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EWSR1-negative small round cell tumor

MedGen UID:
1714743
Concept ID:
C5237359
Neoplastic Process
Synonyms: EWSR1-Negative Small Blue Round Cell Tumor; EWSR1-Negative Small Cell Sarcoma; EWSR1-Negative Small Round Cell Tumor
 
Monarch Initiative: MONDO:0858921

Definition

A high-grade sarcoma characterized by the absence of EWSR1 rearrangement and the presence of small round malignant cells with a small amount of cytoplasm. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEWSR1-negative small round cell tumor

Recent clinical studies

Therapy

Italiano A, Sung YS, Zhang L, Singer S, Maki RG, Coindre JM, Antonescu CR
Genes Chromosomes Cancer 2012 Mar;51(3):207-18. Epub 2011 Nov 10 doi: 10.1002/gcc.20945. PMID: 22072439Free PMC Article

Prognosis

Logan SJ, Dehner CA, Alruwaii FI, Din NU, Olson DR, Fritchie KJ, Charville GW, Blessing MM, Folpe AL
Hum Pathol 2024 Jul;149:10-20. Epub 2024 May 21 doi: 10.1016/j.humpath.2024.05.007. PMID: 38782103
Soffer S, Amitai MM, Weizen R, Retter A, Barshack I, Shimon O, Davidson T, Klang E
Nucl Med Commun 2019 Aug;40(8):827-834. doi: 10.1097/MNM.0000000000001031. PMID: 31107830
Antonescu CR, Owosho AA, Zhang L, Chen S, Deniz K, Huryn JM, Kao YC, Huang SC, Singer S, Tap W, Schaefer IM, Fletcher CD
Am J Surg Pathol 2017 Jul;41(7):941-949. doi: 10.1097/PAS.0000000000000846. PMID: 28346326Free PMC Article
Tardío JC, Machado I, Navarro L, Idrovo F, Sanz-Ortega J, Pellín A, Llombart-Bosch A
Pathol Res Pract 2015 Nov;211(11):877-82. Epub 2015 Sep 8 doi: 10.1016/j.prp.2015.08.003. PMID: 26386605

Clinical prediction guides

Logan SJ, Dehner CA, Alruwaii FI, Din NU, Olson DR, Fritchie KJ, Charville GW, Blessing MM, Folpe AL
Hum Pathol 2024 Jul;149:10-20. Epub 2024 May 21 doi: 10.1016/j.humpath.2024.05.007. PMID: 38782103

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