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Epithelioid Sarcoma NCI Grade 2

MedGen UID:
232464
Concept ID:
C1333424
Neoplastic Process
Synonym: Epithelioid Sarcoma NCI Grade II

Definition

An epithelioid sarcoma with less than 15% necrosis. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEpithelioid Sarcoma NCI Grade 2

Professional guidelines

PubMed

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
Piha-Paul SA, Oh DY, Ueno M, Malka D, Chung HC, Nagrial A, Kelley RK, Ros W, Italiano A, Nakagawa K, Rugo HS, de Braud F, Varga AI, Hansen A, Wang H, Krishnan S, Norwood KG, Doi T
Int J Cancer 2020 Oct 15;147(8):2190-2198. Epub 2020 May 2 doi: 10.1002/ijc.33013. PMID: 32359091
Spunt SL, Million L, Chi YY, Anderson J, Tian J, Hibbitts E, Coffin C, McCarville MB, Randall RL, Parham DM, Black JO, Kao SC, Hayes-Jordan A, Wolden S, Laurie F, Speights R, Kawashima E, Skapek SX, Meyer W, Pappo AS, Hawkins DS
Lancet Oncol 2020 Jan;21(1):145-161. Epub 2019 Nov 27 doi: 10.1016/S1470-2045(19)30672-2. PMID: 31786124Free PMC Article

Recent clinical studies

Etiology

Hong DS, Van Tine BA, Biswas S, McAlpine C, Johnson ML, Olszanski AJ, Clarke JM, Araujo D, Blumenschein GR Jr, Kebriaei P, Lin Q, Tipping AJ, Sanderson JP, Wang R, Trivedi T, Annareddy T, Bai J, Rafail S, Sun A, Fernandes L, Navenot JM, Bushman FD, Everett JK, Karadeniz D, Broad R, Isabelle M, Naidoo R, Bath N, Betts G, Wolchinsky Z, Batrakou DG, Van Winkle E, Elefant E, Ghobadi A, Cashen A, Grand'Maison A, McCarthy P, Fracasso PM, Norry E, Williams D, Druta M, Liebner DA, Odunsi K, Butler MO
Nat Med 2023 Jan;29(1):104-114. Epub 2023 Jan 9 doi: 10.1038/s41591-022-02128-z. PMID: 36624315Free PMC Article
O'Malley DM, Bariani GM, Cassier PA, Marabelle A, Hansen AR, De Jesus Acosta A, Miller WH Jr, Safra T, Italiano A, Mileshkin L, Xu L, Jin F, Norwood K, Maio M
J Clin Oncol 2022 Mar 1;40(7):752-761. Epub 2022 Jan 6 doi: 10.1200/JCO.21.01874. PMID: 34990208Free PMC Article
Skoulidis F, Li BT, Dy GK, Price TJ, Falchook GS, Wolf J, Italiano A, Schuler M, Borghaei H, Barlesi F, Kato T, Curioni-Fontecedro A, Sacher A, Spira A, Ramalingam SS, Takahashi T, Besse B, Anderson A, Ang A, Tran Q, Mather O, Henary H, Ngarmchamnanrith G, Friberg G, Velcheti V, Govindan R
N Engl J Med 2021 Jun 24;384(25):2371-2381. Epub 2021 Jun 4 doi: 10.1056/NEJMoa2103695. PMID: 34096690Free PMC Article
Doebele RC, Drilon A, Paz-Ares L, Siena S, Shaw AT, Farago AF, Blakely CM, Seto T, Cho BC, Tosi D, Besse B, Chawla SP, Bazhenova L, Krauss JC, Chae YK, Barve M, Garrido-Laguna I, Liu SV, Conkling P, John T, Fakih M, Sigal D, Loong HH, Buchschacher GL Jr, Garrido P, Nieva J, Steuer C, Overbeck TR, Bowles DW, Fox E, Riehl T, Chow-Maneval E, Simmons B, Cui N, Johnson A, Eng S, Wilson TR, Demetri GD; trial investigators
Lancet Oncol 2020 Feb;21(2):271-282. Epub 2019 Dec 11 doi: 10.1016/S1470-2045(19)30691-6. PMID: 31838007Free PMC Article
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

