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Epithelioid Sarcoma

MedGen UID:
104753
Concept ID:
C0205944
Neoplastic Process
Synonyms: Epithelioid Sarcomas; Sarcoma, Epithelioid; Sarcomas, Epithelioid
Modes of inheritance:
not inherited
MedGen UID:
832438
Concept ID:
CN227390
Intellectual Product
Source: Orphanet
Describes a disorder that is not inherited.
not inherited (Orphanet)
SNOMED CT: Epithelioid sarcoma (59238007); Epithelioid cell sarcoma (59238007)
 
Orphanet: ORPHA293202

Definition

An aggressive malignant neoplasm of uncertain differentiation, characterized by the presence of epithelioid cells forming nodular patterns. The nodules often undergo central necrosis, resulting in a pseudogranulomatous growth pattern. It usually occurs in young adults. The most common sites of involvement are the extremities (distal-type epithelioid sarcoma), and less frequently the pelvis, perineum, and genital organs (proximal-type epithelioid sarcoma). [from NCI]

Recent clinical studies

Etiology

Bhattacharyya R, Ghosh R, Saha K, Chatterjee U
Fetal Pediatr Pathol 2017 Aug;36(4):325-331. Epub 2017 May 25 doi: 10.1080/15513815.2017.1323984. PMID: 28541826
Echchaoui A, Sadrati Y, Elbir Y, Elktaibi A, Benyachou M, Mazouz SE, Gharib NE, Abbassi A
Pan Afr Med J 2016;24:238. Epub 2016 Jul 14 doi: 10.11604/pamj.2016.24.238.8535. PMID: 27800093Free PMC Article
Nishibaba R, Higashi Y, Goto Y, Hisaoka M, Kanekura T
J Med Case Rep 2016 Oct 24;10(1):295. doi: 10.1186/s13256-016-1088-z. PMID: 27776545Free PMC Article
Tsubokawa N, Harada H, Taniyama D, Uemura T, Kuraoka K, Yamashita Y
Asian Cardiovasc Thorac Ann 2016 Oct;24(8):814-817. Epub 2016 Aug 4 doi: 10.1177/0218492316664672. PMID: 27493191
Jamshidi F, Bashashati A, Shumansky K, Dickson B, Gokgoz N, Wunder JS, Andrulis IL, Lazar AJ, Shah SP, Huntsman DG, Nielsen TO
J Pathol 2016 Jan;238(1):63-73. Epub 2015 Oct 19 doi: 10.1002/path.4636. PMID: 26365879

Diagnosis

Bhattacharyya R, Ghosh R, Saha K, Chatterjee U
Fetal Pediatr Pathol 2017 Aug;36(4):325-331. Epub 2017 May 25 doi: 10.1080/15513815.2017.1323984. PMID: 28541826
Nishibaba R, Higashi Y, Goto Y, Hisaoka M, Kanekura T
J Med Case Rep 2016 Oct 24;10(1):295. doi: 10.1186/s13256-016-1088-z. PMID: 27776545Free PMC Article
Lopez G, Song Y, Lam R, Ruder D, Creighton CJ, Bid HK, Bill KL, Bolshakov S, Zhang X, Lev D, Pollock RE
Mol Cancer Res 2016 Jan;14(1):35-43. Epub 2015 Sep 22 doi: 10.1158/1541-7786.MCR-15-0295. PMID: 26396249Free PMC Article
Jurdy LL, Blank LE, Bras J, Saeed P
Ophthalmic Plast Reconstr Surg 2016 Mar-Apr;32(2):e47-8. doi: 10.1097/IOP.0000000000000227. PMID: 25025387
Pendse AA, Dodd LG
Diagn Cytopathol 2015 Oct;43(10):859-62. Epub 2015 Jul 3 doi: 10.1002/dc.23294. PMID: 26138930

