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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
| Field | Value |
|---|---|
| ID | synovial-sarcoma |
| Name | Synovial Sarcoma |
| Status | draft |
| Last reviewed | 2026-06-06 |
| Atlas | 01-human |
| Scale | 07-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
connects-toSchwannomatosis
connects-toEwing Sarcoma
connects-toFibroblast
connects-toRhabdomyosarcoma
connects-toMpnst
connects-toPhoton
connects-toP53
connects-toTert
connects-toLi Fraumeni Syndrome
connects-toImmune System
connects-toDendritic Cell
connects-toHeart
connects-toT Cytotoxic Cell
connects-toMacrophage
connects-toIgf 1
connects-toArid1A
connects-toRegulatory T Cell
connects-toMtor
connects-toOxygen
connects-toBrain
connects-toAkt
connects-toCalcium
connects-toLiver
connects-toFibrosis
connects-toElectron
connects-toBone Marrow
connects-toKidney
connects-toCollagen
connects-toNeuron
connects-toMagnesium
connects-toAntibody
connects-toNeutrophil
connects-toErythrocyte
connects-toCardiomyocyte
connects-toReproductive System
connects-toHif 1Alpha
connects-toBcl 2
connects-toEgfr
connects-toNatural Killer Cell
connects-toStat3
connects-toVenous Thromboembolism
connects-toSepsis
connects-toCkd
connects-toAml
connects-toAnemia Of Chronic Disease
connects-toHeart Failure
connects-toAspergillus Fumigatus
connects-toMajor Depressive Disorder
connects-toWound Healing
connects-toNeuropathic Pain
connects-toGeneralized Anxiety Disorder
connects-toRespiratory System
connects-toVaricella Zoster Virus
connects-toDigestive System
connects-toLymphatic System
connects-toRenal System
connects-toCardiovascular System
connects-toTargeted Therapy
connects-toNervous System
connects-toIntegumentary System
connects-toCancer Chemotherapy
connects-toCar T
connects-toCheckpoint Inhibitors
connects-toAlveolus
connects-toGist
connects-toOvarian Clear Cell Carcinoma
connects-toDesmoid Tumor
connects-toOsteosarcoma
connects-toGerminal Center
connects-toMelanoma
connects-toCortical Bone
connects-toPeripheral Nerve
connects-toMyc
connects-toCyclin D1
connects-toYap1
connects-toPdgf
connects-toFgfr
connects-toMet
connects-toNotch
connects-toTgf Beta
connects-toCxcl12
connects-toCdk4 6
connects-toCcl2
connects-toCaspase 3
connects-toPik3Ca
connects-toE2F1
connects-toSrc Kinase
connects-toErk1 2
connects-toPten
connects-toMdm2
connects-toRb1
connects-toKras
connects-toJak1 2
connects-toGalectin 3
connects-toSmad4
connects-toCgas Sting
connects-toStat1
connects-toFoxo
connects-toPerforin
connects-toGsk 3B
connects-toS100A8 A9
connects-toDnmt3A
connects-toAutophagy
connects-toAmpk
connects-toIl 6
connects-toCcr5
connects-toIl 1B
connects-toCdkn1A
connects-toIl 33
connects-toIl 17A
connects-toComplement C3
connects-toMhc Class Ii
connects-toAxl Receptor
connects-toAngiopoietin
connects-toIl 2
connects-toTroponin Complex
connects-toHemoglobin
connects-toIl 10
connects-toXanthine Oxidase
connects-toNitric Oxide
connects-toIl 4
connects-toIron
connects-toProton
connects-toIl 13
connects-toMacrophage
connects-toCortical Bone
connects-toLeptin
connects-toAdiponectin
connects-toResistin
connects-toType I Interferon
connects-toIfn Gamma
connects-toIl 12
connects-toIl 5
connects-toIl 23
connects-toIge
connects-toMast Cell
connects-toT Helper Cell
connects-toC5Ar1
connects-toB Cell
connects-toComplement C5
connects-toFactor H
connects-toTslp
connects-toErythropoietin
connects-toPeriostin
connects-toBradykinin
connects-toC1 Esterase Inhibitor
connects-toHistamine
connects-toMelatonin
connects-toTestosterone
connects-toSerotonin
connects-toOxytocin
connects-toVasopressin
connects-toSelenium
connects-toIodine
connects-toSodium
connects-toCopper
connects-toZinc
connects-toPotassium
connects-toPhosphorus
connects-toCarbon
connects-toChloride
connects-toHydrogen
connects-toNitrogen
connects-toSulfur
connects-toGlp 1
connects-toAngiotensin Ii
connects-toRankl
connects-toFibronectin
connects-toActivin A
connects-toCgrp
connects-toCalcitonin
connects-toSubstance P
connects-toInsulin Receptor
connects-toAldosterone
connects-toAndrogen Receptor
connects-toNorepinephrine
connects-toAdrenomedullin
connects-toBdnf
connects-toOsteopontin
connects-toEpinephrine
connects-toRenin
connects-toMyostatin
connects-toCortisol
Sources
- Kadoch C, Crabtree GR. Reversible disruption of mSWI/SNF (BAF) complexes by the SS18-SSX oncogenic fusion in synovial sarcoma. Cell. 2013;153(1):71-85. · PubMed 23540691
- Kawai A, Araki N, Sugiura H, et al. Trabectedin monotherapy after standard chemotherapy versus best supportive care in patients with advanced, translocation-related sarcoma: a randomised, open-label, phase 2 study. Lancet Oncol. 2015;16(4):406-416. · PubMed 25795407