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Epithelioid cell synovial sarcoma

MedGen UID:
90796
•Concept ID:
C0334506
•
Neoplastic Process
Synonyms: Epithelial Sarcoma of Synovium; epithelial sarcoma of synovium; Epithelial Sarcoma of the Synovium; epithelial sarcoma of the synovium; Epithelial Synovial Sarcoma; epithelial synovial sarcoma; Epithelioid Cell Sarcoma of Synovium; epithelioid cell sarcoma of synovium; Epithelioid Cell Sarcoma of the Synovium; epithelioid cell sarcoma of the synovium; Epithelioid Cell Synovial Sarcoma; epithelioid cell synovial sarcoma; Epithelioid Synovial Sarcoma; epithelioid synovial sarcoma; Synovial sarcoma - epithelioid cell; Synovial sarcoma, epithelioid cell; synovial sarcoma, epithelioid cell; synovial sarcoma, epithelioid cell (morphologic abnormality)
SNOMED CT: Synovial sarcoma - epithelioid cell (56422000); Synovial sarcoma, epithelioid cell (56422000)
 
Monarch Initiative: MONDO:0003469

Definition

A synovial sarcoma characterized by the presence of an epithelial cell component only. The epithelial cells are arranged in glandular or papillary structures. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEpithelioid cell synovial sarcoma

Professional guidelines

PubMed

Utility of Immunohistochemistry With Antibodies to SS18-SSX Chimeric Proteins and C-Terminus of SSX Protein for Synovial Sarcoma Differential Diagnosis.
Lasota J, Chłopek M, Kaczorowski M, Natálie K, Ryś J, Kopczyński J, Sulaieva O, Michal M, Kruczak A, Harazin-Lechowska A, Szczepaniak M, Koshyk O, Hałoń A, Czapiewski P, Abdullaev Z, Kowalik A, Aldape KD, Michal M, Miettinen M
Am J Surg Pathol 2024 Jan 1;48(1):97-105. Epub 2023 Oct 27 doi: 10.1097/PAS.0000000000002144. PMID: 37899499
Treatment of limb synovial sarcoma with metastasis at presentation.
Guo P, Zhao R, Zhou Y, Shen Y
Medicine (Baltimore) 2020 Jun 5;99(23):e20550. doi: 10.1097/MD.0000000000020550. PMID: 32502019Free PMC Article
Surgical considerations for management of distal extremity soft tissue sarcomas.
Ferguson PC
Curr Opin Oncol 2005 Jul;17(4):366-9. doi: 10.1097/01.cco.0000166651.38417.c8. PMID: 15933470

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
Spindle cell tumors of the pleura and the peritoneum: pathologic diagnosis and updates.
Boyraz B, Hung YP
APMIS 2022 Mar;130(3):140-154. Epub 2022 Jan 11 doi: 10.1111/apm.13203. PMID: 34942046
Epidemiology, incidence, and survival of synovial sarcoma subtypes: SEER database analysis.
Aytekin MN, Öztürk R, Amer K, Yapar A
J Orthop Surg (Hong Kong) 2020 Jan-Apr;28(2):2309499020936009. doi: 10.1177/2309499020936009. PMID: 32618221
The Diagnosis of Pleural Tumors Other Than Mesothelioma.
Attanoos RL, Pugh MR
Arch Pathol Lab Med 2018 Aug;142(8):902-913. doi: 10.5858/arpa.2017-0537-RA. PMID: 30040453
Inhibin-α and synaptophysin immunoreactivity in synovial sarcoma with granular cell features.
Setsu N, Kohashi K, Endo M, Yamamoto H, Ohishi Y, Sueyoshi K, Iwamoto Y, Tsuneyoshi M, Motoi T, Kumagai A, Oda Y
Hum Pathol 2012 Jun;43(6):850-7. Epub 2011 Nov 4 doi: 10.1016/j.humpath.2011.07.012. PMID: 22055401

Diagnosis

Spindle cell tumors of the pleura and the peritoneum: pathologic diagnosis and updates.
Boyraz B, Hung YP
APMIS 2022 Mar;130(3):140-154. Epub 2022 Jan 11 doi: 10.1111/apm.13203. PMID: 34942046
SWI/SNF-deficient thoraco-pulmonary neoplasms.
Sesboue C, Le Loarer F
Semin Diagn Pathol 2021 May;38(3):183-194. Epub 2021 Jan 12 doi: 10.1053/j.semdp.2020.12.002. PMID: 33451916
The epigenomics of sarcoma.
Nacev BA, Jones KB, Intlekofer AM, Yu JSE, Allis CD, Tap WD, Ladanyi M, Nielsen TO
Nat Rev Cancer 2020 Oct;20(10):608-623. Epub 2020 Aug 11 doi: 10.1038/s41568-020-0288-4. PMID: 32782366Free PMC Article
Ewing sarcoma and Ewing-like tumors.
Sbaraglia M, Righi A, Gambarotti M, Dei Tos AP
Virchows Arch 2020 Jan;476(1):109-119. Epub 2019 Dec 4 doi: 10.1007/s00428-019-02720-8. PMID: 31802230
The Diagnosis of Pleural Tumors Other Than Mesothelioma.
Attanoos RL, Pugh MR
Arch Pathol Lab Med 2018 Aug;142(8):902-913. doi: 10.5858/arpa.2017-0537-RA. PMID: 30040453

