ArticleSkeletal radiology2021
Imaging features and clinical course of undifferentiated round cell sarcomas with CIC-DUX4 and BCOR-CCNB3 translocations.
Article in Skeletal radiology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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Who cites it
16 citing papers in PubMed, 29 citations in OpenAlex.
- CIC-rearranged sarcoma of the arm presenting as a soft tissue mass with regional lymphadenopathy and tumor thrombus.Skeletal radiology · 2026Article
- Dural CIC::DUX4 sarcoma presenting with intracranial hemorrhage at both initial diagnosis and recurrence: a case report and literature review.Brain tumor pathology · 2026Article
- Imaging of ewing sarcoma: an updated analysis including presenting features, prognostic imaging biomarkers, and treatment response assessment.Skeletal radiology · 2026Review
- Article
- Mesenchymal Nonmeningothelial Tumors of the CNS: Evolving Molecular Landscape and Implications for Neuroradiologists.AJNR. American journal of neuroradiology · 2025Review
- Update of pediatric bone tumors-other mesenchymal tumors of bone, hematopoietic neoplasms of bone, and WHO classification of undifferentiated small round cell sarcomas of bone.Skeletal radiology · 2023Review
- Rare Case of BCOR::CCNB3 Sarcoma of Bone.The American journal of case reports · 2023Article
- Article
- A Rare Case of Nasal Sarcoma with BCOR Internal Tandem Duplication Showing Complete Pathologic Response to the VDC-IE Chemotherapy Protocol.Case reports in oncological medicine · 2023Article
- Primary retroperitoneal perirenal CIC rearrangement sarcoma: A case report.Oncology letters · 2022Article
- Intramedullary, periosteal, and extraskeletal Ewing sarcomas: retrospective study of a series of 126 cases in a reference center.Skeletal radiology · 2022Article
- Systemic treatments and outcomes in CIC-rearranged Sarcoma: A national multi-centre clinicopathological series and literature review.Cancer medicine · 2022Review
- Article
- Review
- Imaging of bone and soft tissue BCOR-rearranged sarcoma.Skeletal radiology · 2021Review
- Ewing Sarcoma-Diagnosis, Treatment, Clinical Challenges and Future Perspectives.Journal of clinical medicine · 2021Review
Corrections and comments
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Authors and funding
4 authors at 2 institutions in 1 country.
Funding
Abstract
objectiveTo describe the pre-treatment imaging features and clinical course of undifferentiated round cell sarcomas with CIC-DUX4 and BCOR-CCNB3 translocations. MATERIALS AND
methodsIn this retrospective study, several pre-treatment imaging features (tumor location, size, enhancement pattern, necrosis, flow voids, calcification, and FDG avidity) and the clinical course of patients were evaluated.
resultsIn 12 patients with CIC-DUX4 sarcomas (median age, 24 years; range, 12-75), sarcomas were located in the soft tissue (n = 10), bone (n = 1), and lungs (n = 1). On MRI, all 10 CIC-DUX4 sarcomas presented as a large necrotic mass (mean size 6.7 cm, range 2.3-11.3) with 100% demonstrating contrast enhancement, 60% showing flow voids, and 20% demonstrating fluid-fluid levels. On PET, the mean SUVmax was 13.2 (range, 8.5-18.1). Among 12 patients with follow-up, 3 died within a year of diagnosis. The most common site of metastases was the lungs (5/12). In 5 patients with BCOR-CCNB3 sarcomas (median age, 14 years; range, 2-17), sarcomas were located in the spine (n = 2), femur (n = 1), tibia (n = 1), and pelvis (n = 1). On radiograph or CT, 2 were lytic, 3 were sclerotic. Soft tissue calcifications occurred in 40% of BCOR-CCNB3 sarcomas. On MRI, all 3 BCOR-CCNB3 tumors enhanced with 33% demonstrating flow voids and 66% exhibiting necrosis. On PET, the mean SUVmax was 6.3 (range 5.7-6.9).
conclusionCIC-DUX4 sarcomas often present as necrotic and hypermetabolic soft tissue masses while sarcomas with BCOR-CCNB3 translocations are vascular bone lesions with necrosis at imaging. CIC-DUX4 sarcomas are clinically more aggressive than BCOR-CCNB3 sarcomas.
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