Ewing sarcoma
Dernière mise à jour 8 Sept 2020
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Ewing sarcoma is a cancerous tumour that occurs in bones or soft tissues, such as cartilage or nerves. There are several types of Ewing sarcoma, including Ewing sarcoma of bone, extraosseous Ewing sarcoma, peripheral primitive neuroectodermal tumour (pPNET), and Askin tumour. These tumours are considered to be related because they have similar genetic causes. These types of Ewing sarcoma can be distinguished from one another by the tissue in which the tumour develops. Approximately 87 percent of Ewing sarcomas are Ewing sarcoma of bone, which is a bone tumour that usually occurs in the thigh bones (femurs), pelvis, ribs, or shoulder blades. Extraosseous (or extraskeletal) Ewing sarcoma describes tumours in the soft tissues around bones, such as cartilage. pPNETs occur in nerve tissue and can be found in many parts of the body. A type of pPNET found in the chest is called Askin tumour.
Description
Ewing sarcoma is a cancerous tumour that occurs in bones or soft tissues, such as cartilage or nerves. There are several types of Ewing sarcoma, including Ewing sarcoma of bone, extraosseous Ewing sarcoma, peripheral primitive neuroectodermal tumour (pPNET), and Askin tumour. These tumours are considered to be related because they have similar genetic causes. These types of Ewing sarcoma can be distinguished from one another by the tissue in which the tumour develops. Approximately 87 percent of Ewing sarcomas are Ewing sarcoma of bone, which is a bone tumour that usually occurs in the thigh bones (femurs), pelvis, ribs, or shoulder blades. Extraosseous (or extraskeletal) Ewing sarcoma describes tumours in the soft tissues around bones, such as cartilage. pPNETs occur in nerve tissue and can be found in many parts of the body. A type of pPNET found in the chest is called Askin tumour.
Ewing sarcomas most often occur in children and young adults. Affected individuals usually feel stiffness, pain, swelling, or tenderness of the bone or surrounding tissue. Sometimes, there is a lump near the surface of the skin that feels warm and soft to the touch. Often, children have a fever that does not go away. Ewing sarcoma of bone can cause weakening of the involved bone, and affected individuals may have a broken bone with no obvious cause.
It is common for Ewing sarcoma to spread to other parts of the body (metastasize), usually to the lungs, to other bones, or to the bone marrow.
Inheritance patterns
The source lists the following possible inheritance patterns: Not inherited. More than one pattern can apply in different cases.
Related genes
Other names
Ewing family of tumours
Ewing tumour
Ewing's sarcoma
Ewing's tumour
Tumour of the Ewing family
Related patient information and medicines
Patient information leaflet
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A sarcoma is a cancer that originates from cells which occur in and make supporting tissues (connective tissues) of the body - for example, bone, muscle, cartilage, ligaments, etc. Osteosarcoma This is the most common type of primary bone cancer but even this is rare. Howe... Ewing's sarcoma The cells of this cancer look different to the more.
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Lectures complémentaires et références
- Ewing sarcoma; MedlinePlus, National Library of Medicine.
- Bailly RA, Bosselut R, Zucman J, Cormier F, Delattre O, Roussel M, Thomas G, Ghysdael J. DNA-binding and transcriptional activation properties of the EWS-FLI-1 fusion protein resulting from the t(11;22) translocation in Ewing sarcoma. Mol Cell Biol. 1994 May;14(5):3230-41. doi: 10.1128/mcb.14.5.3230-3241.1994.
- Bertolotti A, Melot T, Acker J, Vigneron M, Delattre O, Tora L. EWS, but not EWS-FLI-1, is associated with both TFIID and RNA polymerase II: interactions between two members of the TET family, EWS and hTAFII68, and subunits of TFIID and RNA polymerase II complexes. Mol Cell Biol. 1998 Mar;18(3):1489-97. doi: 10.1128/MCB.18.3.1489.
- Kim SK, Park YK. Ewing sarcoma: a chronicle of molecular pathogenesis. Hum Pathol. 2016 Sep;55:91-100. doi: 10.1016/j.humpath.2016.05.008. Epub 2016 May 28.
- Mao X, Miesfeldt S, Yang H, Leiden JM, Thompson CB. The FLI-1 and chimeric EWS-FLI-1 oncoproteins display similar DNA binding specificities. J Biol Chem. 1994 Jul 8;269(27):18216-22.
- May WA, Gishizky ML, Lessnick SL, Lunsford LB, Lewis BC, Delattre O, Zucman J, Thomas G, Denny CT. Ewing sarcoma 11;22 translocation produces a chimeric transcription factor that requires the DNA-binding domain encoded by FLI1 for transformation. Proc Natl Acad Sci U S A. 1993 Jun 15;90(12):5752-6. doi: 10.1073/pnas.90.12.5752.
- May WA, Lessnick SL, Braun BS, Klemsz M, Lewis BC, Lunsford LB, Hromas R, Denny CT. The Ewing's sarcoma EWS/FLI-1 fusion gene encodes a more potent transcriptional activator and is a more powerful transforming gene than FLI-1. Mol Cell Biol. 1993 Dec;13(12):7393-8. doi: 10.1128/mcb.13.12.7393-7398.1993.
- Ohno T, Rao VN, Reddy ES. EWS/Fli-1 chimeric protein is a transcriptional activator. Cancer Res. 1993 Dec 15;53(24):5859-63.
- Sankar S, Lessnick SL. Promiscuous partnerships in Ewing's sarcoma. Cancer Genet. 2011 Jul;204(7):351-65. doi: 10.1016/j.cancergen.2011.07.008.
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