Our understanding of Ewing's sarcoma development mediated by the EWS/FLI fusion protein has been limited by a lack of knowledge regarding the tumor cell of origin. To circumvent this, we analyzed the function of EWS/FLI in Ewing's sarcoma itself. By combining retroviral-mediated RNA interference with reexpression studies, we show that ongoing EWS/FLI expression is required for the tumorigenic phenotype of Ewing's sarcoma. We used this system to define the full complement of EWS/FLI-regulated genes in Ewing's sarcoma. Functional analysis revealed that NKX2.2 is an EWS/FLI-regulated gene that is necessary for oncogenic transformation in this tumor. Thus, we developed a highly validated transcriptional profile for the EWS/FLI fusion protein and identified a critical target gene in Ewing's sarcoma development.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/j.ccr.2006.04.004 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Radical Resection of a Radial Ewing Sarcoma and Ulnar Centralization in a 10-Month-Old Girl: A Case Report.
JBJS Case Connect
January 2025
Morristown Medical Center, Morristown, New Jersey.
Case: We outline the management of a 10-month-old girl with an Ewing sarcoma involving the entire radius. The patient was treated with neoadjuvant chemotherapy resulting in almost a complete response. A limb-sparing procedure was performed consisting of a radical resection of the entire radius and centralization of the carpus to the ulna.
View Article and Find Full Text PDFExtraskeletal Ewing sarcoma with uterine cornua attachment mimicking high grade endometrial stromal sarcoma: A case report and brief literature review.
Gynecol Oncol Rep
December 2024
Department of Pathology, University of California San Diego, La Jolla, CA, USA.
Background: Ewing sarcoma is an EWSR1-rearranged aggressive malignancy that occurs commonly in bone and has small round blue cell morphology. A diagnostic challenge is presented in the cases of extraskeletal Ewing sarcoma involving solid organs, such as the uterus.
Case Report And Brief Literature Review: We present the case of a 54-year-old female with a large pelvic mass connected to the uterine cornua and retroperitoneal soft tissue.
Confirmatory diagnosis and successive chemotherapeutic treatments of metastatic skeletal sarcoma: A case report.
Oncol Lett
March 2025
Department of Medical Oncology, Cancer Institute Hospital of Japanese Foundation for Cancer Research, Tokyo 135-8550, Japan.
sarcoma is rare and its clinical features remain unclear. Given the similarity in presentation, it is possible that previously reported cases of Ewing-like adamantinoma may have been sarcoma. The present case report describes a tumor in a 55-year-old man that was originally thought to be a Ewing-like adamantinoma, but was recently found to be an sarcoma following direct sequencing.
View Article and Find Full Text PDFA Novel Disulfidptosis-Related Risk Signature for Prognostic Prediction in Patients With Ewing Sarcoma.
J Orthop Res
January 2025
Department of East Hospital Orthopaedic Trauma, Zibo Central Hospital, Zibo, China.
Ewing sarcoma (ES) is a malignant bone tumor prevalent among children and adolescents. Disulfidptosis represents a novel form of cell death; however, the mechanism of disulfidptosis in ES remains unclear. Our aim is to explore the disulfidptosis-related prognostic signature in ES.
View Article and Find Full Text PDFTranslocation: A Common Tumor Driver of Distinct Human Neoplasms.
Int J Mol Sci
December 2024
School of Medicine and Dentistry, Faculty of Clinical and Biomedical Sciences, University of Central Lancashire, Preston PR1 2HE, UK.
Cancer is among the leading causes of mortality in developed countries due to limited available therapeutic modalities and high rate of morbidity. Although malignancies might show individual genetic landscapes, recurring aberrations in the neoplastic genome have been identified in the wide range of transformed cells. These include translocations of frequently affected loci of the human genetic material like the Ewing sarcoma breakpoint region 1 () of chromosome 22 that results in malignancies with mesodermal origin.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!