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Epithelioid Sarcoma NCI Grade 3

MedGen UID:
232465
Concept ID:
C1333425
Neoplastic Process
Synonym: Epithelioid Sarcoma NCI Grade III

Definition

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

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEpithelioid Sarcoma NCI Grade 3

Professional guidelines

PubMed

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
Crago AM, Dickson MA
Surg Oncol Clin N Am 2016 Oct;25(4):761-73. Epub 2016 Jul 30 doi: 10.1016/j.soc.2016.05.007. PMID: 27591497Free PMC Article

Recent clinical studies

Etiology

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
Stacchiotti S, Miah AB, Frezza AM, Messiou C, Morosi C, Caraceni A, Antonescu CR, Bajpai J, Baldini E, Bauer S, Biagini R, Bielack S, Blay JY, Bonvalot S, Boukovinas I, Bovee JVMG, Boye K, Brodowicz T, Callegaro D, De Alava E, Deoras-Sutliff M, Dufresne A, Eriksson M, Errani C, Fedenko A, Ferraresi V, Ferrari A, Fletcher CDM, Garcia Del Muro X, Gelderblom H, Gladdy RA, Gouin F, Grignani G, Gutkovich J, Haas R, Hindi N, Hohenberger P, Huang P, Joensuu H, Jones RL, Jungels C, Kasper B, Kawai A, Le Cesne A, Le Grange F, Leithner A, Leonard H, Lopez Pousa A, Martin Broto J, Merimsky O, Merriam P, Miceli R, Mir O, Molinari M, Montemurro M, Oldani G, Palmerini E, Pantaleo MA, Patel S, Piperno-Neumann S, Raut CP, Ravi V, Razak ARA, Reichardt P, Rubin BP, Rutkowski P, Safwat AA, Sangalli C, Sapisochin G, Sbaraglia M, Scheipl S, Schöffski P, Strauss D, Strauss SJ, Sundby Hall K, Tap WD, Trama A, Tweddle A, van der Graaf WTA, Van De Sande MAJ, Van Houdt W, van Oortmerssen G, Wagner AJ, Wartenberg M, Wood J, Zaffaroni N, Zimmermann C, Casali PG, Dei Tos AP, Gronchi A
ESMO Open 2021 Jun;6(3):100170. Epub 2021 Jun 2 doi: 10.1016/j.esmoop.2021.100170. PMID: 34090171Free 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
Crago AM, Dickson MA
Surg Oncol Clin N Am 2016 Oct;25(4):761-73. Epub 2016 Jul 30 doi: 10.1016/j.soc.2016.05.007. PMID: 27591497Free PMC Article

Diagnosis

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
Stacchiotti S, Miah AB, Frezza AM, Messiou C, Morosi C, Caraceni A, Antonescu CR, Bajpai J, Baldini E, Bauer S, Biagini R, Bielack S, Blay JY, Bonvalot S, Boukovinas I, Bovee JVMG, Boye K, Brodowicz T, Callegaro D, De Alava E, Deoras-Sutliff M, Dufresne A, Eriksson M, Errani C, Fedenko A, Ferraresi V, Ferrari A, Fletcher CDM, Garcia Del Muro X, Gelderblom H, Gladdy RA, Gouin F, Grignani G, Gutkovich J, Haas R, Hindi N, Hohenberger P, Huang P, Joensuu H, Jones RL, Jungels C, Kasper B, Kawai A, Le Cesne A, Le Grange F, Leithner A, Leonard H, Lopez Pousa A, Martin Broto J, Merimsky O, Merriam P, Miceli R, Mir O, Molinari M, Montemurro M, Oldani G, Palmerini E, Pantaleo MA, Patel S, Piperno-Neumann S, Raut CP, Ravi V, Razak ARA, Reichardt P, Rubin BP, Rutkowski P, Safwat AA, Sangalli C, Sapisochin G, Sbaraglia M, Scheipl S, Schöffski P, Strauss D, Strauss SJ, Sundby Hall K, Tap WD, Trama A, Tweddle A, van der Graaf WTA, Van De Sande MAJ, Van Houdt W, van Oortmerssen G, Wagner AJ, Wartenberg M, Wood J, Zaffaroni N, Zimmermann C, Casali PG, Dei Tos AP, Gronchi A
ESMO Open 2021 Jun;6(3):100170. Epub 2021 Jun 2 doi: 10.1016/j.esmoop.2021.100170. PMID: 34090171Free 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
Jiang M, Bennani NN, Feldman AL
Expert Rev Hematol 2017 Mar;10(3):239-249. Epub 2017 Jan 29 doi: 10.1080/17474086.2017.1281122. PMID: 28133975Free 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

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
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
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
Jiang M, Bennani NN, Feldman AL
Expert Rev Hematol 2017 Mar;10(3):239-249. Epub 2017 Jan 29 doi: 10.1080/17474086.2017.1281122. PMID: 28133975Free 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
Liu APY, Dhanda SK, Lin T, Sioson E, Vasilyeva A, Gudenas B, Tatevossian RG, Jia S, Neale G, Bowers DC, Hassall T, Partap S, Crawford JR, Chintagumpala M, Bouffet E, McCowage G, Broniscer A, Qaddoumi I, Armstrong G, Wright KD, Upadhyaya SA, Vinitsky A, Tinkle CL, Lucas J, Chiang J, Indelicato DJ, Sanders R, Klimo P Jr, Boop FA, Merchant TE, Ellison DW, Northcott PA, Orr BA, Zhou X, Onar-Thomas A, Gajjar A, Robinson GW
Acta Neuropathol 2022 Oct;144(4):733-746. Epub 2022 Aug 18 doi: 10.1007/s00401-022-02484-7. PMID: 35982322Free 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
Abdou Y, Elkhanany A, Attwood K, Ji W, Takabe K, Opyrchal M
Breast Cancer Res Treat 2019 Dec;178(3):523-533. Epub 2019 Sep 14 doi: 10.1007/s10549-019-05432-4. PMID: 31522347Free 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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