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The mystery of UPS tumors: Why is it called "undifferentiated pleomorphic sarcoma"
Undifferentiated pleomorphic sarcoma (UPS) is a rare and challenging tumor type that is often classified as a tumor of poor differentiation. The characteristic of this tumor is that the microscopic morphology of its cells is unclear and difficult to differentiate, making it difficult for doctors to make a clear judgment on its specific origin and nature. According to the World Health Organization (WHO) definition, UPS is classified as an undifferentiated/unclassified sarcoma, which belongs to the category of tumors with poor differentiation.
UPS tumors usually develop in adults between the ages of 50 and 70 and often present as a fast-growing, painless mass.
Sarcoma is a type of cancer that originates from mesenchymal stem cells and usually occurs in tissues such as bones, muscles, fat, blood vessels, lymphatic vessels, tendons and ligaments. Scientific research has described more than 70 subtypes of sarcoma, and UPS is a highly undifferentiated tumor with tumor cells of varying morphologies, including fibroblasts, histiocytes, and macrophages.
In the process of diagnosing UPS, doctors will use histology, immunohistochemistry and ultrastructural examinations. When these examinations cannot determine the specific type of cells, UPS can be confirmed. In particular, this tumor is often misdiagnosed as malignant fibrous histiocytoma (MFH), but studies have shown that MFH is not actually derived from histiocytes but from mesenchymal cells. The recent findings may also lead to revisions in the treatment and prognosis of this type of tumor with further research.
The vast majority of UPS tumors are highly invasive and often recur and metastasize after surgical resection.
Treatment of UPS usually includes surgical resection, radiation therapy, and chemotherapy. In recent years, antibody therapy has also been gradually applied to the treatment of UPS, in which antibodies bind to specific antigens on the surface of T cells, thereby enhancing the ability of T cells to attack UPS tumor cells. Learning about these new treatment options is undoubtedly a path of hope for tumor patients with this condition.
Symptoms and manifestations
UPS commonly presents as a deep, rapidly enlarging, painless mass, usually in adults between the ages of 50 and 70. Studies have shown that the diameter of UPS tumors can range from 1 to 55 cm, with an average of 8.8 cm. Most of them occur in the limbs and abdomen, and in a few cases they may also appear in other parts such as the liver and lungs.
Pathology
Because the histology of UPS tumors is nonspecific, they are usually diagnosed by exclusion. The tumor cells of UPS are undifferentiated and pleomorphic, showing highly variable characteristics under the microscope. The diagnosis is mainly based on the expression of certain specific proteins. Results from different studies have shown that UPS tumor cells may express markers such as PD-L1 and IDO1, and the presence of these markers is closely related to prognosis.
Treatment and PrognosisFor localized UPS tumors, the most common treatment is complete surgical removal to ensure that no tumor cells remain. In addition, adjuvant radiation therapy or chemotherapy may be given to high-risk patients. In terms of prognosis, the five-year survival rate of most patients is affected by factors such as tumor size, location, and whether there is metastasis.
Although current treatment options are mixed, future research is needed to identify optimal treatment strategies for UPS tumors.
Immunotherapy, especially inhibitors targeting PD-1 such as pembrolizumab, has been tried for the treatment of UPS and has shown some efficacy. Then, examining the long-term effectiveness of these new treatments and how they compare to traditional approaches will undoubtedly be a focus of future research.
People are still very curious about the mysterious UPS tumor. Can we gain a deeper understanding of its nature and open a new chapter in future treatment?
