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Cancer of unknown origin: Why is the identity of UPS cells so ambiguous?
Undifferentiated Pleomorphic Sarcoma (UPS) is a rare and difficult-to-classify tumor in which the cell of origin and identity of the cancer is unclear. According to the World Health Organization, UPS are difficult to classify tumors and are often confused with other types of sarcomas, which makes the diagnostic process complicated and challenging. This article will discuss the characteristics, pathogenesis, treatment, and current research trends of UPS.
UPS is often viewed as a clinical and pathological challenge because the cells are not clearly defined.
Clinical manifestations and diagnosis of UPS
UPS usually presents as a deep, painless mass up to several centimeters in diameter and occurs primarily in adults between the ages of 50 and 70. Most of these tumors are located in the extremities, abdomen, or chest and can rarely occur in other locations. When patients are first diagnosed, distant metastasis has already occurred in approximately 6.4% of cases, and after long-term follow-up, the metastasis rate may be as high as 40%.
Studies have shown that UPS cells are highly variable and cannot be classified as any specific cell type, making diagnosis more difficult.
Pathological features and protein markers
In the pathological examination of UPS, the tumor cells are mainly pleomorphic and undifferentiated. Therefore, a definitive diagnosis usually relies on a panel of specific protein markers, such as PD-L1 and IDO1. These markers can help doctors distinguish UPS from other similar tumor types.
The expression level of PD-L1 can predict the prognosis. Patients with strong expression usually have a poor prognosis, while IDO1 shows a better prognosis.
Current treatments
Treatment of UPS usually includes surgical resection, radiation therapy, and chemotherapy. The latest research on this disease has begun to explore the effects of antibody therapy, especially targeting T cells. This new treatment approach hopes to directly mobilize the patient's immune system to fight tumors. According to research, some patients can show certain therapeutic effects after receiving immunotherapy.
Novel immunotherapies, such as pembrolizumab, are emerging as an emerging strategy for treating UPS, but further studies are needed to evaluate their long-term effects.
Prognostic factors and future challenges
The prognosis of UPS is affected by many factors, including tumor size, location, and the presence of distant metastasis. The results showed that patients with tumors larger than 10 centimeters had a nearly six-fold higher risk of metastasis than those with tumors smaller than 4 centimeters. As for the choice of treatment, although surgery is the main treatment method, the effects of adjuvant radiotherapy and chemotherapy are still controversial, and some studies have pointed out that they do not significantly improve prognosis.
ConclusionAs our understanding of UPS deepens, the clinical management and treatment strategies for this disease are expected to become more precise.
Pleomorphic sarcoma of unknown origin (UPS) is a major clinical challenge because its cells cannot be clearly defined. Although existing research has provided some insights into this disease, there is still a lack of consensus on its optimal treatment. How can we more effectively combat this annoying cancer in the future?
