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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

Rasic P, Jeremic M, Jeremic R, Dusanovic Pjevic M, Rasic M, Djuricic SM, Milickovic M, Vukadin M, Mijovic T, Savic D
Molecules 2023 Apr 11;28(8) doi: 10.3390/molecules28083356. PMID: 37110590Free PMC Article
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
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

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

Peng J, Han XM
Medicine (Baltimore) 2022 Jul 22;101(29):e29564. doi: 10.1097/MD.0000000000029564. PMID: 35866758Free PMC Article
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
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

Peng J, Han XM
Medicine (Baltimore) 2022 Jul 22;101(29):e29564. doi: 10.1097/MD.0000000000029564. PMID: 35866758Free PMC Article
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
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
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

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

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