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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]
A sarcoma is a malignant tumor, a type of cancer that arises from cells of mesenchymal (connective tissue) origin. [1] [2] Connective tissue is a broad term that includes bone, cartilage, muscle, fat, vascular, or other structural tissues, and sarcomas can arise in any of these types of tissues.
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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]
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.
However, these tumors have high recurrence rates at the sites of their resections. [6] Local recurrences followed by surgical resections may be repeated multiple times but during these cycles MFS tumors often progress from a lower grade to a higher more aggressive grade, metastasize , and become life-threatening. [ 4 ]