U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Epithelioid sarcoma

MedGen UID:
104753
•Concept ID:
C0205944
•
Neoplastic Process
Synonyms: Epithelioid Sarcoma; Epithelioid Sarcomas; Sarcoma, Epithelioid; Sarcomas, Epithelioid
SNOMED CT: Epithelioid sarcoma (782827000); Epithelioid sarcoma (59238007); Epithelioid cell sarcoma (59238007)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0017387
Orphanet: ORPHA293202

Definition

Epithelioid sarcoma is a rare, soft tissue tumor characterized by high incidence of local recurrence, regional lymph node involvement and distant metastases. It commonly affects the soft tissue under the skin of a finger, hand, forearm, lower leg or foot, less often other areas of the body. [from ORDO]

Professional guidelines

PubMed

The evolving management of epithelioid sarcoma.
Alves A, Constantinidou A, Thway K, Fisher C, Huang P, Jones RL
Eur J Cancer Care (Engl) 2021 Nov;30(6):e13489. Epub 2021 Jul 18 doi: 10.1111/ecc.13489. PMID: 34278633Free PMC Article
Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 1.
Chokoeva AA, Tchernev G, Cardoso JC, Patterson JW, Dechev I, Valkanov S, Zanardelli M, Lotti T, Wollina U
Int J Immunopathol Pharmacol 2015 Jun;28(2):168-77. Epub 2015 Mar 26 doi: 10.1177/0394632015576029. PMID: 25816394
Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 2.
Chokoeva AA, Tchernev G, Cardoso JC, Patterson JW, Dechev I, Valkanov S, Zanardelli M, Lotti T, Wollina U
Int J Immunopathol Pharmacol 2015 Jun;28(2):178-86. Epub 2015 Mar 26 doi: 10.1177/0394632015575977. PMID: 25816393

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

Recent clinical studies

Etiology

Translational Aspects of Epithelioid Sarcoma: Current Consensus.
Grünewald TGP, Postel-Vinay S, Nakayama RT, Berlow NE, Bolzicco A, Cerullo V, Dermawan JK, Frezza AM, Italiano A, Jin JX, Le Loarer F, Martin-Broto J, Pecora A, Perez-Martinez A, Tam YB, Tirode F, Trama A, Pasquali S, Vescia M, Wortmann L, Wortmann M, Yoshida A, Webb K, Huang PH, Keller C, Antonescu CR
Clin Cancer Res 2024 Mar 15;30(6):1079-1092. doi: 10.1158/1078-0432.CCR-23-2174. PMID: 37916971Free PMC Article
Tazemetostat for tumors harboring SMARCB1/SMARCA4 or EZH2 alterations: results from NCI-COG pediatric MATCH APEC1621C.
Chi SN, Yi JS, Williams PM, Roy-Chowdhuri S, Patton DR, Coffey BD, Reid JM, Piao J, Saguilig L, Alonzo TA, Berg SL, Ramirez NC, Jaju A, Mhlanga JC, Fox E, Hawkins DS, Mooney MM, Takebe N, Tricoli JV, Janeway KA, Seibel NL, Parsons DW
J Natl Cancer Inst 2023 Nov 8;115(11):1355-1363. doi: 10.1093/jnci/djad085. PMID: 37228094Free PMC Article
Soft-tissue sarcoma in adolescents and young adults.
Kunisada T, Nakata E, Fujiwara T, Hosono A, Takihira S, Kondo H, Ozaki T
Int J Clin Oncol 2023 Jan;28(1):1-11. Epub 2022 Jan 27 doi: 10.1007/s10147-022-02119-7. PMID: 35084598
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

Diagnosis

SWI/SNF-deficient thoraco-pulmonary neoplasms.
Sesboue C, Le Loarer F
Semin Diagn Pathol 2021 May;38(3):183-194. Epub 2021 Jan 12 doi: 10.1053/j.semdp.2020.12.002. PMID: 33451916
The epigenomics of sarcoma.
Nacev BA, Jones KB, Intlekofer AM, Yu JSE, Allis CD, Tap WD, Ladanyi M, Nielsen TO
Nat Rev Cancer 2020 Oct;20(10):608-623. Epub 2020 Aug 11 doi: 10.1038/s41568-020-0288-4. PMID: 32782366Free PMC Article
Pseudomyogenic Hemangioendothelioma.
Ansai SI, Morimoto M, Akaishi S
J Nippon Med Sch 2019;86(2):126-130. doi: 10.1272/jnms.JNMS.2019_86-209. PMID: 31130564
Pseudomyogenic Hemangioendothelioma.
Al-Qaderi A, Mansour AT
Arch Pathol Lab Med 2019 Jun;143(6):763-767. Epub 2018 Dec 21 doi: 10.5858/arpa.2017-0430-RS. PMID: 30576238
Pseudomyogenic hemangioendothelioma.
Horan NA, DiMaio DJ
Cutis 2017 Dec;100(6):E13-E16. PMID: 29360904

Therapy

Tazemetostat for tumors harboring SMARCB1/SMARCA4 or EZH2 alterations: results from NCI-COG pediatric MATCH APEC1621C.
Chi SN, Yi JS, Williams PM, Roy-Chowdhuri S, Patton DR, Coffey BD, Reid JM, Piao J, Saguilig L, Alonzo TA, Berg SL, Ramirez NC, Jaju A, Mhlanga JC, Fox E, Hawkins DS, Mooney MM, Takebe N, Tricoli JV, Janeway KA, Seibel NL, Parsons DW
J Natl Cancer Inst 2023 Nov 8;115(11):1355-1363. doi: 10.1093/jnci/djad085. PMID: 37228094Free 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 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
Epigenetic Therapy for Epithelioid Sarcoma.
Rothbart SB, Baylin SB
Cell 2020 Apr 16;181(2):211. doi: 10.1016/j.cell.2020.03.042. PMID: 32302562
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

