U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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

Sarcoma treatment in the era of molecular medicine.
Grünewald TG, Alonso M, Avnet S, Banito A, Burdach S, Cidre-Aranaz F, Di Pompo G, Distel M, Dorado-Garcia H, Garcia-Castro J, González-González L, Grigoriadis AE, Kasan M, Koelsche C, Krumbholz M, Lecanda F, Lemma S, Longo DL, Madrigal-Esquivel C, Morales-Molina Á, Musa J, Ohmura S, Ory B, Pereira-Silva M, Perut F, Rodriguez R, Seeling C, Al Shaaili N, Shaabani S, Shiavone K, Sinha S, Tomazou EM, Trautmann M, Vela M, Versleijen-Jonkers YM, Visgauss J, Zalacain M, Schober SJ, Lissat A, English WR, Baldini N, Heymann D
EMBO Mol Med 2020 Nov 6;12(11):e11131. Epub 2020 Oct 13 doi: 10.15252/emmm.201911131. PMID: 33047515Free 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
Pathogenesis, diagnosis, and management of cholangiocarcinoma.
Ilyas SI, Gores GJ
Gastroenterology 2013 Dec;145(6):1215-29. Epub 2013 Oct 15 doi: 10.1053/j.gastro.2013.10.013. PMID: 24140396Free PMC Article

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: 38520556
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

Table of contents

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...