Diagnosis

Momeni-Boroujeni A, Yousefi E, Balakrishnan R, Riviere S, Kertowidjojo E, Hensley ML, Ladanyi M, Ellenson LH, Chiang S
Mod Pathol 2023 Apr;36(4):100084. Epub 2023 Jan 10 doi: 10.1016/j.modpat.2022.100084. PMID: 36788080Free PMC Article
Bogani G, Ray-Coquard I, Concin N, Ngoi NYL, Morice P, Caruso G, Enomoto T, Takehara K, Denys H, Lorusso D, Coleman R, Vaughan MM, Takano M, Provencher DM, Sagae S, Wimberger P, Póka R, Segev Y, Kim SI, Kim JW, Candido Dos Reis FJ, Ramirez PT, Mariani A, Leitao M, Makker V, Abu-Rustum NR, Vergote I, Zannoni G, Tan D, McCormack M, Paolini B, Bini M, Raspagliesi F, Benedetti Panici P, Di Donato V, Muzii L, Colombo N, Pignata S, Scambia G, Monk BJ
Int J Gynecol Cancer 2023 Feb 6;33(2):147-174. doi: 10.1136/ijgc-2022-004073. PMID: 36585027
Weiss AR, Chen YL, Scharschmidt TJ, Chi YY, Tian J, Black JO, Davis JL, Fanburg-Smith JC, Zambrano E, Anderson J, Arens R, Binitie O, Choy E, Davis JW, Hayes-Jordan A, Kao SC, Kayton ML, Kessel S, Lim R, Meyer WH, Million L, Okuno SH, Ostrenga A, Parisi MT, Pryma DA, Randall RL, Rosen MA, Schlapkohl M, Shulkin BL, Smith EA, Sorger JI, Terezakis S, Hawkins DS, Spunt SL, Wang D
Lancet Oncol 2020 Aug;21(8):1110-1122. Epub 2020 Jul 20 doi: 10.1016/S1470-2045(20)30325-9. PMID: 32702309Free PMC Article
Italiano A, Mir O, Mathoulin-Pelissier S, Penel N, Piperno-Neumann S, Bompas E, Chevreau C, Duffaud F, Entz-Werlé N, Saada E, Ray-Coquard I, Lervat C, Gaspar N, Marec-Berard P, Pacquement H, Wright J, Toulmonde M, Bessede A, Crombe A, Kind M, Bellera C, Blay JY
Lancet Oncol 2020 Mar;21(3):446-455. Epub 2020 Feb 17 doi: 10.1016/S1470-2045(19)30825-3. PMID: 32078813Free PMC Article
Peres LC, Cushing-Haugen KL, Köbel M, Harris HR, Berchuck A, Rossing MA, Schildkraut JM, Doherty JA
J Natl Cancer Inst 2019 Jan 1;111(1):60-68. doi: 10.1093/jnci/djy071. PMID: 29718305Free PMC Article