Translational Aspects of Epithelioid Sarcoma: Current Consensus.
Grünewald TGP, Postel-Vinay S, Nakayama RT, Berlow NE, Bolzicco A, Cerullo V, Dermawan JK, Frezza AM, Italiano A, Jin JX, Le Loarer F, Martin-Broto J, Pecora A, Perez-Martinez A, Tam YB, Tirode F, Trama A, Pasquali S, Vescia M, Wortmann L, Wortmann M, Yoshida A, Webb K, Huang PH, Keller C, Antonescu CR
Clin Cancer Res 2024 Mar 15;30(6):1079-1092. doi: 10.1158/1078-0432.CCR-23-2174. PMID: 37916971Free PMC Article
Soft-tissue sarcoma in adolescents and young adults.
Kunisada T, Nakata E, Fujiwara T, Hosono A, Takihira S, Kondo H, Ozaki T
Int J Clin Oncol 2023 Jan;28(1):1-11. Epub 2022 Jan 27 doi: 10.1007/s10147-022-02119-7. PMID: 35084598
Pseudomyogenic Hemangioendothelioma (Epithelioid Sarcoma-Like Hemangioendothelioma).
Caballero GA, Roitman PD
Arch Pathol Lab Med 2020 Apr;144(4):529-533. Epub 2019 Apr 24 doi: 10.5858/arpa.2018-0395-RS. PMID: 31017450
Pseudomyogenic Hemangioendothelioma.
Al-Qaderi A, Mansour AT
Arch Pathol Lab Med 2019 Jun;143(6):763-767. Epub 2018 Dec 21 doi: 10.5858/arpa.2017-0430-RS. PMID: 30576238
Epithelioid Sarcoma: Diagnostic Features and Genetics.
Thway K, Jones RL, Noujaim J, Fisher C
Adv Anat Pathol 2016 Jan;23(1):41-9. doi: 10.1097/PAP.0000000000000102. PMID: 26645461

Clinical prediction guides

Tazemetostat for tumors harboring SMARCB1/SMARCA4 or EZH2 alterations: results from NCI-COG pediatric MATCH APEC1621C.
Chi SN, Yi JS, Williams PM, Roy-Chowdhuri S, Patton DR, Coffey BD, Reid JM, Piao J, Saguilig L, Alonzo TA, Berg SL, Ramirez NC, Jaju A, Mhlanga JC, Fox E, Hawkins DS, Mooney MM, Takebe N, Tricoli JV, Janeway KA, Seibel NL, Parsons DW
J Natl Cancer Inst 2023 Nov 8;115(11):1355-1363. doi: 10.1093/jnci/djad085. PMID: 37228094Free PMC Article
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
SWI/SNF complex-deficient soft tissue neoplasms: An update.
Schaefer IM, Hornick JL
Semin Diagn Pathol 2021 May;38(3):222-231. Epub 2020 Jun 5 doi: 10.1053/j.semdp.2020.05.005. PMID: 32646614Free PMC Article
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
Challenging epithelioid mesenchymal neoplasms: mimics and traps.
Cacciatore M, Dei Tos AP
Pathology 2014 Feb;46(2):126-34. doi: 10.1097/PAT.0000000000000063. PMID: 24384587

Recent systematic reviews

Role of Fine Needle Aspiration Cytology in Paratesticular Neoplasms-A Case Reports-Based Systematic Review.
Nigam JS, Bharti JN, Pradeep I, Rath A
Diagn Cytopathol 2024 Dec;52(12):779-788. Epub 2024 Sep 19 doi: 10.1002/dc.25404. PMID: 39295546
Systematic Review of Published Cases of Primary Epithelioid Sarcoma of the Spine.
Hu W, Wu X, Ma H, Wang H, Shi X, Zhang K, Gao Y
Med Sci Monit 2023 Jan 6;29:e938416. doi: 10.12659/MSM.938416. PMID: 36604864Free PMC Article
Targeting Enhancer of Zeste Homolog 2 for the Treatment of Hematological Malignancies and Solid Tumors: Candidate Structure-Activity Relationships Insights and Evolution Prospects.
Xia J, Li J, Tian L, Ren X, Liu C, Liang C
J Med Chem 2022 May 26;65(10):7016-7043. Epub 2022 May 7 doi: 10.1021/acs.jmedchem.2c00047. PMID: 35531606
Pseudomyogenic hemangioendothelioma with bone and soft tissue involvement with favorable response to pamidronate: a case report and systematic review of the literature.
Brance ML, Cóccaro NM, Roitman P, Castiglioni A, Agostinis F, Spense M, Scheitlin B, Rene N, Brun LR
Arch Osteoporos 2022 Feb 1;17(1):28. doi: 10.1007/s11657-022-01062-4. PMID: 35106633
Epithelioid sarcoma of the hand: a wolf in sheep's clothing.
Persitz J, Beit Ner E, Chechik I, Keren T, Avisar E
J Plast Surg Hand Surg 2021 Apr;55(2):96-104. Epub 2020 Nov 11 doi: 10.1080/2000656X.2020.1838914. PMID: 33176534

Supplemental Content

Table of contents

    Clinical resources

    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.

    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

    Reviews

    Related information

    • MeSH
      Related Medical Subject Headings
    • 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...