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Epithelioid sarcoma

MedGen UID:
104753
Concept ID:
C0205944
Neoplastic Process
Synonyms: Epithelioid Sarcoma; Epithelioid Sarcomas; Sarcoma, Epithelioid; Sarcomas, Epithelioid
SNOMED CT: Epithelioid sarcoma (782827000); Epithelioid sarcoma (59238007); Epithelioid cell sarcoma (59238007)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0017387
Orphanet: ORPHA293202

Definition

A rare soft tissue tumour characterised by high incidence of local recurrence, regional lymph node involvement and distant metastases. It commonly affects the soft tissue under the skin of a finger, hand, forearm, lower leg or foot, less often other areas of the body. [from SNOMEDCT_US]

Professional guidelines

PubMed

Chokoeva AA, Tchernev G, Cardoso JC, Patterson JW, Dechev I, Valkanov S, Zanardelli M, Lotti T, Wollina U
Int J Immunopathol Pharmacol 2015 Jun;28(2):168-77. Epub 2015 Mar 26 doi: 10.1177/0394632015576029. PMID: 25816394
Chokoeva AA, Tchernev G, Cardoso JC, Patterson JW, Dechev I, Valkanov S, Zanardelli M, Lotti T, Wollina U
Int J Immunopathol Pharmacol 2015 Jun;28(2):178-86. Epub 2015 Mar 26 doi: 10.1177/0394632015575977. PMID: 25816393
Babu R, Karikari IO, Cummings TJ, Gottfried ON, Bagley CA
J Clin Neurosci 2013 Oct;20(10):1342-5. Epub 2013 Apr 23 doi: 10.1016/j.jocn.2012.12.009. PMID: 23623614

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
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
Geoerger B, Kang HJ, Yalon-Oren M, Marshall LV, Vezina C, Pappo A, Laetsch TW, Petrilli AS, Ebinger M, Toporski J, Glade-Bender J, Nicholls W, Fox E, DuBois SG, Macy ME, Cohn SL, Pathiraja K, Diede SJ, Ebbinghaus S, Pinto N
Lancet Oncol 2020 Jan;21(1):121-133. Epub 2019 Dec 4 doi: 10.1016/S1470-2045(19)30671-0. PMID: 31812554
Italiano A, Soria JC, Toulmonde M, Michot JM, Lucchesi C, Varga A, Coindre JM, Blakemore SJ, Clawson A, Suttle B, McDonald AA, Woodruff M, Ribich S, Hedrick E, Keilhack H, Thomson B, Owa T, Copeland RA, Ho PTC, Ribrag V
Lancet Oncol 2018 May;19(5):649-659. Epub 2018 Apr 9 doi: 10.1016/S1470-2045(18)30145-1. PMID: 29650362
Sennwald G, Schmid U, Segmüller G, Jungi F, Hardmeier T, Gloor F
Ann Chir Main 1983;2(4):313-8. doi: 10.1016/s0753-9053(83)80027-1. PMID: 9336649

Diagnosis

Renn A, Adejolu M, Messiou C, Bhaludin B, Strauss DC, Thway K, Moskovic E
Clin Radiol 2021 Dec;76(12):940.e1-940.e16. Epub 2021 Oct 1 doi: 10.1016/j.crad.2021.08.011. PMID: 34607656
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
Ansai SI, Morimoto M, Akaishi S
J Nippon Med Sch 2019;86(2):126-130. doi: 10.1272/jnms.JNMS.2019_86-209. PMID: 31130564
Al-Qaderi A, Mansour AT
Arch Pathol Lab Med 2019 Jun;143(6):763-767. Epub 2018 Dec 21 doi: 10.5858/arpa.2017-0430-RS. PMID: 30576238
Pawel BR
Pediatr Dev Pathol 2018 Jan-Feb;21(1):6-28. Epub 2017 Dec 27 doi: 10.1177/1093526617749671. PMID: 29280680

