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Novel Therapeutics in Soft Tissue Sarcoma.
Cancers (Basel)
December 2024
Sarcoma Unit, The Royal Marsden Hospital and Institute of Cancer Research, London SW3 6JZ, UK.
There has been noteworthy progress in molecular characterisation and therapeutics in soft tissue sarcomas. Novel agents have gained regulatory approval by the FDA. Examples are the tyrosine kinase inhibitors avapritinib and ripretinib in gastrointestinal stromal tumours (GIST), the immune check point inhibitor atezolizumab in alveolar soft part tissue sarcoma, the γ-secretase inhibitor nirogacestat in desmoid tumours, the NTRK inhibitors larotrectinib and entrectinib in tumours with fusions, the mTOR inhibitor nab-sirolimus in PEComa, and the EZH-2 inhibitor tazemetostat in epithelioid sarcoma.
View Article and Find Full Text PDFCardiac metastasis of a soft tissue sarcoma: Case report and review of literature.
Acta Radiol Open
January 2025
Radiology Department, Mohammed VI University Hospital, Tangier, Morroco.
Cardiac metastases are the most frequent cardiac tumors. They can cause dysrhythmia, myocardial dysfunction, pericardial effusion, and heart failure. In decreasing order, the major primary malignancies associated with cardiac metastases are pleural mesothelioma, lung adenocarcinoma, undifferentiated carcinomas, lung squamous cell carcinoma, and breast carcinoma.
View Article and Find Full Text PDFTreatment of a Radiation-Associated Femur Fracture with an Intercalary Endoprosthesis: A Case Report.
JBJS Case Connect
October 2024
Department of Orthopedic Oncology, MD Anderson Cancer Center, Houston, Texas.
Case: A 72-year-old woman with undifferentiated pleomorphic sarcoma of the thigh received neoadjuvant chemotherapy and radiotherapy. She underwent wide resection and was scheduled for prophylactic fixation of the femur. However, prophylactic fixation was deferred secondary to COVID-19 pandemic.
View Article and Find Full Text PDFAssessing the Utility of DNA Methylation Profiling in the Classification of Myogenic Sarcomas of Bone.
Int J Surg Pathol
January 2025
Department of Laboratory Medicine and Pathology, University of Washington, Seattle, WA, USA.
The diagnosis of primary leiomyosarcoma (LMS) of bone is generally established based on integrative findings of morphologic features, immunohistochemical staining, and clinical and radiological findings. There are no specific genetic alterations that can be used to confirm the diagnosis of LMS in challenging diagnoses of bone sarcomas with myogenic differentiation. In this study, we assessed the utility of a DNA methylation-based classifier as an ancillary diagnostic tool for subclassifying bone sarcomas with myogenic differentiation.
View Article and Find Full Text PDFSarcomas developed in patients with Lynch Syndrome are enriched in pleomorphic soft-tissue sarcomas and are sensitive to immunotherapy.
Eur J Cancer
December 2024
Department of Digestive Medical Oncology, Toulouse University Hospital, Toulouse, France; Toulouse Cancer Research Centre, Toulouse, France; Department of Oncogenetics, Oncopole Claudius Regaud, Toulouse, France; Groupe Génétique et Cancer, Unicancer, France. Electronic address:
Article Synopsis
- Sarcomas are not typically associated with Lynch Syndrome (LS), but recent literature suggests a connection, prompting a national study to investigate their characteristics in LS patients.
- The SarcLynch study included 81 patients, finding that 83% had soft-tissue sarcomas, particularly pleomorphic variants like undifferentiated pleomorphic sarcoma and pleomorphic rhabdomyosarcoma, with 40% having sarcoma as their first cancer event.
- Results showed a high prevalence of mismatch repair deficiency and promising responses to immune checkpoint inhibitors, suggesting the need for screening and potential immunotherapy for these sarcomas.
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