Category Ranking
98%
Total Visits
921
Avg Visit Duration
2 minutes
Citations
20
Article Abstract
Introduction: Capicua transcriptional repressor (CIC)-DUX4 rearranged sarcoma is a subtype of CIC-rearranged sarcomas composed of undifferentiated Wilms' tumor 1 (WT1), CD99 round cells with recurrent CIC gene rearrangement. The diagnosis of CIC-rearranged sarcoma remains challenging, and the prognosis of CIC-rearranged sarcomas is poor.
Case Presentation: In this report, we described a case of CIC-DUX4 rearranged sarcoma presenting in the skin, expressing WT1 and CD99 in a dot-like pattern. In addition, the assessment of genomic alterations using genome panel testing was useful to confirm the accurate diagnosis.
Conclusion: Our present case suggests that widespread use of genomic panel testing in the future may lead to early treatment and improve the prognosis of CIC-rearranged sarcomas.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11249449 | PMC |
| http://dx.doi.org/10.1159/000539501 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Patient-derived tumoroids from CIC::DUX4 rearranged sarcoma identify MCL1 as a therapeutic target.
Nat Commun
August 2025
Department of Oncology and Children's Research Center, University Children's Hospital, University of Zurich, Zurich, Switzerland.
High-risk sarcomas, such as metastatic and relapsed Ewing and CIC-rearranged sarcoma, still have a poor prognosis despite intensive therapeutic regimens. Precision medicine approaches offer hope, and ex vivo drug response profiling of patient-derived tumor cells emerges as a promising tool to identify effective therapies for individual patients. Here, we establish ex vivo culture conditions to propagate Ewing sarcoma and CIC::DUX4 sarcoma as tumoroids.
View Article and Find Full Text PDFSmall round cell sarcoma tumoroid biobank reveals CIC::DUX4 sarcoma vulnerability to MCL-1 inhibition.
Nat Commun
August 2025
Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands.
Small round cell sarcomas (SRCS) are highly aggressive tumors in soft tissues and bone of mostly children and young adults. Despite being different in many aspects, including genetics, possible cell-of-origin, and pathology, patients with any of these entities all receive the same therapeutic regimen. Although several pre-clinical models of Ewing sarcoma have been established, such as cell lines and patient-derived tumor xenografts, few models exist for other SRCS.
View Article and Find Full Text PDFThe CIC::DUX4 oncoprotein maintains DNA integrity through direct regulation of the catalytic subunit of DNA polymerase epsilon (POLE).
Oncogene
August 2025
Department of Medicine, University of California, San Francisco, CA, USA.
Transcription factor (TF) fusion oncoproteins represent cancer-specific alterations that arise from chromosomal rearrangements. Through target gene recognition, TF fusions can disseminate transcriptional responses that collectively work to drive tumorigenesis. Thus, identifying the molecular targets that operate as a disease-driving network can potentially uncover key actionable dependencies.
View Article and Find Full Text PDF-Rearranged Sarcoma: A Clinical and Pathological Study of a Peculiar Entity.
Diagnostics (Basel)
July 2025
Department of Pathology and Laboratory Medicine, King Hussein Cancer Center, Amman P.O. Box 1269, Jordan.
: -rearranged sarcoma is a rare and aggressive type of undifferentiated round cell tumor characterized by gene fusion, most commonly This study presents a series of eleven cases, highlighting their clinicopathological features. : Pathology records (2019 to 2024) were searched using "sarcoma with ", identifying eleven cases, of which seven referred cases were initially misdiagnosed. Pathological and clinical analysis was conducted.
View Article and Find Full Text PDFCIC::DUX4 Sarcoma With Preferentially Expressed Antigen in Melanoma (PRAME) Immunopositivity.
Cureus
May 2025
Pathology, University of Pittsburgh Medical Center, Pittsburgh, USA.
-rearranged sarcomas are rare, high-grade, undifferentiated, small round cell sarcomas of bone and soft tissue classified by gene fusions involving the gene with other gene partners, most commonly the gene. These tumors tend to affect a wide age range, with a predilection for adult males, with the most common anatomical location being the deep soft tissues of the limbs or trunk. -rearranged sarcomas have proven not only to be challenging to diagnose but also to treat due to their high resistance to conventional therapies.
View Article and Find Full Text PDF