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Undifferentiated pleomorphic sarcoma (UPS), also termed pleomorphic myofibrosarcoma, [1] high-grade myofibroblastic sarcoma, and high-grade myofibrosarcoma, [2] is characterized by the World Health Organization (WHO) as a rare, poorly differentiated neoplasm (i.e., an abnormal growth of cells that have an unclear identity and/or cell of origin). [3]
Kimberley Nix, a Canadian physician, chronicled her journey with undifferentiated pleomorphic sarcoma, from her diagnosis to eventual death, on TikTok under the username @cancerpatientmd. [38] Nix died on 8 May 2024 at the age of 31, and her death was announced in a video uploaded posthumously to her TikTok page. [39]
As a novel form of treatment used in other cancers, immunotherapy may have a role in treating soft-tissue sarcomas like alveolar soft part sarcoma and pleomorphic undifferentiated sarcoma. However, as of 2023, only alveolar soft part sarcoma has a regulatory approval for such an agent, in this case atezolizumab. [19]
malignant fibrous histiocytoma: This tumor is now termed undifferentiated pleomorphic sarcoma (UPS) because more recently developed immunohistochemistry and cytogenetic analyses suggest that its neoplastic cells most likely arise from mesenchymal stem cells [5] or fibroblasts [6] rather than histiocytes. histiocytoma (dog)
Pleomorphic rhabdomyosarcoma (undifferentiated rhabdomyosarcoma), also known as anaplastic rhabdomyosarcoma, is defined by the presence of pleomorphic cells with large, lobate hyperchromatic nuclei and multipolar mitotic figures. These tumors display high heterogeneity and extremely poor differentiation.
Consequently, they have been mistakenly diagnosed as undifferentiated pleomorphic sarcoma (also termed malignant fibrous histiocytoma), rhabdomyosarcoma, [1] or other types of sarcoma [8] and treated unnecessarily with aggressive measures used for such malignancies, e.g. wide surgical resection, radiation therapy, and chemotherapy.
Of particular importance, the presence of pseudo-lipoblasts in a myxoid sarcoma-like background is an extremely strong indicator that the tumor is a MFS. [ 6 ] and tumors with a myxofibrosarcoma-like histopathology that initiate in the retroperitoneum, abdominal cavity, or pelvis are nearly always dedifferentiated liposarcomas .
Low-grade myofibroblastic sarcoma (LGMS) is a subtype of the malignant sarcomas. [1] As it is currently recognized, LGMS was first described as a rare, atypical myofibroblastic tumor (i.e. a tumor consisting of cells with the microscopic features of fibroblasts and smooth muscle cells ) by Mentzel et al. in 1998. [ 2 ]
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