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Round cell sarcoma with EWSR1-non-ETS fusion

MedGen UID:
1790023
•Concept ID:
C4727985
•
Neoplastic Process
Synonyms: Round Cell Sarcoma with EWSR1-non-ETS Fusion; round cell sarcoma with EWSR1-non-ETS fusion; Round cell sarcoma with EWSR1-non-ETS fusions; Round Cell Sarcoma with EWSR1::non-ETS Fusion
SNOMED CT: Round cell sarcoma with EWSR1-non-ETS fusions (1186898003)
 
Monarch Initiative: MONDO:0958160

Definition

Round cell sarcoma with EWSR1-non-ETS fusions are round and spindle cell sarcomas with fusions outside the ETS family of transcription factors. [from SNOMEDCT_US]

Recent clinical studies

Etiology

Bone and soft tissue tumors: clinicoradiologic-pathologic molecular-genetic correlation of novel fusion spindled, targetable-ovoid, giant-cell-rich, and round cell sarcomas.
Fanburg-Smith JC, Smith JD, Flemming DJ
Skeletal Radiol 2023 Mar;52(3):517-540. Epub 2022 Dec 21 doi: 10.1007/s00256-022-04244-w. PMID: 36542130
Ewing and Ewing-like sarcomas: A morphological guide through genetically-defined entities.
Yoshida A
Pathol Int 2023 Jan;73(1):12-26. Epub 2022 Dec 9 doi: 10.1111/pin.13293. PMID: 36484765Free PMC Article
Small round cell sarcomas.
Cidre-Aranaz F, Watson S, Amatruda JF, Nakamura T, Delattre O, de Alava E, Dirksen U, Grünewald TGP
Nat Rev Dis Primers 2022 Oct 6;8(1):66. doi: 10.1038/s41572-022-00393-3. PMID: 36202860
From the ashes of "Ewing-like" sarcoma: A contemporary update of the classification, immunohistochemistry, and molecular genetics of round cell sarcomas.
Kallen ME, Hornick JL
Semin Diagn Pathol 2022 Jan;39(1):29-37. Epub 2021 Oct 29 doi: 10.1053/j.semdp.2021.10.002. PMID: 34763921
Cytology of Undifferentiated Round-Cell Sarcomas of Bone and Soft Tissue: Ewing Sarcoma or Not Ewing Sarcoma, That Is the Question.
Gajdzis P, Pierron G, Klijanienko J
Acta Cytol 2022;66(4):295-306. Epub 2021 Aug 25 doi: 10.1159/000518146. PMID: 34515032

Diagnosis

Sarcomas with EWSR1::Non-ETS Fusion (EWSR1::NFATC2 and EWSR1::PATZ1).
Machado I, Llombart-Bosch A, Charville GW, Navarro S, Domínguez Franjo MP, Bridge JA, Linos K
Surg Pathol Clin 2024 Mar;17(1):31-55. Epub 2023 Aug 7 doi: 10.1016/j.path.2023.07.001. PMID: 38278606
Bone and soft tissue tumors: clinicoradiologic-pathologic molecular-genetic correlation of novel fusion spindled, targetable-ovoid, giant-cell-rich, and round cell sarcomas.
Fanburg-Smith JC, Smith JD, Flemming DJ
Skeletal Radiol 2023 Mar;52(3):517-540. Epub 2022 Dec 21 doi: 10.1007/s00256-022-04244-w. PMID: 36542130
Ewing and Ewing-like sarcomas: A morphological guide through genetically-defined entities.
Yoshida A
Pathol Int 2023 Jan;73(1):12-26. Epub 2022 Dec 9 doi: 10.1111/pin.13293. PMID: 36484765Free PMC Article
Small round cell sarcomas.
Cidre-Aranaz F, Watson S, Amatruda JF, Nakamura T, Delattre O, de Alava E, Dirksen U, Grünewald TGP
Nat Rev Dis Primers 2022 Oct 6;8(1):66. doi: 10.1038/s41572-022-00393-3. PMID: 36202860
Small Round Blue Cell Sarcoma Other Than Ewing Sarcoma: What Should an Oncologist Know?
Davis JL, Rudzinski ER
Curr Treat Options Oncol 2020 Sep 1;21(11):90. doi: 10.1007/s11864-020-00785-1. PMID: 32875423

