Undifferentiated Pleomorphic Sarcoma (UPS) | Primary Research (KOL's Insight) | Market Intelligence | Epidemiology & Market Forecast-2035

An aggressive high-grade soft tissue sarcoma (STS), undifferentiated pleomorphic sarcoma (UPS) was formerly known as malignant fibrous histiocytoma. Tumors most likely originate from mesenchymal stem cells, not tissue cells, as was once believed. Soft tissues, bones, the retroperitoneum, and various organs can all be impacted. UPS is included under malignant tumors of uncertain differentiation in the 2020 World Health Organization classification of STSs. For head, neck, trunk, and extremity UPS, complete surgical resection with negative microscopic margins is the gold standard of care, particularly for stage I tumors. This can be done by performing a wide local excision with 2 cm margins of intact tissue, but in some circumstances, it may be more challenging due to the involvement of significant neurovascular structures.

Description

An aggressive high-grade soft tissue sarcoma (STS), undifferentiated pleomorphic sarcoma (UPS) was formerly known as malignant fibrous histiocytoma. Tumors are produced by mesenchymal stem cells rather than tissue cells, as was once believed. Soft tissues, bones, the retroperitoneum, and various organs can all be impacted. The name "undifferentiated pleomorphic sarcoma" refers to the way the cancer cells look under a microscope. Undifferentiated cells are those that are not similar to the tissues of the body from which they originate. Because the cells grow in a variety of sizes and shapes, cancer is referred to as pleomorphic (plee-o-MOR-fik). The retroperitoneum, or back of the abdominal cavity, is where UPS is most frequently found. It is a rapidly expanding sarcoma that has the potential to spread to other body organs, most frequently the lungs. elderly people tend to have more of it. The most typical sarcoma, UPS, develops where patients have had radiation treatment in the past. This sarcoma typically manifests as an enlarged, painless soft tissue mass. It may develop quickly, at which point the lump's size may be painful or extremely pressing. Some patients may experience fever, malaise, and/or weight loss if the lump grows large enough. It has been a contentious idea ever since Kaufman and Stott first put it forth in 1961. A histiocyte-rich tumor with a growth pattern resembling a buffer was described as MFH. Adults with MFH have the most common type of soft tissue sarcoma, which was first identified in 1977. MFH is still a mystery despite the high rate of diagnosis. The cells' real origin is still unknown. The World Health Organization changed the name of MFH to unspecified undifferentiated pleomorphic sarcoma, or NOS, in 2002 and added it to the list of official diagnostic entities.

Undifferentiated Pleomorphic Sarcoma (UPS) (Epidemiology)

UPS is included under "malignant tumors of uncertain differentiation" in the STS classification of the 2020 World Health Organization. The Surveillance, Epidemiology and End Results (SEER) program found that regardless of where the primary tumor was located, UPS accounted for 17% of the 26,758 cases and was the second most prevalent STD after leiomyosarcoma. Men are more likely to experience it than women, and white men are more likely to do so than Black men. Additionally, increasing linearly with age, incidence is highest after age 60. Undifferentiated pleomorphic sarcoma typically affects adults (range, 32 to 80 years; mean age, 59 years), with a slight male predominance (M: F 1.2:1). The most prevalent type of aggressive soft tissue tumors in adults is undifferentiated pleomorphic sarcoma, which makes up 25-40% of all adult soft tissue tumors. The classification system has tightened up though, as a lot of tumors have been reclassified as sarcoma variants. At the moment, it accounts for 5% of adult sarcomas. The majority of the time, it only affects soft tissue, though it can sporadically develop in or come from bone (1-5%). Undifferentiated pleomorphic sarcomas can develop almost anywhere on the body, but they frequently develop in the extremities.

Undifferentiated Pleomorphic Sarcoma (UPS) -Current Market Size & Forecast Trends

The market for undifferentiated pleomorphic sarcoma (UPS) is projected to grow significantly, with estimates indicating a market size of approximately USD 300 million in 2024. This market is expected to reach around USD 500 million by 2034, reflecting a compound annual growth rate (CAGR) of about 5.5% during this period. The growth is driven by the increasing incidence of UPS, advancements in treatment options, and rising awareness of the disease.

The standard of care for UPS of the head, neck, trunk, and extremities is complete surgical excision with negative microscopic margins, especially for stage I tumors. This can be achieved by wide local excision of 2 cm margins of intact tissue but may be more difficult in some cases due to invasion of the underlying neurovascular. Postoperative radiotherapy (RT) is indicated if the margins are close to the tumor (<1 cm), microscopically benign, or involve bone, major vessels, or nerves. Muscle microsurgery (MMS) has been used in the past to preserve healthy tissue. Amputation is considered a last resort because survival rates do not exceed limb salvage. In general, chemotherapy with various anticancer agents is used in advanced, diffuse, or irreversible stages of various types of sexually transmitted diseases. The second and third steps should include a multidisciplinary procedure to discuss the appropriateness of chemotherapy before and after surgery. If surgery is not possible, it can be treated with chemotherapy, chemotherapy, or local extremity therapy. There is insufficient data to support a specific plan for stage IV patients and advice from an STS oncologist is needed. In a comparative study of 286 patients, histologically corrected neoadjuvant chemotherapy was not superior to standard chemotherapy (epirubicin plus ifosfamide) in high-risk STS patients. Indeed, disease-free survival (HR 2.17, 95% CI, 0.98-4.80) was histologically better in UPS patients who received standard chemotherapy than in patients who received specific therapies (gemcitabine plus docetaxel). This is a large, open-label, randomized controlled trial of 435 STI patients from 4 countries, which confirmed that anthracyclines and ifosfamide remain treatment options. In addition to conventional UPS chemotherapy, immune checkpoint inhibition with pembrolizumab (anti-PD1), nivolumab (anti-PD1), and ipilimumab (anti-CTLA4) is currently being investigated. Similar principles apply to intra, retroperitoneal, or intraperitoneal administration of UPS. Resection with negative margins, with or without IORT, is the treatment of choice for resectable disease; in inoperable cases, chemotherapy, chemotherapy or HDR is preferred.

Report Highlights

Undifferentiated Pleomorphic Sarcoma (UPS) - Current Market Trends

Undifferentiated Pleomorphic Sarcoma (UPS) - Current & Forecasted Cases across the G8 Countries

Undifferentiated Pleomorphic Sarcoma (UPS) - Market Opportunities and Sales Potential for Agents

Undifferentiated Pleomorphic Sarcoma (UPS) - Patient-based Market Forecast to 2035

Undifferentiated Pleomorphic Sarcoma (UPS) - Untapped Business Opportunities

Undifferentiated Pleomorphic Sarcoma (UPS) - Product Positioning Vis-a-vis Competitors' Products

Undifferentiated Pleomorphic Sarcoma (UPS) - KOLs Insight