Therapy

New targeted treatments for advanced sarcomas.
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
Nanoparticle albumin-bound paclitaxel and PD-1 inhibitor (sintilimab) combination therapy for soft tissue sarcoma: a retrospective study.
Tian Z, Dong S, Yang Y, Gao S, Yang Y, Yang J, Zhang P, Wang X, Yao W
BMC Cancer 2022 Jan 12;22(1):56. doi: 10.1186/s12885-022-09176-1. PMID: 35022029Free PMC Article
TLE1 expression fails to distinguish between synovial sarcoma, atypical fibroxanthoma, and dermatofibrosarcoma protuberans.
Pukhalskaya T, Smoller BR
J Cutan Pathol 2020 Feb;47(2):135-138. Epub 2019 Nov 8 doi: 10.1111/cup.13596. PMID: 31614009
Histology- and non-histology-driven therapy for treatment of soft tissue sarcomas.
Casali PG
Ann Oncol 2012 Sep;23 Suppl 10:x167-9. doi: 10.1093/annonc/mds349. PMID: 22987955
Sarcomas with spindle cell morphology.
Collini P, Sorensen PH, Patel S, Blay JY, Issels RD, Maki RG, Eriksson M, del Muro XG
Semin Oncol 2009 Aug;36(4):324-37. doi: 10.1053/j.seminoncol.2009.06.007. PMID: 19664493

Prognosis

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
Spindle cell tumors of the pleura and the peritoneum: pathologic diagnosis and updates.
Boyraz B, Hung YP
APMIS 2022 Mar;130(3):140-154. Epub 2022 Jan 11 doi: 10.1111/apm.13203. PMID: 34942046
Ewing sarcoma and Ewing-like tumors.
Sbaraglia M, Righi A, Gambarotti M, Dei Tos AP
Virchows Arch 2020 Jan;476(1):109-119. Epub 2019 Dec 4 doi: 10.1007/s00428-019-02720-8. PMID: 31802230
The Diagnosis of Pleural Tumors Other Than Mesothelioma.
Attanoos RL, Pugh MR
Arch Pathol Lab Med 2018 Aug;142(8):902-913. doi: 10.5858/arpa.2017-0537-RA. PMID: 30040453
SMARCB1-deficient Tumors of Childhood: A Practical Guide.
Pawel BR
Pediatr Dev Pathol 2018 Jan-Feb;21(1):6-28. Epub 2017 Dec 27 doi: 10.1177/1093526617749671. PMID: 29280680

Clinical prediction guides

POU2AF3-rearranged sarcomas: A novel tumor defined by fusions of EWSR1 or FUS to a gene formerly designated COLCA2.
Hiemenz MC, Kaur J, Kuang Z, Huang RSP, Harries L, Metzger D, Schiavone K, Millis SZ, Lin DI, Lechpammer M, Decker B, Mata DA, Reddy A, Parke M, Lee EY, Cui X, Iwenofu OH, Buehler D, Henderson L, Baldwin EM, Boikos SA, Ramkissoon SH, Smith SC
Genes Chromosomes Cancer 2023 Aug;62(8):460-470. Epub 2023 Mar 31 doi: 10.1002/gcc.23136. PMID: 36862145
SWI/SNF complex-deficient soft tissue neoplasms: An update.
Schaefer IM, Hornick JL
Semin Diagn Pathol 2021 May;38(3):222-231. Epub 2020 Jun 5 doi: 10.1053/j.semdp.2020.05.005. PMID: 32646614Free PMC Article
Treatment of limb synovial sarcoma with metastasis at presentation.
Guo P, Zhao R, Zhou Y, Shen Y
Medicine (Baltimore) 2020 Jun 5;99(23):e20550. doi: 10.1097/MD.0000000000020550. PMID: 32502019Free PMC Article
Ewing sarcoma and Ewing-like tumors.
Sbaraglia M, Righi A, Gambarotti M, Dei Tos AP
Virchows Arch 2020 Jan;476(1):109-119. Epub 2019 Dec 4 doi: 10.1007/s00428-019-02720-8. PMID: 31802230
Epithelioid sarcoma of Enzinger.
Fisher C
Adv Anat Pathol 2006 May;13(3):114-21. doi: 10.1097/00125480-200605000-00002. PMID: 16778474

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