defined by the SS18–SSX1/2/4 chromosomal translocation t(X;18), classified under Soft Tissue Sarcoma in OncoTree (parent: SOFT_TISSUE). It predominates in adolescents and young adults (AYA), most commonly in the extremities, and accounts for ~5–10% of all soft-tissue sarcomas.

Cohorts in the corpus

• sarc_mskcc: 24 SYNS patients (11.6% of 207 high-grade STS), profiled by targeted resequencing (722 genes), 250K SNP-array SCNA, LOH, and expression arrays. PMID:20601955
• sarcoma_ucla_2024: represented in the 24-subtype UCLA sarcoma PDTO biobank (194 specimens from 126 patients, Feb 2018–May 2022). PMID:39305899

Recurrent alterations

• SS18–SSX1/SSX2/SSX4 fusion (canonical driver, t(X;18)(p11.2;q11.2)): not directly genotyped in this study.
• Relatively normal karyotype: SYNS clusters with MRLS and GIST as low-complexity subtypes by copy-number analysis in the seven-subtype cohort. PMID:20601955

Subtypes

• Biphasic synovial sarcoma (epithelial + spindle-cell components)
• Monophasic synovial sarcoma (spindle-cell predominant)
• Poorly differentiated synovial sarcoma

Therapeutic landscape

• Patient-derived tumor organoid (PDTO) drug screening was applied across all 24 sarcoma subtypes in the UCLA cohort, including SYNS. Pediatric and AYA sarcoma PDTOs (the predominant age group in SYNS) showed less resistance to cediranib compared to adult PDTOs (p=0.013 vs AYA). PMID:39305899
• Across the full 24-subtype cohort, 59% of samples had ≥1 FDA-approved or NCCN-recommended top-five regimen. PMID:39305899

Sources

• PMID:20601955 — Barretina et al. Nature 2010. Integrative genomic analysis of 207 high-grade soft tissue sarcomas across seven subtypes (MSKCC Sarcoma Genome Project).
• PMID:39305899 — Al Shihabi et al. Cell Stem Cell 2024. UCLA sarcoma PDTO drug-sensitivity landscape.

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