U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Ewing sarcoma/peripheral primitive neuroectodermal tumor

MedGen UID:
760735
Concept ID:
C3536893
Neoplastic Process
Synonyms: EFTs; Ewing Family of Tumors; Ewing family of tumors; Ewing family of tumours; Ewing sarcoma family of tumors; Ewing sarcoma family of tumours; Ewing sarcoma/peripheral PNET; Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Ewing's Family of Tumors; Ewing's family of tumors; Ewing's Family of Tumours; Ewing's family of tumours; Ewing's Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Ewing's sarcoma/peripheral primitive neuroectodermal tumor; Ewing's sarcoma/peripheral primitive neuroectodermal tumour; Tumors of Ewing's Family; tumors of Ewing's family; Tumors of the Ewing's Family; tumors of the Ewing's family; tumours of Ewing's family; tumours of the Ewing's family
 
Monarch Initiative: MONDO:0021038

Definition

A spectrum of malignant tumors, affecting mostly males under age 20, characterized morphologically by the presence of small round cells. Ewing sarcoma and peripheral primitive neuroectodermal tumor represent the ends of a spectrum, with Ewing sarcoma lacking evidence of neural differentiation and the markers that characterize the peripheral primitive neuroectodermal tumor. Ewing sarcoma and peripheral primitive neuroectodermal tumor may share cytogenetic abnormalities, proto-oncogene expression, cell culture and immunohistochemical abnormalities. These tumors may occur in the soft tissues or the bones. Pain and the presence of a mass are the most common clinical symptoms. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEwing sarcoma/peripheral primitive neuroectodermal tumor

Professional guidelines

PubMed

Slotkin EK, Meyers PA
Pediatr Blood Cancer 2023 Jan;70(1):e30005. Epub 2022 Oct 2 doi: 10.1002/pbc.30005. PMID: 36184748Free PMC Article
Liu Y, Yuan Y, Zhang F, Hu K, Qiu J, Hou X, Yan J, Lian X, Sun S, Liu Z, Shen J
Sci Rep 2020 Sep 24;10(1):15656. doi: 10.1038/s41598-020-72680-6. PMID: 32973274Free PMC Article
Cherif El Asri A, Benzagmout M, Chakour K, Chaoui MF, Laaguili J, Chahdi H, Gazzaz M, El Mostarchid B
World Neurosurg 2018 Jul;115:346-356. Epub 2018 May 3 doi: 10.1016/j.wneu.2018.04.164. PMID: 29729469

Curated

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

Recent clinical studies

Etiology

Peng J, Han XM
Medicine (Baltimore) 2022 Jul 22;101(29):e29564. doi: 10.1097/MD.0000000000029564. PMID: 35866758Free PMC Article
Inagaki C, Shimoi T, Sumiyoshi Okuma H, Kawachi A, Sudo K, Shimomura A, Noguchi E, Kodaira M, Yunokawa M, Yonemori K, Shimizu C, Arakawa A, Ogawa C, Yoshida A, Fujiwara Y, Tamura K
Med Oncol 2019 May 18;36(7):58. doi: 10.1007/s12032-019-1279-8. PMID: 31104192
Warwick AB, Malempati S, Krailo M, Melemed A, Gorlick R, Ames MM, Safgren SL, Adamson PC, Blaney SM
Pediatr Blood Cancer 2013 Feb;60(2):237-41. Epub 2012 Jun 28 doi: 10.1002/pbc.24244. PMID: 22745043Free PMC Article
Llombart-Bosch A, Pellín A, Carda C, Noguera R, Navarro S, Peydró-Olaya A
Diagn Mol Pathol 2000 Sep;9(3):137-44. doi: 10.1097/00019606-200009000-00003. PMID: 10976720

