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Synovial sarcoma

MedGen UID:
21050
•Concept ID:
C0039101
•
Neoplastic Process
Synonyms: Sarcoma, Synovial; Sarcomas, Synovial; Synovial Sarcoma; Synovial Sarcomas; Synovioma; Synoviomas
SNOMED CT: Synovial sarcoma (63211008); Synovioma, malignant (63211008); Synovioma (63211008); Synovial sarcoma (302851001); Malignant synovioma (302851001)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Gene (location): SSX2 (Xp11.22)
 
HPO: HP:0012570
Monarch Initiative: MONDO:0010434
OMIM®: 300813
Orphanet: ORPHA3273

Definition

Synovial sarcomas, which represent approximately 10% of all soft tissue sarcomas, are aggressive spindle cell sarcomas containing in some cases areas of epithelial differentiation. They consistently show a specific t(X;18)(p11.2;q11.2), which usually represents either of 2 gene fusions, SYT (600192)-SSX1 (312820) or SYT-SSX2 (300192), encoding putative transcriptional proteins differing at 13 amino acid positions (summary by Ladanyi et al., 2002). Synovial sarcoma, according to the experience of Enzinger and Weiss (1983), is the fourth most common type of soft tissue sarcoma. It usually develops in adolescents and young adults, is more common in males than in females, and has no racial predilection. [from OMIM]

Clinical features

From HPO
Synovial sarcoma
MedGen UID:
21050
•Concept ID:
C0039101
•
Neoplastic Process
Synovial sarcomas, which represent approximately 10% of all soft tissue sarcomas, are aggressive spindle cell sarcomas containing in some cases areas of epithelial differentiation. They consistently show a specific t(X;18)(p11.2;q11.2), which usually represents either of 2 gene fusions, SYT (600192)-SSX1 (312820) or SYT-SSX2 (300192), encoding putative transcriptional proteins differing at 13 amino acid positions (summary by Ladanyi et al., 2002). Synovial sarcoma, according to the experience of Enzinger and Weiss (1983), is the fourth most common type of soft tissue sarcoma. It usually develops in adolescents and young adults, is more common in males than in females, and has no racial predilection.
See: Feature record | Search on this feature

Conditions with this feature

Synovial sarcoma
MedGen UID:
21050
•Concept ID:
C0039101
•
Neoplastic Process
Synovial sarcomas, which represent approximately 10% of all soft tissue sarcomas, are aggressive spindle cell sarcomas containing in some cases areas of epithelial differentiation. They consistently show a specific t(X;18)(p11.2;q11.2), which usually represents either of 2 gene fusions, SYT (600192)-SSX1 (312820) or SYT-SSX2 (300192), encoding putative transcriptional proteins differing at 13 amino acid positions (summary by Ladanyi et al., 2002). Synovial sarcoma, according to the experience of Enzinger and Weiss (1983), is the fourth most common type of soft tissue sarcoma. It usually develops in adolescents and young adults, is more common in males than in females, and has no racial predilection.
See: Condition Record
Synovial sarcoma

Professional guidelines

PubMed

Relapsed Synovial Sarcoma: Treatment Options.
Weiss MC, Van Tine BA
Curr Treat Options Oncol 2023 Mar;24(3):229-239. Epub 2023 Mar 3 doi: 10.1007/s11864-023-01056-5. PMID: 36867389
Management of Synovial Sarcoma and Myxoid Liposarcoma.
Hindi N, Haas RL
Surg Oncol Clin N Am 2022 Jul;31(3):547-558. doi: 10.1016/j.soc.2022.03.012. PMID: 35715149
The many faces of solitary fibrous tumor; diversity of histological features, differential diagnosis and role of molecular studies and surrogate markers in avoiding misdiagnosis and predicting the behavior.
Tariq MU, Din NU, Abdul-Ghafar J, Park YK
Diagn Pathol 2021 Apr 20;16(1):32. doi: 10.1186/s13000-021-01095-2. PMID: 33879215Free PMC Article