Therapy

Schuetze SM, Bolejack V, Choy E, Ganjoo KN, Staddon AP, Chow WA, Tawbi HA, Samuels BL, Patel SR, von Mehren M, D'Amato G, Leu KM, Loeb DM, Forscher CA, Milhem MM, Rushing DA, Lucas DR, Chugh R, Reinke DK, Baker LH
Cancer 2017 Jan 1;123(1):90-97. Epub 2016 Oct 3 doi: 10.1002/cncr.30379. PMID: 27696380
Lopez G, Song Y, Lam R, Ruder D, Creighton CJ, Bid HK, Bill KL, Bolshakov S, Zhang X, Lev D, Pollock RE
Mol Cancer Res 2016 Jan;14(1):35-43. Epub 2015 Sep 22 doi: 10.1158/1541-7786.MCR-15-0295. PMID: 26396249Free PMC Article
Jurdy LL, Blank LE, Bras J, Saeed P
Ophthalmic Plast Reconstr Surg 2016 Mar-Apr;32(2):e47-8. doi: 10.1097/IOP.0000000000000227. PMID: 25025387
Jones RL, Constantinidou A, Olmos D, Thway K, Fisher C, Al-Muderis O, Scurr M, Judson IR
Am J Clin Oncol 2012 Aug;35(4):351-7. doi: 10.1097/COC.0b013e3182118cf7. PMID: 21422990
Uschuplich V, Gadre SA, Debski RF, Sahoo S
Diagn Cytopathol 2010 Oct;38(10):744-7. doi: 10.1002/dc.21304. PMID: 20058307

Prognosis

Suárez-Zamora DA, Barrera-Herrera LE, Rodríguez-Urrego PA, Palau-Lázaro MA
Int J Surg Pathol 2017 Aug;25(5):468-471. Epub 2017 Feb 1 doi: 10.1177/1066896917696744. PMID: 28381140
Echchaoui A, Sadrati Y, Elbir Y, Elktaibi A, Benyachou M, Mazouz SE, Gharib NE, Abbassi A
Pan Afr Med J 2016;24:238. Epub 2016 Jul 14 doi: 10.11604/pamj.2016.24.238.8535. PMID: 27800093Free PMC Article
Tsubokawa N, Harada H, Taniyama D, Uemura T, Kuraoka K, Yamashita Y
Asian Cardiovasc Thorac Ann 2016 Oct;24(8):814-817. Epub 2016 Aug 4 doi: 10.1177/0218492316664672. PMID: 27493191
Lopez G, Song Y, Lam R, Ruder D, Creighton CJ, Bid HK, Bill KL, Bolshakov S, Zhang X, Lev D, Pollock RE
Mol Cancer Res 2016 Jan;14(1):35-43. Epub 2015 Sep 22 doi: 10.1158/1541-7786.MCR-15-0295. PMID: 26396249Free PMC Article
Colombo F, Franceschelli A, Schiavina R, Gentile G, Passaretti G, Martorana G
J Sex Med 2013 Nov;10(11):2871-4. Epub 2012 May 22 doi: 10.1111/j.1743-6109.2012.02774.x. PMID: 22616634

Clinical prediction guides

Tsubokawa N, Harada H, Taniyama D, Uemura T, Kuraoka K, Yamashita Y
Asian Cardiovasc Thorac Ann 2016 Oct;24(8):814-817. Epub 2016 Aug 4 doi: 10.1177/0218492316664672. PMID: 27493191
Stockman DL, Hornick JL, Deavers MT, Lev DC, Lazar AJ, Wang WL
Mod Pathol 2014 Apr;27(4):496-501. Epub 2013 Sep 27 doi: 10.1038/modpathol.2013.161. PMID: 24072183
Miettinen M, Wang Z, Sarlomo-Rikala M, Abdullaev Z, Pack SD, Fetsch JF
Am J Surg Pathol 2013 Oct;37(10):1580-5. doi: 10.1097/PAS.0b013e31828de23a. PMID: 23774169Free PMC Article
Brenca M, Rossi S, Lorenzetto E, Piccinin E, Piccinin S, Rossi FM, Giuliano A, Dei Tos AP, Maestro R, Modena P
Mol Cancer Ther 2013 Jun;12(6):1060-72. Epub 2013 Apr 10 doi: 10.1158/1535-7163.MCT-13-0005. PMID: 23576573
Hoshino M, Kawashima H, Ogose A, Kudo N, Ariizumi T, Hotta T, Umezu H, Hatano H, Morita T, Nishio J, Iwasaki H, Endo N
J Cancer Res Clin Oncol 2010 Mar;136(3):457-64. Epub 2009 Sep 16 doi: 10.1007/s00432-009-0678-1. PMID: 19756736

Recent systematic reviews

Iavazzo C, Gkegkes ID, Vrachnis N
Eur J Obstet Gynecol Reprod Biol 2014 May;176:1-4. Epub 2014 Feb 20 doi: 10.1016/j.ejogrb.2014.02.013. PMID: 24636595

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