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EWSR1-SMAD3fibroblastic tumour of bone: expanding the clinical spectrum

De Noon, S; Flanagan, AM; Tirabosco, R; O'Donnell, P; Amary, F; (2020) EWSR1-SMAD3fibroblastic tumour of bone: expanding the clinical spectrum. Skeletal Radiology 10.1007/s00256-020-03563-0. (In press). Green open access

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Abstract

EWSR1-SMAD3 fibroblastic tumour is a recently described soft tissue lesion. To date, eight cases have been reported, all sited in superficial soft tissue, typically occurring in the hands and feet with a tendency for local recurrence if incompletely excised. No metastatic spread has been reported, and hence, these tumours are currently considered benign. Herein, we present the radiological and histological features of the first reported occurrence of this entity in bone: a 44-year-old man with a tumour in the right tibia, treated with en bloc resection and showing no signs of relapse at 7 years. This tumour should be added to the differential diagnosis of bone lesions which harbour EWSR1 gene rearrangement.

Type: Article
Title: EWSR1-SMAD3fibroblastic tumour of bone: expanding the clinical spectrum
Open access status: An open access version is available from UCL Discovery
DOI: 10.1007/s00256-020-03563-0
Publisher version: https://doi.org/10.1007/s00256-020-03563-0
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher's terms and conditions.
Keywords: EWSR1, EWSR1-SMAD3, Bone tumour, Tumour, Sarcoma
UCL classification: UCL
UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Cancer Institute
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Cancer Institute > Research Department of Pathology
URI: https://discovery.ucl.ac.uk/id/eprint/10110325
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