Ewing Sarcoma, Desmoplastic Small Round Cell Tumor, and Other Round Cell Sarcomas - 01/07/25

Doi : 10.1016/j.hoc.2025.04.003

M. Jane Underdown, MD ^a,^{^{1
Present address: 4509 S Loren Von Drive, Millcreek, UT 841242.}}, Sarah Kappa, MD ^b,^{^{2
Present address: 4115 Drummond Street, Houston, TX 770253.}}, Jonathan Metts, MD ^c,^d,^{^{3
Present address: 2977 Exeter Drive, Clearwater, FL 33761.}}, Thomas Cash, MD ^e,^⁎
^a Department of Pediatrics, Division of Pediatric Hematology/Oncology/BMT, University of Utah Health, Salt Lake City, UT, USA
^b Department of Pediatrics, Texas Children’s Cancer and Hematology Centers, Baylor College of Medicine, Houston, TX, USA
^c Sarcoma Department, Moffitt Cancer Center, Tampa, FL, USA
^d Cancer and Blood Disorders Institute, Johns Hopkins All Children’s Hospital, St Petersburg, FL, USA
^e Department of Pediatrics, Aflac Cancer and Blood Disorders Center, Children’s Healthcare of Atlanta, Emory University School of Medicine, Atlanta, GA, USA

^∗Corresponding author. Children’s Healthcare of Atlanta – Arthur M. Blank Hospital, South Tower, 14th Floor, CL.14124, 2220 North Druid Hills Road, Atlanta, GA 30329.Children’s Healthcare of Atlanta – Arthur M. Blank HospitalSouth Tower14th FloorCL.141242220 North Druid Hills RoadAtlantaGA30329

Résumé

The classification of small round cell sarcomas encompasses a spectrum of diseases ranging from those that are more widely understood, such as Ewing sarcoma and desmoplastic small round cell tumor, to more newly characterized diseases such as capicua (CIC)-rearranged sarcomas, BCOR-altered sarcomas, and sarcomas with EWSR1-non-ETS fusions. This article provides background, unique molecular features, and current and emerging therapies for each round cell sarcoma subtype.

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Keywords : Ewing sarcoma, Ewing-like sarcoma, Small round blue cell tumor, Round cell sarcomas, Desmoplastic small round cell tumor, CIC-DUX4, BCOR, EWSR1

Plan

Recurrent/Refractory Disease

Emerging Therapies and Future Directions

Other Round Cell Sarcomas

Desmoplastic Small Round Cell Tumor

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Vol 39 - N° 4

P. 709-726 - août 2025 Retour au numéro

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Ewing Sarcoma, Desmoplastic Small Round Cell Tumor, and Other Round Cell Sarcomas - 01/07/25

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