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Advanced Epithelioid Sarcoma

MedGen UID:
1717222
•Concept ID:
C5238508
•
Neoplastic Process

Definition

Epithelioid sarcoma that has spread extensively to other anatomic sites or is no longer responding to treatment. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAdvanced Epithelioid Sarcoma

Professional guidelines

PubMed

International Consensus Statement on Allergy and Rhinology: Sinonasal Tumors.
Kuan EC, Wang EW, Adappa ND, Beswick DM, London NR Jr, Su SY, Wang MB, Abuzeid WM, Alexiev B, Alt JA, Antognoni P, Alonso-Basanta M, Batra PS, Bhayani M, Bell D, Bernal-Sprekelsen M, Betz CS, Blay JY, Bleier BS, Bonilla-Velez J, Callejas C, Carrau RL, Casiano RR, Castelnuovo P, Chandra RK, Chatzinakis V, Chen SB, Chiu AG, Choby G, Chowdhury NI, Citardi MJ, Cohen MA, Dagan R, Dalfino G, Dallan I, Dassi CS, de Almeida J, Dei Tos AP, DelGaudio JM, Ebert CS, El-Sayed IH, Eloy JA, Evans JJ, Fang CH, Farrell NF, Ferrari M, Fischbein N, Folbe A, Fokkens WJ, Fox MG, Lund VJ, Gallia GL, Gardner PA, Geltzeiler M, Georgalas C, Getz AE, Govindaraj S, Gray ST, Grayson JW, Gross BA, Grube JG, Guo R, Ha PK, Halderman AA, Hanna EY, Harvey RJ, Hernandez SC, Holtzman AL, Hopkins C, Huang Z, Huang Z, Humphreys IM, Hwang PH, Iloreta AM, Ishii M, Ivan ME, Jafari A, Kennedy DW, Khan M, Kimple AJ, Kingdom TT, Knisely A, Kuo YJ, Lal D, Lamarre ED, Lan MY, Le H, Lechner M, Lee NY, Lee JK, Lee VH, Levine CG, Lin JC, Lin DT, Lobo BC, Locke T, Luong AU, Magliocca KR, Markovic SN, Matnjani G, McKean EL, Meço C, Mendenhall WM, Michel L, Na'ara S, Nicolai P, Nuss DW, Nyquist GG, Oakley GM, Omura K, Orlandi RR, Otori N, Papagiannopoulos P, Patel ZM, Pfister DG, Phan J, Psaltis AJ, Rabinowitz MR, Ramanathan M Jr, Rimmer R, Rosen MR, Sanusi O, Sargi ZB, Schafhausen P, Schlosser RJ, Sedaghat AR, Senior BA, Shrivastava R, Sindwani R, Smith TL, Smith KA, Snyderman CH, Solares CA, Sreenath SB, Stamm A, Stölzel K, Sumer B, Surda P, Tajudeen BA, Thompson LDR, Thorp BD, Tong CCL, Tsang RK, Turner JH, Turri-Zanoni M, Udager AM, van Zele T, VanKoevering K, Welch KC, Wise SK, Witterick IJ, Won TB, Wong SN, Woodworth BA, Wormald PJ, Yao WC, Yeh CF, Zhou B, Palmer JN
Int Forum Allergy Rhinol 2024 Feb;14(2):149-608. Epub 2024 Jan 2 doi: 10.1002/alr.23262. PMID: 37658764
Management of Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma.
Crago AM, Cardona K, Koseła-Paterczyk H, Rutkowski P
Surg Oncol Clin N Am 2022 Jul;31(3):419-430. doi: 10.1016/j.soc.2022.03.006. PMID: 35715142Free PMC Article
Myeloid Sarcoma: Presentation, Diagnosis, and Treatment.
Almond LM, Charalampakis M, Ford SJ, Gourevitch D, Desai A
Clin Lymphoma Myeloma Leuk 2017 May;17(5):263-267. Epub 2017 Mar 7 doi: 10.1016/j.clml.2017.02.027. PMID: 28342811

Recent clinical studies

Etiology

Tazemetostat in advanced epithelioid sarcoma with loss of INI1/SMARCB1: an international, open-label, phase 2 basket study.
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
Targeting epigenetics in sarcomas through EZH2 inhibition.
Italiano A
J Hematol Oncol 2020 Apr 7;13(1):33. doi: 10.1186/s13045-020-00868-4. PMID: 32264965Free PMC Article
Role of palliative chemotherapy in advanced epithelioid sarcoma.
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

Diagnosis

Inhibition of EZH2 enhances the therapeutic effect of 5-FU via PUMA upregulation in colorectal cancer.
Tan X, Zhang Z, Liu P, Yao H, Shen L, Tong JS
Cell Death Dis 2020 Dec 12;11(12):1061. doi: 10.1038/s41419-020-03266-3. PMID: 33311453Free PMC Article
Role of palliative chemotherapy in advanced epithelioid sarcoma.
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
Expression of lung resistance protein in epithelioid sarcoma in vitro and in vivo.
Kusakabe H, Iwasaki H, Sano K, Kiyokane K
Arch Dermatol Res 2000 Jun;292(6):292-300. doi: 10.1007/s004030000127. PMID: 10929770

Therapy

Pharmacology and pharmacokinetics of tazemetostat.
Orleni M, Beumer JH
Cancer Chemother Pharmacol 2024 May;93(5):509-517. Epub 2024 Mar 23 doi: 10.1007/s00280-024-04658-4. PMID: 38520556Free PMC Article
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
Tazemetostat for advanced epithelioid sarcoma: current status and future perspectives.
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
Tazemetostat in advanced epithelioid sarcoma with loss of INI1/SMARCB1: an international, open-label, phase 2 basket study.
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
Targeting epigenetics in sarcomas through EZH2 inhibition.
Italiano A
J Hematol Oncol 2020 Apr 7;13(1):33. doi: 10.1186/s13045-020-00868-4. PMID: 32264965Free PMC Article

Prognosis

Tazemetostat for advanced epithelioid sarcoma: current status and future perspectives.
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
Role of palliative chemotherapy in advanced epithelioid sarcoma.
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

Clinical prediction guides

Tazemetostat for advanced epithelioid sarcoma: current status and future perspectives.
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
Tazemetostat in advanced epithelioid sarcoma with loss of INI1/SMARCB1: an international, open-label, phase 2 basket study.
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
Expression of lung resistance protein in epithelioid sarcoma in vitro and in vivo.
Kusakabe H, Iwasaki H, Sano K, Kiyokane K
Arch Dermatol Res 2000 Jun;292(6):292-300. doi: 10.1007/s004030000127. PMID: 10929770

Supplemental Content

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