EWSR1-SMAD3 fibroblastic tumour of bone: expanding the clinical spectrum.

Solange De Noon, Adrienne M Flanagan, Roberto Tirabosco, Paul O'Donnell, Fernanda Amary

Skeletal radiology 2021 Feb

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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.

Citation

Solange De Noon, Adrienne M Flanagan, Roberto Tirabosco, Paul O'Donnell, Fernanda Amary. EWSR1-SMAD3 fibroblastic tumour of bone: expanding the clinical spectrum. Skeletal radiology. 2021 Feb;50(2):445-450