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

Recent clinical studies

Etiology

Soft-tissue sarcoma in adolescents and young adults.
Kunisada T, Nakata E, Fujiwara T, Hosono A, Takihira S, Kondo H, Ozaki T
Int J Clin Oncol 2023 Jan;28(1):1-11. Epub 2022 Jan 27 doi: 10.1007/s10147-022-02119-7. PMID: 35084598
Management of Synovial Sarcoma and Myxoid Liposarcoma.
Hindi N, Haas RL
Surg Oncol Clin N Am 2022 Jul;31(3):547-558. doi: 10.1016/j.soc.2022.03.012. PMID: 35715149
Metastasectomy in synovial sarcoma: A systematic review and meta-analysis.
Wang Y, Delisle M, Smith D, Alshamsan B, Srikanthan A
Eur J Surg Oncol 2022 Sep;48(9):1901-1910. Epub 2022 Jun 2 doi: 10.1016/j.ejso.2022.05.022. PMID: 35672231
Periarticular calcifications.
Yan YY, Dous YNB, Ouellette HA, Munk PL, Murray N, Mallinson PI, Sheikh MA
Skeletal Radiol 2022 Mar;51(3):451-475. Epub 2021 Jun 21 doi: 10.1007/s00256-021-03842-4. PMID: 34155550
Sarcoma Stem Cell Heterogeneity.
Hatina J, Kripnerova M, Houfkova K, Pesta M, Kuncova J, Sana J, Slaby O, Rodríguez R
Adv Exp Med Biol 2019;1123:95-118. doi: 10.1007/978-3-030-11096-3_7. PMID: 31016597

Diagnosis

A review on pulmonary and mediastinal synovial sarcoma.
Dewi KP, Dewi IP, Iswanto I, Wulandari L
J Basic Clin Physiol Pharmacol 2023 Mar 1;34(2):169-175. Epub 2023 Feb 20 doi: 10.1515/jbcpp-2022-0286. PMID: 36800987
Management of Synovial Sarcoma and Myxoid Liposarcoma.
Hindi N, Haas RL
Surg Oncol Clin N Am 2022 Jul;31(3):547-558. doi: 10.1016/j.soc.2022.03.012. PMID: 35715149
Synovial Sarcoma: A Clinical Review.
Gazendam AM, Popovic S, Munir S, Parasu N, Wilson D, Ghert M
Curr Oncol 2021 May 19;28(3):1909-1920. doi: 10.3390/curroncol28030177. PMID: 34069748Free PMC Article
Primary Intraprostatic Synovial Sarcoma.
Olofson AM, Linos K
Arch Pathol Lab Med 2017 Feb;141(2):301-304. doi: 10.5858/arpa.2016-0101-RS. PMID: 28134577
Synovial sarcoma.
Fisher C
Ann Diagn Pathol 1998 Dec;2(6):401-21. doi: 10.1016/s1092-9134(98)80042-7. PMID: 9930576

