Atlas One · Host Biology · Scale 07 — System

Synovial Sarcoma

Synovial sarcoma is defined by SS18-SSX1/SSX2 fusion (t(X;18)) → SMARCB1 displacement from BAF → EZH2 dependency; ~800/year USA; biphasic/monophasic histology; TLE1 IHC positive; ifosfamide-based chemotherapy; tazemetostat (SARC057 ORR ~22%) and trabectedin active.

Also known as: synovial sarcoma, SS18-SSX sarcoma, biphasic synovial sarcoma, monophasic synovial sarcoma, t(X;18) sarcoma, TLE1-positive sarcoma, translocation sarcoma SYT-SSX, synovial cell sarcoma

Scale 07 — SystemScale
draftStatus
2026-06-06Last Reviewed
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synovial-sarcoma

Synovial Sarcoma

Synovial sarcoma is defined by SS18-SSX1/SSX2 fusion (t(X;18)) → SMARCB1 displacement from BAF → EZH2 dependency; ~800/year USA; biphasic/monophasic histology; TLE1 IHC positive; ifosfamide-based chemotherapy; tazemetostat (SARC057 ORR ~22%) and trabectedin active.

Entry Metadata

FieldValue
IDsynovial-sarcoma
NameSynovial Sarcoma
Statusdraft
Last reviewed2026-06-06
Atlas01-human
Scale07-system

Cross-Atlas Connections

connects-toSs18 connects-toEzh2 connects-toSmarcb1 connects-toCdkn2A connects-toVegf connects-toPd 1 connects-toWnt Beta Catenin connects-toAtypical Teratoid Rhabdoid Tumor connects-toMusculoskeletal System connects-toLung

Sources