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Undifferentiated Pleomorphic Sarcoma

Origin and causes

Undifferentiated pleomorphic sarcoma (UPS) is a rare soft tissue cancer that accounts for 10% of all sarcomas. Adults between the ages of 50 and 70 are most at risk of developing UPS. It is very rare in people under the age of 20. No cause has been identified to date to explain the onset of this cancer.

Symptoms and signs

UPS can occur in any part of the body, but is primarily found in the arms and legs. It most often presents as a progressive, sometimes painful mass that can quickly progress. UPS is an aggressive cancer that can cause metastases, primarily in the lungs.

Diagnosis

When a sarcoma is suspected, the initial evaluation may include a CT scan or MRI. The diagnosis is made through a biopsy of the suspected area. The investigation may be completed with a chest CT scan and a PET scan.

Treatment

When UPS is localized, the preferred treatment is surgery to remove all of it. Radiation therapy before or after surgery is frequently necessary.

In the event that metastases are found, chemotherapy is offered with the goal of controlling symptoms and disease progression. The preferred agents are doxorubicin, gemcitabine-docetaxel, ifosfamide, and pazopanib. Recently, immunotherapy treatments such as pembrolizumab may be indicated in some patients. Clinical trials may also be explored.

Follow-up

After surgery, patients with undifferentiated pleomorphic sarcoma will be closely monitored due to the risk of recurrence.