Therapy

Meric-Bernstam F, Makker V, Oaknin A, Oh DY, Banerjee S, González-Martín A, Jung KH, Ługowska I, Manso L, Manzano A, Melichar B, Siena S, Stroyakovskiy D, Fielding A, Ma Y, Puvvada S, Shire N, Lee JY
J Clin Oncol 2024 Jan 1;42(1):47-58. Epub 2023 Oct 23 doi: 10.1200/JCO.23.02005. PMID: 37870536Free PMC Article
Bogani G, Ray-Coquard I, Concin N, Ngoi NYL, Morice P, Caruso G, Enomoto T, Takehara K, Denys H, Lorusso D, Coleman R, Vaughan MM, Takano M, Provencher DM, Sagae S, Wimberger P, Póka R, Segev Y, Kim SI, Kim JW, Candido Dos Reis FJ, Ramirez PT, Mariani A, Leitao M, Makker V, Abu-Rustum NR, Vergote I, Zannoni G, Tan D, McCormack M, Paolini B, Bini M, Raspagliesi F, Benedetti Panici P, Di Donato V, Muzii L, Colombo N, Pignata S, Scambia G, Monk BJ
Int J Gynecol Cancer 2023 Feb 6;33(2):147-174. doi: 10.1136/ijgc-2022-004073. PMID: 36585027
O'Malley DM, Bariani GM, Cassier PA, Marabelle A, Hansen AR, De Jesus Acosta A, Miller WH Jr, Safra T, Italiano A, Mileshkin L, Xu L, Jin F, Norwood K, Maio M
J Clin Oncol 2022 Mar 1;40(7):752-761. Epub 2022 Jan 6 doi: 10.1200/JCO.21.01874. PMID: 34990208Free PMC Article
Skoulidis F, Li BT, Dy GK, Price TJ, Falchook GS, Wolf J, Italiano A, Schuler M, Borghaei H, Barlesi F, Kato T, Curioni-Fontecedro A, Sacher A, Spira A, Ramalingam SS, Takahashi T, Besse B, Anderson A, Ang A, Tran Q, Mather O, Henary H, Ngarmchamnanrith G, Friberg G, Velcheti V, Govindan R
N Engl J Med 2021 Jun 24;384(25):2371-2381. Epub 2021 Jun 4 doi: 10.1056/NEJMoa2103695. PMID: 34096690Free PMC Article
Doebele RC, Drilon A, Paz-Ares L, Siena S, Shaw AT, Farago AF, Blakely CM, Seto T, Cho BC, Tosi D, Besse B, Chawla SP, Bazhenova L, Krauss JC, Chae YK, Barve M, Garrido-Laguna I, Liu SV, Conkling P, John T, Fakih M, Sigal D, Loong HH, Buchschacher GL Jr, Garrido P, Nieva J, Steuer C, Overbeck TR, Bowles DW, Fox E, Riehl T, Chow-Maneval E, Simmons B, Cui N, Johnson A, Eng S, Wilson TR, Demetri GD; trial investigators
Lancet Oncol 2020 Feb;21(2):271-282. Epub 2019 Dec 11 doi: 10.1016/S1470-2045(19)30691-6. PMID: 31838007Free PMC Article

Prognosis

Chen AP, Sharon E, O'Sullivan-Coyne G, Moore N, Foster JC, Hu JS, Van Tine BA, Conley AP, Read WL, Riedel RF, Burgess MA, Glod J, Davis EJ, Merriam P, Naqash AR, Fino KK, Miller BL, Wilsker DF, Begum A, Ferry-Galow KV, Deshpande HA, Schwartz GK, Ladle BH, Okuno SH, Beck JC, Chen JL, Takebe N, Fogli LK, Rosenberger CL, Parchment RE, Doroshow JH
N Engl J Med 2023 Sep 7;389(10):911-921. doi: 10.1056/NEJMoa2303383. PMID: 37672694Free PMC Article
O'Malley DM, Bariani GM, Cassier PA, Marabelle A, Hansen AR, De Jesus Acosta A, Miller WH Jr, Safra T, Italiano A, Mileshkin L, Xu L, Jin F, Norwood K, Maio M
J Clin Oncol 2022 Mar 1;40(7):752-761. Epub 2022 Jan 6 doi: 10.1200/JCO.21.01874. PMID: 34990208Free PMC Article
Wagner AJ, Ravi V, Riedel RF, Ganjoo K, Van Tine BA, Chugh R, Cranmer L, Gordon EM, Hornick JL, Du H, Grigorian B, Schmid AN, Hou S, Harris K, Kwiatkowski DJ, Desai NP, Dickson MA
J Clin Oncol 2021 Nov 20;39(33):3660-3670. Epub 2021 Oct 12 doi: 10.1200/JCO.21.01728. PMID: 34637337Free PMC Article
Blay JY, Serrano C, Heinrich MC, Zalcberg J, Bauer S, Gelderblom H, Schöffski P, Jones RL, Attia S, D'Amato G, Chi P, Reichardt P, Meade J, Shi K, Ruiz-Soto R, George S, von Mehren M
Lancet Oncol 2020 Jul;21(7):923-934. Epub 2020 Jun 5 doi: 10.1016/S1470-2045(20)30168-6. PMID: 32511981Free PMC Article
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

