Imaging of bone and soft tissue BCOR-rearranged sarcoma

With recent advances in molecular research, an ever-increasing number of undifferentiated round cell sarcomas without the characteristic gene fusions of Ewing sarcoma are being discovered. One specific subtype termed BCOR-rearranged sarcoma belongs to this group. Previously termed ‘Ewing-like’ sarco...

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Bibliographic Details
Published in:Skeletal radiology 2021-07, Vol.50 (7), p.1291-1301
Main Authors: Sirisena, Udawattage Darshana Nadeeka, Rajakulasingam, Ramanan, Saifuddin, Asif
Format: Article
Language:English
Subjects:
Bone imaging
Bone lesions
Bone mass
Bone tumors
Classification
Demography
Ewing's sarcoma
Ewings sarcoma
Imaging
Medicine
Medicine & Public Health
Nuclear Medicine
Orthopedics
Pathology
Radiology
Review Article
Sarcoma
Soft tissues
Tumors
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Summary:With recent advances in molecular research, an ever-increasing number of undifferentiated round cell sarcomas without the characteristic gene fusions of Ewing sarcoma are being discovered. One specific subtype termed BCOR-rearranged sarcoma belongs to this group. Previously termed ‘Ewing-like’ sarcoma, it was formally included with undifferentiated round cell tumours in the 2013 WHO Classification of Soft Tissue and Bone Tumours. However, in the 2020 WHO Classification, BCOR-sarcoma is now recognized as a distinct entity due to particular morphological and immunohistochemical features and differing clinical outcomes. As with classical Ewing sarcoma, osseous BCOR-rearranged sarcoma is an aggressive tumour with a similar clinical presentation. However, there are only a small handful of case series and isolated reports detailing the imaging characteristics, typically demonstrating an aggressive bone lesion with a large soft tissue mass. Soft tissue BCOR-sarcoma is even rarer. The aim of the current review is to describe the patient demographics, lesion locations and various imaging characteristics of histologically proven cases of musculoskeletal bone and soft tissue BCOR-sarcoma as described in the literature.
ISSN:0364-2348
1432-2161
DOI:10.1007/s00256-020-03683-7