The Rationale: Undifferentiated pleomorphic sarcoma originally known as malignant fibrous histiocytoma was declassified by the World Health Organization in 2002 as a formal diagnostic entity and renamed as an undifferentiated pleomorphic sarcoma. It accounts for <1% of malignant tumours of the long bone.
Patient Concerns: A 33-year-old male patient reported with swelling and pain in the lower left posterior jaw region for 3 months with a history of fall on the floor 3 months back.
Diagnosis: On examination, diffuse solitary swelling was present on the left lower third of the face and was diagnosed radiographically and histopathologically as undifferentiated pleomorphic sarcoma of the mandible.
Treatment And Outcomes: Selective neck dissection, followed by reconstruction with fibula osteomyocutaneous flap and then referred for adjuvant radiotherapy.
Take-away Lessons: Vimentin staining plays a substantial role in the diagnosis of undifferentiated pleomorphic sarcoma. A long-term follow-up after treatment is required to increase the chances of disease-free survival for the patients.
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http://dx.doi.org/10.4103/ams.ams_334_20 | DOI Listing |
J Med Case Rep
January 2025
Department of Oral and Maxillofacial Pathology, School of Dentistry, Shahid Sadoughi University of Medical Sciences, Yazd, Iran.
Background: Undifferentiated pleomorphic sarcoma, previously called malignant fibrous histiocytoma, is a type of malignant mesenchymal tumor (sarcoma) of soft tissue and sometimes bone. It is uncommon in the oral cavity and very sporadic in the maxillary sinus. Microscopic diagnosis of this malignancy in the maxillary sinus can be very challenging, because there is a range of features that may overlap with other benign and malignant tumors.
View Article and Find Full Text PDFJACC Case Rep
December 2024
Wellington Hospital, Wellington, New Zealand.
Angiosarcomas are rare and often a fatal manifestation of cardiac tumors. This paper presents a case of a sarcoma with protrusion through the mitral valve. Angiographic findings demonstrated a tumor blush, supplied by the right coronary and left anterior descending arteries.
View Article and Find Full Text PDFCancer Res
January 2025
Stanford University, Stanford, California, United States.
Radiotherapy is an integral component in the treatment of many types of cancer, with approximately half of cancer patients receiving radiotherapy. Systemic therapy applies pressure that can select for resistant tumor subpopulations, underscoring the importance of understanding how radiation impacts tumor evolution to improve treatment outcomes. We integrated temporal genomic profiling of 120 spatially distinct tumor regions from 20 patients with undifferentiated pleomorphic sarcomas (UPS), longitudinal circulating tumor DNA (ctDNA) analysis, and evolutionary biology computational pipelines to study UPS evolution during tumorigenesis and in response to radiotherapy.
View Article and Find Full Text PDFCancers (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 PDFJBJS 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 PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!