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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
Datta NK, Das KP, Aish PK, Datta M, Banik SK, Sen SK, Chowdhury RM
Mymensingh Med J 2023 Jan;32(1):135-143. PMID: 36594313
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

Singh A, Thorpe S, Keegan T, Maguire F, Malogolowkin M, Abrahão R, Spunt S, Carr-Asher J, Alvarez E
J Adolesc Young Adult Oncol 2023 Oct;12(5):634-643. Epub 2023 Apr 27 doi: 10.1089/jayao.2022.0143. PMID: 37104039Free PMC Article
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
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
Haas RL, Floot BGJ, Scholten AN, van der Graaf WTA, van Houdt W, Schrage Y, van de Ven M, Bovée JVMG, van Coevorden F, Vens C
Radiat Res 2021 Jul 1;196(1):23-30. doi: 10.1667/RADE-20-00226.1. PMID: 33914890
Gladish GW, Sabloff BM, Munden RF, Truong MT, Erasmus JJ, Chasen MH
Radiographics 2002 May-Jun;22(3):621-37. doi: 10.1148/radiographics.22.3.g02ma17621. PMID: 12006691

Diagnosis

Yang XY, He X, Zhao Y
Comput Intell Neurosci 2022;2022:4748628. Epub 2022 Jun 10 doi: 10.1155/2022/4748628. PMID: 35720922Free PMC Article
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
Fisher C
Ann Diagn Pathol 1998 Dec;2(6):401-21. doi: 10.1016/s1092-9134(98)80042-7. PMID: 9930576

Therapy

Li CC, Chen TW
Curr Opin Oncol 2023 Jul 1;35(4):309-314. Epub 2023 May 3 doi: 10.1097/CCO.0000000000000955. PMID: 37222206
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
Gronchi A, Palmerini E, Quagliuolo V, Martin Broto J, Lopez Pousa A, Grignani G, Brunello A, Blay JY, Tendero O, Diaz Beveridge R, Ferraresi V, Lugowska I, Merlo DF, Fontana V, Marchesi E, Braglia L, Donati DM, Palassini E, Bianchi G, Marrari A, Morosi C, Stacchiotti S, Bagué S, Coindre JM, Dei Tos AP, Picci P, Bruzzi P, Casali PG
J Clin Oncol 2020 Jul 1;38(19):2178-2186. Epub 2020 May 18 doi: 10.1200/JCO.19.03289. PMID: 32421444
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

Fice M, Almajnooni A, Gusho C, Chapman R, Mallikarjunappa S, Batus M, Gitelis S, Colman M, Miller I, Blank A
J Surg Oncol 2022 Jun;125(8):1301-1311. Epub 2022 Mar 6 doi: 10.1002/jso.26838. PMID: 35249228
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
Zhang G, Xiao B, Huang H, Zhang Y, Zhang X, Zhang J, Wang Y
Eur J Surg Oncol 2019 Dec;45(12):2379-2385. Epub 2019 Aug 16 doi: 10.1016/j.ejso.2019.08.015. PMID: 31445769
Paderno A, Gronchi A, Piazza C
Curr Opin Otolaryngol Head Neck Surg 2018 Apr;26(2):94-101. doi: 10.1097/MOO.0000000000000440. PMID: 29329114
Ud Din N, Hopkins A, Memon A, Ahmad Z, Ahmed R
Indian J Pathol Microbiol 2015 Jan-Mar;58(1):55-8. doi: 10.4103/0377-4929.151188. PMID: 25673593

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
Stacchiotti S, Van Tine BA
J Clin Oncol 2018 Jan 10;36(2):180-187. Epub 2017 Dec 8 doi: 10.1200/JCO.2017.75.1941. PMID: 29220290

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
Haider AS, Palmisciano P, Sagoo NS, Bin Alamer O, El Ahmadieh TY, Pan E, Garzon-Muvdi T
Clin Neurol Neurosurg 2022 Mar;214:107127. Epub 2022 Jan 12 doi: 10.1016/j.clineuro.2022.107127. PMID: 35151057
Blas L, Roberti J
Curr Urol Rep 2021 Mar 11;22(4):25. doi: 10.1007/s11934-021-01038-w. PMID: 33704587
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
Yao X, Ghert M, Dickson BC, Popovic S, Purgina BM, Verma S, Werier J, Kandel RA
Cancer Treat Rev 2020 Apr;85:101987. Epub 2020 Feb 14 doi: 10.1016/j.ctrv.2020.101987. PMID: 32092619

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
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      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

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