Ewing family tumours: a paediatric perspective |
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| Affiliation: | 1. Wolfson Wohl Cancer Research Centre, Institute of Cancer Sciences, University of Glasgow, Garscube Estate, Switchback Road, Bearsden, Glasgow, Scotland G61 1BD, United Kingdom;2. West of Scotland Pancreatic Unit, Glasgow Royal Infirmary, Glasgow, Scotland G4 0SF, United Kingdom;3. The Kinghorn Cancer Centre, Cancer Division, Garvan Institute of Medical Research, 370 Victoria Street, Darlinghurst, Sydney, NSW 2010, Australia;4. St Vincent''s Clinical School, Faculty of Medicine, University of NSW, Australia;5. Department of Surgery, Bankstown Hospital, Eldridge Road, Bankstown, Sydney, NSW 2200, Australia;6. South Western Sydney Clinical School, Faculty of Medicine, University of NSW, Liverpool, NSW 2170, Australia;7. Queensland Centre for Medical Genomics, Institute for Molecular Bioscience, The University of Queensland, St Lucia, Brisbane, QLD, Australia;1. Shanghai Key Laboratory of Crime Scene Evidence, Shanghai Research Institute of Criminal Science and Technology, Shanghai 200083, China;2. Key Laboratory of Forensic Evidence and Science Technology, Ministry of Public Security, Institute of Forensic Science, Shanghai Public Security Bureau, Shanghai 200083, China;3. State Key Laboratory of Genetic Engineering and MOE Key Laboratory of Contemporary Anthropology, Institute of Genetics, School of Life Sciences, Fudan University, Shanghai 200438, China;4. Shanghai Center for Bioinformation Technology, Shanghai Academy of Science and Technology, Shanghai 201203, China;1. University of Pittsburgh Cancer Institute, Pittsburgh, PA, USA;2. Department of Pathology, Pittsburgh, PA, USA;3. Department of Immunology, Pittsburgh, PA, USA;4. Rush University Medical Center, Department of Neurosurgery, Chicago, IL, USA |
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| Abstract: | Sarcomas are malignant tumours of the connective tissues and are proportionately much more common in children than in adults. The Ewing family of tumours (EFT) is a group of sarcomas sharing rearrangement of the EWSR1 gene on 22q12, and include Ewing sarcoma/primitive neuroectodermal tumour, desmoplastic small round cell tumour, angiomatoid fibrous histiocytoma and clear cell sarcoma. Other tumours harbouring EWSR1 rearrangements include myoepithelial tumours, myxoid liposarcoma and extraskeletal chondrosarcoma. In addition, a group of Ewing-like primitive round cell sarcomas have been recently described in a paediatric population, further expanding the list of EFT. This review will focus on the histopathological, immunohistochemical and molecular genetic features of EFT, with an emphasis on those predominantly occurring in the paediatric population. |
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| Keywords: | angiomatoid fibrous histiocytoma clear cell sarcoma desmoplastic small round cell tumour Ewing sarcoma paediatric |
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