Therapy

Afamitresgene autoleucel for advanced synovial sarcoma and myxoid round cell liposarcoma (SPEARHEAD-1): an international, open-label, phase 2 trial.
D'Angelo SP, Araujo DM, Abdul Razak AR, Agulnik M, Attia S, Blay JY, Carrasco Garcia I, Charlson JA, Choy E, Demetri GD, Druta M, Forcade E, Ganjoo KN, Glod J, Keedy VL, Le Cesne A, Liebner DA, Moreno V, Pollack SM, Schuetze SM, Schwartz GK, Strauss SJ, Tap WD, Thistlethwaite F, Valverde Morales CM, Wagner MJ, Wilky BA, McAlpine C, Hudson L, Navenot JM, Wang T, Bai J, Rafail S, Wang R, Sun A, Fernandes L, Van Winkle E, Elefant E, Lunt C, Norry E, Williams D, Biswas S, Van Tine BA
Lancet 2024 Apr 13;403(10435):1460-1471. Epub 2024 Mar 27 doi: 10.1016/S0140-6736(24)00319-2. PMID: 38554725Free PMC Article
Autologous T cell therapy for MAGE-A4(+) solid cancers in HLA-A*02(+) patients: a phase 1 trial.
Hong DS, Van Tine BA, Biswas S, McAlpine C, Johnson ML, Olszanski AJ, Clarke JM, Araujo D, Blumenschein GR Jr, Kebriaei P, Lin Q, Tipping AJ, Sanderson JP, Wang R, Trivedi T, Annareddy T, Bai J, Rafail S, Sun A, Fernandes L, Navenot JM, Bushman FD, Everett JK, Karadeniz D, Broad R, Isabelle M, Naidoo R, Bath N, Betts G, Wolchinsky Z, Batrakou DG, Van Winkle E, Elefant E, Ghobadi A, Cashen A, Grand'Maison A, McCarthy P, Fracasso PM, Norry E, Williams D, Druta M, Liebner DA, Odunsi K, Butler MO
Nat Med 2023 Jan;29(1):104-114. Epub 2023 Jan 9 doi: 10.1038/s41591-022-02128-z. PMID: 36624315Free PMC Article
Opposing immune and genetic mechanisms shape oncogenic programs in synovial sarcoma.
Jerby-Arnon L, Neftel C, Shore ME, Weisman HR, Mathewson ND, McBride MJ, Haas B, Izar B, Volorio A, Boulay G, Cironi L, Richman AR, Broye LC, Gurski JM, Luo CC, Mylvaganam R, Nguyen L, Mei S, Melms JC, Georgescu C, Cohen O, Buendia-Buendia JE, Segerstolpe A, Sud M, Cuoco MS, Labes D, Gritsch S, Zollinger DR, Ortogero N, Beechem JM, Petur Nielsen G, Chebib I, Nguyen-Ngoc T, Montemurro M, Cote GM, Choy E, Letovanec I, Cherix S, Wagle N, Sorger PK, Haynes AB, Mullen JT, Stamenkovic I, Rivera MN, Kadoch C, Wucherpfennig KW, Rozenblatt-Rosen O, Suvà ML, Riggi N, Regev A
Nat Med 2021 Feb;27(2):289-300. Epub 2021 Jan 25 doi: 10.1038/s41591-020-01212-6. PMID: 33495604Free PMC Article
Tazemetostat in advanced epithelioid sarcoma with loss of INI1/SMARCB1: an international, open-label, phase 2 basket study.
Gounder M, Schöffski P, Jones RL, Agulnik M, Cote GM, Villalobos VM, Attia S, Chugh R, Chen TW, Jahan T, Loggers ET, Gupta A, Italiano A, Demetri GD, Ratan R, Davis LE, Mir O, Dileo P, Van Tine BA, Pressey JG, Lingaraj T, Rajarethinam A, Sierra L, Agarwal S, Stacchiotti S
Lancet Oncol 2020 Nov;21(11):1423-1432. Epub 2020 Oct 6 doi: 10.1016/S1470-2045(20)30451-4. PMID: 33035459
Pembrolizumab in advanced soft-tissue sarcoma and bone sarcoma (SARC028): a multicentre, two-cohort, single-arm, open-label, phase 2 trial.
Tawbi HA, Burgess M, Bolejack V, Van Tine BA, Schuetze SM, Hu J, D'Angelo S, Attia S, Riedel RF, Priebat DA, Movva S, Davis LE, Okuno SH, Reed DR, Crowley J, Butterfield LH, Salazar R, Rodriguez-Canales J, Lazar AJ, Wistuba II, Baker LH, Maki RG, Reinke D, Patel S
Lancet Oncol 2017 Nov;18(11):1493-1501. Epub 2017 Oct 4 doi: 10.1016/S1470-2045(17)30624-1. PMID: 28988646Free PMC Article

Prognosis

The Biology of Synovial Sarcoma: State-of-the-Art and Future Perspectives.
Fiore M, Sambri A, Spinnato P, Zucchini R, Giannini C, Caldari E, Pirini MG, De Paolis M
Curr Treat Options Oncol 2021 Oct 23;22(12):109. doi: 10.1007/s11864-021-00914-4. PMID: 34687366Free PMC Article
Synovial Sarcoma: A Clinical Review.
Gazendam AM, Popovic S, Munir S, Parasu N, Wilson D, Ghert M
Curr Oncol 2021 May 19;28(3):1909-1920. doi: 10.3390/curroncol28030177. PMID: 34069748Free PMC Article
Single-Institution Experience of Synovial Sarcoma.
Farkas A, Lirette ST, Al Hmada Y, Collier AB, Barr J, Vijayakumar S, Vijayakumar V
South Med J 2020 Jan;113(1):16-19. doi: 10.14423/SMJ.0000000000001054. PMID: 31897493
Synovial sarcoma: defining features and diagnostic evolution.
Thway K, Fisher C
Ann Diagn Pathol 2014 Dec;18(6):369-80. Epub 2014 Oct 13 doi: 10.1016/j.anndiagpath.2014.09.002. PMID: 25438927
Synovial sarcoma in children and adolescents.
Kerouanton A, Jimenez I, Cellier C, Laurence V, Helfre S, Pannier S, Mary P, Freneaux P, Orbach D
J Pediatr Hematol Oncol 2014 May;36(4):257-62. doi: 10.1097/MPH.0000000000000154. PMID: 24633301

