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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.
 
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.

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.

Professional guidelines

PubMed

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
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
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

Recent clinical studies

Etiology

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
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
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
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
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

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
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
Olofson AM, Linos K
Arch Pathol Lab Med 2017 Feb;141(2):301-304. doi: 10.5858/arpa.2016-0101-RS. PMID: 28134577
Richards NQ, Kofler JK, Chu CT, Stefko ST
Retin Cases Brief Rep 2017 Fall;11(4):302-305. doi: 10.1097/ICB.0000000000000348. PMID: 27315324
Fisher C
Ann Diagn Pathol 1998 Dec;2(6):401-21. doi: 10.1016/s1092-9134(98)80042-7. PMID: 9930576

Therapy

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
Somaiah N, Conley AP, Parra ER, Lin H, Amini B, Solis Soto L, Salazar R, Barreto C, Chen H, Gite S, Haymaker C, Nassif EF, Bernatchez C, Mitra A, Livingston JA, Ravi V, Araujo DM, Benjamin R, Patel S, Zarzour MA, Sabir S, Lazar AJ, Wang WL, Daw NC, Zhou X, Roland CL, Cooper ZA, Rodriguez-Canales J, Futreal A, Soria JC, Wistuba II, Hwu P
Lancet Oncol 2022 Sep;23(9):1156-1166. Epub 2022 Aug 4 doi: 10.1016/S1470-2045(22)00392-8. PMID: 35934010
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
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
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

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
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
Ferrari A, Dirksen U, Bielack S
Prog Tumor Res 2016;43:128-41. Epub 2016 Sep 5 doi: 10.1159/000447083. PMID: 27595362
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
Wright PH, Sim FH, Soule EH, Taylor WF
J Bone Joint Surg Am 1982 Jan;64(1):112-22. PMID: 6274877

Clinical prediction guides

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
Moreno Tellez C, Leyfman Y, D'Angelo SP, Wilky BA, Dufresne A
Surg Oncol Clin N Am 2022 Jul;31(3):381-397. doi: 10.1016/j.soc.2022.03.004. PMID: 35715140
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
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
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

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
Blas L, Roberti J
Curr Urol Rep 2021 Mar 11;22(4):25. doi: 10.1007/s11934-021-01038-w. PMID: 33704587
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
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
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

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