ArticleVirchows Archiv : an international journal of pathology2017
Histological and immunohistochemical characteristics of undifferentiated small round cell sarcomas associated with CIC-DUX4 and BCOR-CCNB3 fusion genes.
Article in Virchows Archiv : an international journal of pathology, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 34 papers.
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34 citing papers in PubMed, 101 citations in OpenAlex.
- Clinicopathological features of CIC-rearranged sarcomas: a retrospective study including novel visceral locations and methylation profile analysis.Virchows Archiv : an international journal of pathology · 2026Article
- Primary capicua transcriptional repressor-rearranged sarcoma in the thyroid gland with cervical lymph node and lung metastases: a case report and literature review.Discover oncology · 2025Article
- CIC-DUX4 Sarcoma of the Skin: A Rare Case Report and Literature Review.Journal of cutaneous pathology · 2025Review
- Primary ovarian CIC-rearranged sarcoma in a child: a rare case report and review of the literature.Frontiers in medicine · 2025Article
- Sarcomas with EWSR1::Non-ETS Fusion (EWSR1::NFATC2 and EWSR1::PATZ1).Surgical pathology clinics · 2024Review
- Clinical characteristics and outcomes for children, adolescents and young adults with "CIC-fused" or "BCOR-rearranged" soft tissue sarcomas: A multi-institutional European retrospective analysis.Cancer medicine · 2023Article
- Patterns of care and outcome of CIC-rearranged sarcoma patients: A nationwide study of the French sarcoma group.Cancer medicine · 2023Article
- 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
- Case Report: Colon malignant tumor caused by retroperitoneal small round cell undifferentiated sarcoma.Frontiers in oncology · 2023Article
- Ewing and Ewing-like sarcomas: A morphological guide through genetically-defined entities.Pathology international · 2023Review
- Small round cell sarcomas.Nature reviews. Disease primers · 2022Review
- BCOR-CCNB3 sarcoma with concurrent RNF213-SLC26A11 gene fusion: a rare sarcoma with altered histopathological features after chemotherapy.World journal of surgical oncology · 2022Article
- Novel RGAG1-BCOR gene fusion revealed in a somatic soft tissue sarcoma with a long follow-up.Virchows Archiv : an international journal of pathology · 2022Article
- Article
- Diagnosis of soft tissue tumors using immunohistochemistry as a surrogate for recurrent fusion oncoproteins.Seminars in diagnostic pathology · 2022Review
- Primary cardiacPediatric investigation · 2021Article
- Imaging of bone and soft tissue BCOR-rearranged sarcoma.Skeletal radiology · 2021Review
- EWSR1-PATZ1-rearranged sarcoma: a report of nine cases of spindle and round cell neoplasms with predilection for thoracoabdominal soft tissues and frequent expression of neural and skeletal muscle markers.Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · 2021Article
- A Rare Case of Round Cell Sarcoma with CIC-DUX4 Mutation Mimicking a Phlegmon: Review of Literature.The American journal of case reports · 2020Review
- DNA Methylation Profiling for Diagnosing Undifferentiated Sarcoma with Capicua Transcriptional Receptor (International journal of molecular sciences · 2020Review
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Authors and funding
23 authors at 7 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
CIC-DUX4 and BCOR-CCNB3 fusion-gene-associated small round cell sarcomas account for a proportion of pediatric small round cell sarcomas, but their pathological features have not been sufficiently clarified. We reviewed a large number of soft tissue tumors registered at our institution, retrieved the cases of unclassified tumors with a small round cell component, and subjected them to histopathological, immunohistochemical, and gene profile analysis. We reviewed 164 cases of unclassified tumors with a small round cell component and analyzed them by RT-PCR and FISH. Tumors positive for a specific fusion-gene were also subjected to histopathological and immunohistochemical examinations. We identified 16 cases of BCOR-CCNB3/CIC-associated (CIC-DUX4 or CIC gene rearrangement-positive) sarcomas. These included seven BCOR-CCNB3 sarcomas and nine CIC-associated sarcomas. Heterogeneous elements included a myxoid spindle cell component in three BCOR-CCNB3 sarcomas and an epithelioid cell component in two CIC-associated sarcomas (one CIC-DUX4-positive and one CIC-DUX4-negative sarcomas). Mitotic activity was low in both heterogeneous components. By immunohistochemistry, in seven BCOR-CCNB3 sarcomas expression of EMA was positive in two cases, of p63 in three, of CD56 in six, of TLE1 in seven, of NKX2.2 in two, of CCNB3 in seven, and of BCOR in six cases (one case could not be tested for BCOR). In nine cases of CIC-associated sarcoma, CD56 was expressed in five, alpha-smooth muscle actin in one, ERG in three, and CD99, WT1 and TLE1 each in eight cases. Both sarcoma types showed not only a small round cell component, but also a myxoid/epithelioid component with low mitotic activity.
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