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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

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
DOG1 antibody in the differential diagnosis of gastrointestinal stromal tumors: a study of 1840 cases.
Miettinen M, Wang ZF, Lasota J
Am J Surg Pathol 2009 Sep;33(9):1401-8. doi: 10.1097/PAS.0b013e3181a90e1a. PMID: 19606013
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
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
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
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
Treatment of soft-tissue sarcomas of the hand.
Brien EW, Terek RM, Geer RJ, Caldwell G, Brennan MF, Healey JH
J Bone Joint Surg Am 1995 Apr;77(4):564-71. doi: 10.2106/00004623-199504000-00009. PMID: 7713973

Diagnosis

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
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
Immunohistochemistry of soft tissue tumours - review with emphasis on 10 markers.
Miettinen M
Histopathology 2014 Jan;64(1):101-18. Epub 2013 Nov 28 doi: 10.1111/his.12298. PMID: 24111893Free PMC Article

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
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
High dose tumor necrosis factor-alpha and melphalan administered via isolated limb perfusion for advanced limb soft tissue sarcoma results in a >90% response rate and limb preservation.
Gutman M, Inbar M, Lev-Shlush D, Abu-Abid S, Mozes M, Chaitchik S, Meller I, Klausner JM
Cancer 1997 Mar 15;79(6):1129-37. doi: 10.1002/(sici)1097-0142(19970315)79:6<1129::aid-cncr11>3.0.co;2-1. PMID: 9070490

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
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
Distal extremities soft tissue sarcomas: Are they so different from other limb localizations?
Mattei JC, Brouste V, Terrier P, Bonvalot S, Lecesne A, Stoeckle E, Italiano A, Ranchere-Vince D, Meeus P, Laé M, Rosset P, Rochwerger A, Coindre JM, Salas S
J Surg Oncol 2019 Mar;119(4):479-488. Epub 2019 Jan 4 doi: 10.1002/jso.25359. PMID: 30609044
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

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
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
Loss of H3K27 trimethylation distinguishes malignant peripheral nerve sheath tumors from histologic mimics.
Schaefer IM, Fletcher CD, Hornick JL
Mod Pathol 2016 Jan;29(1):4-13. Epub 2015 Nov 20 doi: 10.1038/modpathol.2015.134. PMID: 26585554
Epithelioid sarcoma of Enzinger.
Fisher C
Adv Anat Pathol 2006 May;13(3):114-21. doi: 10.1097/00125480-200605000-00002. PMID: 16778474
Expression of receptor tyrosine kinases epidermal growth factor receptor and HER-2/neu in synovial sarcoma.
Thomas DG, Giordano TJ, Sanders D, Biermann S, Sondak VK, Trent JC, Yu D, Pollock RE, Baker L
Cancer 2005 Feb 15;103(4):830-8. doi: 10.1002/cncr.20847. PMID: 15641030

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