Therapy

Zhang D, Hu J, Liu Z, Wu H, Cheng H, Li C
Cancer Med 2023 Feb;12(3):3079-3088. Epub 2022 Sep 8 doi: 10.1002/cam4.5230. PMID: 36073670Free PMC Article
Simeone N, Frezza AM, Zaffaroni N, Stacchiotti S
Future Oncol 2021 Apr;17(10):1253-1263. Epub 2020 Dec 8 doi: 10.2217/fon-2020-0781. PMID: 33289402
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
Geoerger B, Kang HJ, Yalon-Oren M, Marshall LV, Vezina C, Pappo A, Laetsch TW, Petrilli AS, Ebinger M, Toporski J, Glade-Bender J, Nicholls W, Fox E, DuBois SG, Macy ME, Cohn SL, Pathiraja K, Diede SJ, Ebbinghaus S, Pinto N
Lancet Oncol 2020 Jan;21(1):121-133. Epub 2019 Dec 4 doi: 10.1016/S1470-2045(19)30671-0. PMID: 31812554
Italiano A, Soria JC, Toulmonde M, Michot JM, Lucchesi C, Varga A, Coindre JM, Blakemore SJ, Clawson A, Suttle B, McDonald AA, Woodruff M, Ribich S, Hedrick E, Keilhack H, Thomson B, Owa T, Copeland RA, Ho PTC, Ribrag V
Lancet Oncol 2018 May;19(5):649-659. Epub 2018 Apr 9 doi: 10.1016/S1470-2045(18)30145-1. PMID: 29650362

Prognosis

Zhang D, Hu J, Liu Z, Wu H, Cheng H, Li C
Cancer Med 2023 Feb;12(3):3079-3088. Epub 2022 Sep 8 doi: 10.1002/cam4.5230. PMID: 36073670Free 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
Simeone N, Frezza AM, Zaffaroni N, Stacchiotti S
Future Oncol 2021 Apr;17(10):1253-1263. Epub 2020 Dec 8 doi: 10.2217/fon-2020-0781. PMID: 33289402
Al-Qaderi A, Mansour AT
Arch Pathol Lab Med 2019 Jun;143(6):763-767. Epub 2018 Dec 21 doi: 10.5858/arpa.2017-0430-RS. PMID: 30576238
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

Zhang D, Hu J, Liu Z, Wu H, Cheng H, Li C
Cancer Med 2023 Feb;12(3):3079-3088. Epub 2022 Sep 8 doi: 10.1002/cam4.5230. PMID: 36073670Free PMC Article
Renn A, Adejolu M, Messiou C, Bhaludin B, Strauss DC, Thway K, Moskovic E
Clin Radiol 2021 Dec;76(12):940.e1-940.e16. Epub 2021 Oct 1 doi: 10.1016/j.crad.2021.08.011. PMID: 34607656
Simeone N, Frezza AM, Zaffaroni N, Stacchiotti S
Future Oncol 2021 Apr;17(10):1253-1263. Epub 2020 Dec 8 doi: 10.2217/fon-2020-0781. PMID: 33289402
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
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

Recent systematic reviews

Hu W, Wu X, Ma H, Wang H, Shi X, Zhang K, Gao Y
Med Sci Monit 2023 Jan 6;29:e938416. doi: 10.12659/MSM.938416. PMID: 36604864Free PMC Article
Xia J, Li J, Tian L, Ren X, Liu C, Liang C
J Med Chem 2022 May 26;65(10):7016-7043. Epub 2022 May 7 doi: 10.1021/acs.jmedchem.2c00047. PMID: 35531606
Brance ML, Cóccaro NM, Roitman P, Castiglioni A, Agostinis F, Spense M, Scheitlin B, Rene N, Brun LR
Arch Osteoporos 2022 Feb 1;17(1):28. doi: 10.1007/s11657-022-01062-4. PMID: 35106633
Persitz J, Beit Ner E, Chechik I, Keren T, Avisar E
J Plast Surg Hand Surg 2021 Apr;55(2):96-104. Epub 2020 Nov 11 doi: 10.1080/2000656X.2020.1838914. PMID: 33176534

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