Clinical prediction guides

Meric-Bernstam F, Makker V, Oaknin A, Oh DY, Banerjee S, González-Martín A, Jung KH, Ługowska I, Manso L, Manzano A, Melichar B, Siena S, Stroyakovskiy D, Fielding A, Ma Y, Puvvada S, Shire N, Lee JY
J Clin Oncol 2024 Jan 1;42(1):47-58. Epub 2023 Oct 23 doi: 10.1200/JCO.23.02005. PMID: 37870536Free PMC Article
Chen AP, Sharon E, O'Sullivan-Coyne G, Moore N, Foster JC, Hu JS, Van Tine BA, Conley AP, Read WL, Riedel RF, Burgess MA, Glod J, Davis EJ, Merriam P, Naqash AR, Fino KK, Miller BL, Wilsker DF, Begum A, Ferry-Galow KV, Deshpande HA, Schwartz GK, Ladle BH, Okuno SH, Beck JC, Chen JL, Takebe N, Fogli LK, Rosenberger CL, Parchment RE, Doroshow JH
N Engl J Med 2023 Sep 7;389(10):911-921. doi: 10.1056/NEJMoa2303383. PMID: 37672694Free PMC Article
Bauer S, Jones RL, Blay JY, Gelderblom H, George S, Schöffski P, von Mehren M, Zalcberg JR, Kang YK, Razak AA, Trent J, Attia S, Le Cesne A, Su Y, Meade J, Wang T, Sherman ML, Ruiz-Soto R, Heinrich MC
J Clin Oncol 2022 Dec 1;40(34):3918-3928. Epub 2022 Aug 10 doi: 10.1200/JCO.22.00294. PMID: 35947817Free PMC Article
Skoulidis F, Li BT, Dy GK, Price TJ, Falchook GS, Wolf J, Italiano A, Schuler M, Borghaei H, Barlesi F, Kato T, Curioni-Fontecedro A, Sacher A, Spira A, Ramalingam SS, Takahashi T, Besse B, Anderson A, Ang A, Tran Q, Mather O, Henary H, Ngarmchamnanrith G, Friberg G, Velcheti V, Govindan R
N Engl J Med 2021 Jun 24;384(25):2371-2381. Epub 2021 Jun 4 doi: 10.1056/NEJMoa2103695. PMID: 34096690Free PMC Article
Davis LE, Bolejack V, Ryan CW, Ganjoo KN, Loggers ET, Chawla S, Agulnik M, Livingston MB, Reed D, Keedy V, Rushing D, Okuno S, Reinke DK, Riedel RF, Attia S, Mascarenhas L, Maki RG
J Clin Oncol 2019 Jun 1;37(16):1424-1431. Epub 2019 Apr 23 doi: 10.1200/JCO.18.02374. PMID: 31013172Free PMC Article

Recent systematic reviews

Keung EZ, Krause KJ, Maxwell J, Morris CD, Crago AM, Houdek MT, Kane J, Lewis V, Callegaro D, Miller B, Lazar AJ, Gladdy R, Raut CP, Fabbri N, Al-Refaie W, Fairweather M, Wong SL, Roland CL
Ann Surg Oncol 2023 Feb;30(2):958-967. Epub 2022 Oct 28 doi: 10.1245/s10434-022-12688-6. PMID: 36307665
Suurmeijer AJH, Dickson BC, Swanson D, Zhang L, Sung YS, Cotzia P, Fletcher CDM, Antonescu CR
Genes Chromosomes Cancer 2018 Dec;57(12):611-621. Epub 2018 Oct 1 doi: 10.1002/gcc.22671. PMID: 30276917Free PMC Article
Chan DLH, Segelov E, Wong RS, Smith A, Herbertson RA, Li BT, Tebbutt N, Price T, Pavlakis N
Cochrane Database Syst Rev 2017 Jun 27;6(6):CD007047. doi: 10.1002/14651858.CD007047.pub2. PMID: 28654140Free PMC Article
Cheng H, Miura JT, Lalehzari M, Rajeev R, Donahue AE, Bedi M, Gamblin TC, Turaga KK, Johnston FM
J Surg Oncol 2016 May;113(6):628-34. Epub 2016 Mar 16 doi: 10.1002/jso.24221. PMID: 26990903

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