We and others have identified FGFR4 as a direct transcriptional target of the alveolar rhabdomyosarcoma (ARMS) specific fusion protein, PAX3-FOXO1. We hypothesized fibroblast growth factor receptor 4 (FGFR4) may act as an effector of PAX3-FOXO1, contributing to PAX3-FOXO1 tumorigenic phenotypes. However, we demonstrate that enhanced expression of FGFR4 does not contribute to inhibited differentiation, enhanced proliferation, or transformation downstream of PAX3-FOXO1 in primary mouse myoblasts. Therefore we were unable to identify any contribution of up regulation of wild type FGFR4 to PAX3-FOXO1 driven tumorigenesis. Conversely, a constitutively active mutant of FGFR4 can enhance primary myoblast proliferation and transformation, indicating activating mutations of FGFR4 could contribute to the development and progression of ARMS. We sequenced the FGFR4 mRNA from five ARMS cell lines and identified no somatic mutations, nor any association with any human single nucleotide polymorphism within the FGFR4 coding region.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1002/mc.20848 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Primary perianal alveolar rhabdomyosarcoma with uncommon metastatic sites: a case report and follow-up using F-FDG PET/CT.
Front Med (Lausanne)
December 2024
Department of Nuclear Medicine, Peking University First Hospital, Beijing, China.
Background: Rhabdomyosarcoma (RMS), a rare pediatric soft tissue neoplasm, predominantly develops in late childhood and adolescence with no discernible gender bias. Alveolar rhabdomyosarcoma (ARMS) stems from mesenchymal cells and may develop most frequently in the trunk, extremities, and head/neck areas, while occurrences in the pelvic cavity are less frequent. The manifestation is typically characterized by a high rate of aggressive metastasis and a poor overall survival prognosis.
View Article and Find Full Text PDFOrbital and sinus rhabdomyosarcoma with concurrent central retinal artery occlusion: A case report.
World J Clin Oncol
December 2024
Department of Ophthalmology, The Sixth Affiliated Hospital of Jinan University, Dongguan 523000, Guangdong Province, China.
Background: Both rhabdomyosarcoma (RMS) and central retinal artery occlusion (CRAO) are rare medical diseases, and their simultaneous occurrence in the same patient is extraordinarily uncommon. This study presents a comprehensive overview of the clinical manifestations, diagnostic imaging results, and therapeutic interventions of a patient with both conditions.
Case Summary: In this report, we present a 30-year-old male who presented with significant protrusion, pain and vision loss and was diagnosed with RMS in the orbit and sinus with CRAO.
Rhabdomyosarcoma in children and young adults.
Virchows Arch
December 2024
Department of Pathology and Laboratory Medicine, Indiana University, 350 W 11st St, Indianapolis, IN, 46202, USA.
Rhabdomyosarcoma (RMS) is the most common soft tissue malignancy in childhood, accounting for 3% of all pediatric malignancies and 50% of all pediatric soft tissue sarcomas. In adolescents and young adults (AYA) however, RMS comprises only 6.5% of all soft tissue sarcomas.
View Article and Find Full Text PDFMaintenance therapy with trofosfamide, idarubicin and etoposide in patients with rhabdomyosarcoma and other high-risk soft tissue sarcomas (CWS-2007-HR): a multicentre, open-label, randomised controlled phase 3 trial.
EClinicalMedicine
December 2024
University Hospital Frankfurt, Department for Children and Adolescents, Goethe University, Frankfurt am Main, Germany.
Background: Rhabdomyosarcoma and other soft tissue sarcomas (STS) with high-risk features are still associated with an unsatisfactory outcome. We evaluated the efficacy of oral maintenance therapy added at the end of standard therapy in patients with high-risk rhabdomyosarcoma and STS.
Methods: CWS-2007-HR was a multicentre, open-label, randomised controlled, phase 3 trial done at 87 centers in 5 countries.
[Characteristics of the cytogenetic variants of alveolar rhabdomyosarcoma].
Arkh Patol
December 2024
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russia.
Unlabelled: Rhabdomyosarcomas (RMS) are one of the most common types of sarcomas in children and adolescents. The alveolar RMS subgroup is of particular interest because in some cases, the translocation of the and genes is combined with an amplification of the corresponding hybrid gene. According to literature data, the frequency of the translocation is 70-90% and the translocation 10-30%.
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!