Clinical prediction guides

Sarcoma care in the era of precision medicine.
Wallander K, Öfverholm I, Boye K, Tsagkozis P, Papakonstantinou A, Lin Y, Haglund de Flon F
J Intern Med 2023 Dec;294(6):690-707. Epub 2023 Sep 7 doi: 10.1111/joim.13717. PMID: 37643281
Popliteal Fossa Sarcomas.
Erdogan O, Çeli K A, Yildirim ANT, Tekçe E, Altun G, Demi Röz S, Güler Y, Ozkan K, Gurkan V
Acta Chir Orthop Traumatol Cech 2023;90(3):181-187. PMID: 37395425
Synovial Sarcoma: A Clinical Review.
Gazendam AM, Popovic S, Munir S, Parasu N, Wilson D, Ghert M
Curr Oncol 2021 May 19;28(3):1909-1920. doi: 10.3390/curroncol28030177. PMID: 34069748Free PMC Article
The many faces of solitary fibrous tumor; diversity of histological features, differential diagnosis and role of molecular studies and surrogate markers in avoiding misdiagnosis and predicting the behavior.
Tariq MU, Din NU, Abdul-Ghafar J, Park YK
Diagn Pathol 2021 Apr 20;16(1):32. doi: 10.1186/s13000-021-01095-2. PMID: 33879215Free PMC Article
BCOR-CCNB3 Fusion Positive Sarcomas: A Clinicopathologic and Molecular Analysis of 36 Cases With Comparison to Morphologic Spectrum and Clinical Behavior of Other Round Cell Sarcomas.
Kao YC, Owosho AA, Sung YS, Zhang L, Fujisawa Y, Lee JC, Wexler L, Argani P, Swanson D, Dickson BC, Fletcher CDM, Antonescu CR
Am J Surg Pathol 2018 May;42(5):604-615. doi: 10.1097/PAS.0000000000000965. PMID: 29300189Free PMC Article

Recent systematic reviews

Metastasectomy in synovial sarcoma: A systematic review and meta-analysis.
Wang Y, Delisle M, Smith D, Alshamsan B, Srikanthan A
Eur J Surg Oncol 2022 Sep;48(9):1901-1910. Epub 2022 Jun 2 doi: 10.1016/j.ejso.2022.05.022. PMID: 35672231
Primary Renal Synovial Sarcoma and Clinical and Pathological Findings: a Systematic Review.
Blas L, Roberti J
Curr Urol Rep 2021 Mar 11;22(4):25. doi: 10.1007/s11934-021-01038-w. PMID: 33704587
Laryngeal Synovial Sarcoma: A Systematic Review of the Last 40 Years of Reported Cases.
Shein G, Sandhu G, Potter A, Loo C, Jacobson I, Anazodo A
Ear Nose Throat J 2021 Feb;100(2):NP93-NP104. Epub 2019 Jul 16 doi: 10.1177/0145561319850697. PMID: 31309846
Pediatric laryngeal sarcoma: Systematic review and pooled analysis.
Mur TA, Pellegrini WR, Jaleel Z, Edwards HA, Levi JR
Int J Pediatr Otorhinolaryngol 2020 Dec;139:110471. Epub 2020 Oct 23 doi: 10.1016/j.ijporl.2020.110471. PMID: 33120103
Synovial Sarcoma of the Nerve-Clinical and Pathological Features: Case Series and Systematic Review.
Burks SS, Puffer RC, Cajigas I, Valdivia D, Rosenberg AE, Spinner RJ, Levi AD
Neurosurgery 2019 Dec 1;85(6):E975-E991. doi: 10.1093/neuros/nyz321. PMID: 31435657Free PMC Article

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

    Reviews

    Related information

    • Gene
      Related information in NCBI Gene
    • GTR
      Related information in GTR
    • GTR(Clinical)
      Clinical tests in GTR
    • MeSH
      Related Medical Subject Headings
    • OMIM
      Related records in OMIM
    • OMIM(Genes)
      OMIM records containing genes associated with phenotypes registered in MedGen
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

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