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Extraskeletal Ewing sarcoma/peripheral primitive neuroectodermal tumor

MedGen UID:
232170
Concept ID:
C1333514
Neoplastic Process
Synonyms: Extraosseous Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; extraosseous Ewing sarcoma/peripheral primitive neuroectodermal tumor; extraosseous Ewing sarcoma/peripheral primitive neuroectodermal tumour; Extraosseous Ewing's Sarcoma/Peripheral Primitive Neuroectodermal Tumor; extraosseous Ewing's sarcoma/peripheral primitive neuroectodermal tumor; extraosseous Ewing's sarcoma/peripheral primitive neuroectodermal tumour; extraosseous Ewing's tumor; extraosseous Ewing's tumour; extraosseous Ewings sarcoma-primitive neuroepithelial tumor; extraosseous Ewings sarcoma-primitive neuroepithelial tumour; Extraskeletal Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; extraskeletal Ewing sarcoma/peripheral primitive neuroectodermal tumor
 
Monarch Initiative: MONDO:0021039

Definition

A spectrum of malignant tumors arising from the soft tissues, 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. Pain and the presence of a mass are the most common clinical symptoms. [from NCI]

Term Hierarchy

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

Professional guidelines

PubMed

Javery O, Krajewski K, O'Regan K, Kis B, Giardino A, Jagannathan J, Ramaiya NH
AJR Am J Roentgenol 2011 Dec;197(6):W1015-22. doi: 10.2214/AJR.11.6667. PMID: 22109315
Miettinen M, Chatten J, Paetau A, Stevenson A
Am J Surg Pathol 1998 Mar;22(3):327-32. doi: 10.1097/00000478-199803000-00007. PMID: 9500774

Recent clinical studies

Etiology

Bae SH, Hwang JH, Da Nam B, Kim HJ, Kim KU, Kim DW, Choi IH
Medicine (Baltimore) 2016 Feb;95(7):e2725. doi: 10.1097/MD.0000000000002725. PMID: 26886614Free PMC Article
Applebaum MA, Goldsby R, Neuhaus J, DuBois SG
Pediatr Blood Cancer 2012 Oct;59(4):617-20. Epub 2011 Dec 19 doi: 10.1002/pbc.24053. PMID: 22184129Free PMC Article
Applebaum MA, Worch J, Matthay KK, Goldsby R, Neuhaus J, West DC, Dubois SG
Cancer 2011 Jul 1;117(13):3027-32. Epub 2011 Jan 10 doi: 10.1002/cncr.25840. PMID: 21692057Free PMC Article
Devoe K, Weidner N
Semin Diagn Pathol 2000 Aug;17(3):216-24. PMID: 10968707
Siebenrock KA, Nascimento AG, Rock MG
Clin Orthop Relat Res 1996 Aug;(329):288-99. doi: 10.1097/00003086-199608000-00036. PMID: 8769464

Diagnosis

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
Khwaja R, Mantilla E, Fink K, Pan E
Anticancer Res 2019 Aug;39(8):4463-4465. doi: 10.21873/anticanres.13619. PMID: 31366545
Bae SH, Hwang JH, Da Nam B, Kim HJ, Kim KU, Kim DW, Choi IH
Medicine (Baltimore) 2016 Feb;95(7):e2725. doi: 10.1097/MD.0000000000002725. PMID: 26886614Free PMC Article
Applebaum MA, Worch J, Matthay KK, Goldsby R, Neuhaus J, West DC, Dubois SG
Cancer 2011 Jul 1;117(13):3027-32. Epub 2011 Jan 10 doi: 10.1002/cncr.25840. PMID: 21692057Free PMC Article
Devoe K, Weidner N
Semin Diagn Pathol 2000 Aug;17(3):216-24. PMID: 10968707

Therapy

Khwaja R, Mantilla E, Fink K, Pan E
Anticancer Res 2019 Aug;39(8):4463-4465. doi: 10.21873/anticanres.13619. PMID: 31366545
Siebenrock KA, Nascimento AG, Rock MG
Clin Orthop Relat Res 1996 Aug;(329):288-99. doi: 10.1097/00003086-199608000-00036. PMID: 8769464

Prognosis

Applebaum MA, Goldsby R, Neuhaus J, DuBois SG
Pediatr Blood Cancer 2012 Oct;59(4):617-20. Epub 2011 Dec 19 doi: 10.1002/pbc.24053. PMID: 22184129Free PMC Article
Halil S, Kucuk M, Arvas M, Aydin O, Calay ZZ
Eur J Gynaecol Oncol 2011;32(1):117-8. PMID: 21446342
Harimaya K, Oda Y, Matsuda S, Tanaka K, Chuman H, Iwamoto Y
Spine (Phila Pa 1976) 2003 Oct 1;28(19):E408-12. doi: 10.1097/01.BRS.0000085099.47800.DF. PMID: 14520055
Siebenrock KA, Nascimento AG, Rock MG
Clin Orthop Relat Res 1996 Aug;(329):288-99. doi: 10.1097/00003086-199608000-00036. PMID: 8769464
Ushigome S, Shimoda T, Takaki K, Nikaido T, Takakuwa T, Ishikawa E, Spjut HJ
Cancer 1989 Jul 1;64(1):52-62. doi: 10.1002/1097-0142(19890701)64:1<52::aid-cncr2820640110>3.0.co;2-6. PMID: 2543495

Clinical prediction guides

Funakoshi T, Sato T, Hosokawa R, Saito M, Amagai M, Ishiko A, Ohyama M
J Dermatol 2015 Sep;42(9):893-6. Epub 2015 May 11 doi: 10.1111/1346-8138.12930. PMID: 25959109
Applebaum MA, Goldsby R, Neuhaus J, DuBois SG
Pediatr Blood Cancer 2012 Oct;59(4):617-20. Epub 2011 Dec 19 doi: 10.1002/pbc.24053. PMID: 22184129Free PMC Article
Isefuku S, Seki M, Tajino T, Hakozaki M, Asano S, Hojo H, Hatori M
Tohoku J Exp Med 2006 Aug;209(4):369-77. doi: 10.1620/tjem.209.369. PMID: 16864960
Hornick JL, Fletcher CD
Am J Clin Pathol 2002 Feb;117(2):188-93. doi: 10.1309/LX9U-F7P0-UWDH-8Y6R. PMID: 11865845
Ushigome S, Shimoda T, Takaki K, Nikaido T, Takakuwa T, Ishikawa E, Spjut HJ
Cancer 1989 Jul 1;64(1):52-62. doi: 10.1002/1097-0142(19890701)64:1<52::aid-cncr2820640110>3.0.co;2-6. PMID: 2543495

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