Therapy

Undifferentiated sarcoma of bone with a round to epithelioid cell phenotype harboring a novel EWSR1-SSX2 fusion identified by RNA-based next-generation sequencing.
Cordier F, Van der Meulen J, Van Gaever B, Lapeire L, Sys G, Van Dorpe J, Creytens D
Genes Chromosomes Cancer 2022 Jan;61(1):44-49. Epub 2021 Sep 29 doi: 10.1002/gcc.22999. PMID: 34538011

Prognosis

Sarcomas with EWSR1::Non-ETS Fusion (EWSR1::NFATC2 and EWSR1::PATZ1).
Machado I, Llombart-Bosch A, Charville GW, Navarro S, Domínguez Franjo MP, Bridge JA, Linos K
Surg Pathol Clin 2024 Mar;17(1):31-55. Epub 2023 Aug 7 doi: 10.1016/j.path.2023.07.001. PMID: 38278606
Small round cell sarcomas.
Cidre-Aranaz F, Watson S, Amatruda JF, Nakamura T, Delattre O, de Alava E, Dirksen U, Grünewald TGP
Nat Rev Dis Primers 2022 Oct 6;8(1):66. doi: 10.1038/s41572-022-00393-3. PMID: 36202860
From the ashes of "Ewing-like" sarcoma: A contemporary update of the classification, immunohistochemistry, and molecular genetics of round cell sarcomas.
Kallen ME, Hornick JL
Semin Diagn Pathol 2022 Jan;39(1):29-37. Epub 2021 Oct 29 doi: 10.1053/j.semdp.2021.10.002. PMID: 34763921
The spectrum of rare central nervous system (CNS) tumors with EWSR1-non-ETS fusions: experience from three pediatric institutions with review of the literature.
Lopez-Nunez O, Cafferata B, Santi M, Ranganathan S, Pearce TM, Kulich SM, Bailey KM, Broniscer A, Rossi S, Zin A, Nasrallah MP, Li MM, Zhong Y, Miele E, Alaggio R, Surrey LF
Brain Pathol 2021 Jan;31(1):70-83. Epub 2020 Nov 6 doi: 10.1111/bpa.12900. PMID: 32997853Free PMC Article
Small Round Blue Cell Sarcoma Other Than Ewing Sarcoma: What Should an Oncologist Know?
Davis JL, Rudzinski ER
Curr Treat Options Oncol 2020 Sep 1;21(11):90. doi: 10.1007/s11864-020-00785-1. PMID: 32875423

Clinical prediction guides

Bone and soft tissue tumors: clinicoradiologic-pathologic molecular-genetic correlation of novel fusion spindled, targetable-ovoid, giant-cell-rich, and round cell sarcomas.
Fanburg-Smith JC, Smith JD, Flemming DJ
Skeletal Radiol 2023 Mar;52(3):517-540. Epub 2022 Dec 21 doi: 10.1007/s00256-022-04244-w. PMID: 36542130
The spectrum of rare central nervous system (CNS) tumors with EWSR1-non-ETS fusions: experience from three pediatric institutions with review of the literature.
Lopez-Nunez O, Cafferata B, Santi M, Ranganathan S, Pearce TM, Kulich SM, Bailey KM, Broniscer A, Rossi S, Zin A, Nasrallah MP, Li MM, Zhong Y, Miele E, Alaggio R, Surrey LF
Brain Pathol 2021 Jan;31(1):70-83. Epub 2020 Nov 6 doi: 10.1111/bpa.12900. PMID: 32997853Free PMC Article

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