Diagnosis

Deshpande G, Epari S, Gupta C, Shetty O, Gurav M, Chinnaswamy G, Moiyadi A, Gupta T
Childs Nerv Syst 2021 Mar;37(3):839-849. Epub 2020 Aug 6 doi: 10.1007/s00381-020-04850-w. PMID: 32761378
El Housheimi A, Khalil A, Khalifeh D, Berjawi G, Seoud M, Tabbarah A, Khaled C, Mahfouz R, Abdallah R
J Obstet Gynaecol Res 2020 Oct;46(10):2185-2192. Epub 2020 Aug 5 doi: 10.1111/jog.14399. PMID: 32761741
Goudarzipour K, Farahmandi F, Mohammadi A, Taherian R
Iran J Kidney Dis 2018 May;12(3):190-192. PMID: 29891750
Simons SA, Bridge JA, Leon ME
Semin Diagn Pathol 2016 Mar;33(2):91-103. Epub 2015 Sep 10 doi: 10.1053/j.semdp.2015.09.010. PMID: 26585346
Tsokos M, Alaggio RD, Dehner LP, Dickman PS
Pediatr Dev Pathol 2012;15(1 Suppl):108-26. doi: 10.2350/11-08-1078-PB.1. PMID: 22420726Free PMC Article

Therapy

Warwick AB, Malempati S, Krailo M, Melemed A, Gorlick R, Ames MM, Safgren SL, Adamson PC, Blaney SM
Pediatr Blood Cancer 2013 Feb;60(2):237-41. Epub 2012 Jun 28 doi: 10.1002/pbc.24244. PMID: 22745043Free PMC Article

Prognosis

Deshpande G, Epari S, Gupta C, Shetty O, Gurav M, Chinnaswamy G, Moiyadi A, Gupta T
Childs Nerv Syst 2021 Mar;37(3):839-849. Epub 2020 Aug 6 doi: 10.1007/s00381-020-04850-w. PMID: 32761378
Simons SA, Bridge JA, Leon ME
Semin Diagn Pathol 2016 Mar;33(2):91-103. Epub 2015 Sep 10 doi: 10.1053/j.semdp.2015.09.010. PMID: 26585346
VandenHeuvel KA, Al-Rohil RN, Stevenson ME, Qian J, Gross NL, McNall-Knapp R, Li S, Wartchow EP, Mierau GW, Fung KM
Int J Clin Exp Pathol 2015;8(1):260-74. Epub 2015 Jan 1 PMID: 25755713Free PMC Article
Tsokos M, Alaggio RD, Dehner LP, Dickman PS
Pediatr Dev Pathol 2012;15(1 Suppl):108-26. doi: 10.2350/11-08-1078-PB.1. PMID: 22420726Free PMC Article
dos Santos Rubio EJ, Harhangi BS, Kros JM, Vincent AJ, Dirven CM
Neurosurgery 2010 Dec;67(6):E1852-6. doi: 10.1227/NEU.0b013e3181f82569. PMID: 21107154

Clinical prediction guides

Simons SA, Bridge JA, Leon ME
Semin Diagn Pathol 2016 Mar;33(2):91-103. Epub 2015 Sep 10 doi: 10.1053/j.semdp.2015.09.010. PMID: 26585346
VandenHeuvel KA, Al-Rohil RN, Stevenson ME, Qian J, Gross NL, McNall-Knapp R, Li S, Wartchow EP, Mierau GW, Fung KM
Int J Clin Exp Pathol 2015;8(1):260-74. Epub 2015 Jan 1 PMID: 25755713Free PMC Article
Warwick AB, Malempati S, Krailo M, Melemed A, Gorlick R, Ames MM, Safgren SL, Adamson PC, Blaney SM
Pediatr Blood Cancer 2013 Feb;60(2):237-41. Epub 2012 Jun 28 doi: 10.1002/pbc.24244. PMID: 22745043Free PMC Article
Antunes NL, Lellouch-Tubiana A, Kalifa C, Delattre O, Pierre-Kahn A, Rosenblum MK
J Neurooncol 2001 Jan;51(1):51-6. doi: 10.1023/a:1006432919281. PMID: 11349881
Sinkre P, Albores-Saavedra J, Miller DS, Copeland LJ, Hameed A
Int J Gynecol Pathol 2000 Apr;19(2):127-32. doi: 10.1097/00004347-200004000-00005. PMID: 10782408

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

    Consumer resources

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...