Advanced FET-rearranged sarcomas | Primary Research (KOL's Insight) | Market Intelligence | Epidemiology & Market Forecast-2035

Ewing's sarcoma enlarged round cell tumor, myxoid liposarcoma, and clear cell sarcoma are all examples of FET family rearranged sarcomas. Askin tumors, peripheral primitive neuroectodermal tumors, and Ewing sarcoma are all examples of Ewing sarcoma tumors. Ewing sarcoma affects 2.6 out of every million women and 3.3 out of every million men. Late adolescence is when these tumors are most prevalent. Depending on the patient's tolerance and the hospital's proximity, chemotherapy may be administered as an inpatient or outpatient procedure. Vincristine, doxorubicin, cyclophosphamide and ifosfamide and etoposide are the two chemotherapy regimens that are alternated during the course of the treatment, which lasts between 6 and 9 months.

Description

Ewing's sarcoma enlarged round cell tumor, myxoid liposarcoma, and clear cell sarcoma are all sarcomas that have had their FET family rearranged. Ewing sarcoma tumors include peripheral primitive neuroectodermal tumors, Askin tumors, and Ewing sarcoma itself. In addition to sharing a chromosomal translocation, these tumors have a similar cellular physiology. A rare childhood renal neoplasm is clear cell sarcoma of the kidney (CCSK). Usually, the kidney is replaced entirely or noticeably deformed by a large unicentric mass. Shallow-uterine therapy produced clinically significant soft tissue sarcoma endpoints of stable disease (SD) and long-term progression (TTP) in patients with FET-rearranged sarcoma, which are indicators of disease control.

Advanced FET-rearranged sarcomas (Epidemiology)

The incidence of Ewing sarcoma is 2.6 per million women and 3.3 per million men. The highest incidence of these tumors occurs during adolescence. Overall, 27% of onset occurred in the first decade of life, 64% in the 20s, and 9% in the 30s. The male to female ratio was 2.04:1, with male students predominating. The age at visit ranged from 2 months to 14 years, and the mean age was 36 months. Most renal cell sarcoma occurs in children aged 2 to 3 years and is diagnosed in 50% of cases. The incidence in children aged 3 years and older has decreased dramatically. Clear cell sarcoma of the kidney is very rare but has been reported in infants and young children under 6 months of age.

Advanced FET-rearranged sarcomas -Current Market Size & Forecast Trends

The market for advanced FET-rearranged sarcomas is expected to grow significantly, driven by increasing awareness and advancements in treatment options. As of 2022, the global sarcoma drugs market was valued at approximately USD 1.29 billion and is projected to grow at a compound annual growth rate (CAGR) of 8.9% from 2023 to 2030. This growth is fueled by the rising incidence of soft tissue sarcomas, including FET-rearranged variants, and the development of novel therapies such as targeted inhibitors. Clinical trials are ongoing, notably involving Salarius Pharmaceuticals' seclidemstat, which has shown promising results in treating Ewing sarcoma and FET-rearranged sarcomas, suggesting potential for improved disease control and progression-free survival. Overall, the advanced FET-rearranged sarcoma market is well-positioned for substantial growth through 2035 as new therapies and treatment strategies continue to emerge.

Depending on the patient's tolerance and the distance to the hospital, chemotherapy can be administered as an inpatient or outpatient procedure. Treatment lasts 6-9 months and involves alternating cycles of two chemotherapy regimens: (1) vincristine, doxorubicin, and cyclophosphamide, and (2) ifosfamide and etoposide. Patients with neutropenia experienced frequent febrilities and needed hospitalization for 3 to 7 days in between chemotherapy cycles. In a pilot study using low-dose antiangiogenic therapy along with conventional chemotherapy in patients with recently disseminated Ewing sarcoma, Felgenhauer et al concluded that combination therapy was "feasible by protocol definition" but was also frequently applied to patients receiving radiation therapy. The protocol's utility is thus constrained. Although the 24-month survival of patients with isolated lung metastases in the study was higher than that of historical controls, the researchers pointed out that the study only used a small number of patients and lacked contemporary controls. According to a study by the Pediatric Oncology Group, reducing the time between chemotherapy treatments from the typical 3 to 2 weeks improves outcomes in localized Ewing sarcoma without raising toxicity. The 5-year event-free survival was 73% in patients who received interval compression chemotherapy every 2 weeks versus 65% in patients who received chemotherapy every 3 weeks, according to the researchers. Patients in both groups received granulocyte colony-stimulating factor (G-CSF) to maintain adequate neutrophil counts along with similar chemotherapy doses. In spite of further intensification, there was no discernible increase in treatment-related toxicity. Vincristine, topotecan, and cyclophosphamide are being added to a compact 5-drug backbone in patients with nonmetastatic Ewing's sarcoma in an open-label study being conducted in the Pediatric Oncology Group (AEWS1031) (NCT01231906). Informed consent must be given before any treatment if a patient is to take part in a clinical trial. Oncologists will consult the most recent clinical trials to determine the most effective treatment options if the appropriate patient studies are not gathered. A consent form outlining the suggested medications and their side effects should unquestionably be taken into consideration in such circumstances. For long-term treatment, the primary tumor site must be managed. Wide limb salvage and the removal of debris are two examples of precise surgical margins that are needed. Any surgery should be carried out under the guidance of an experienced oncologist with expertise in the body part where the tumor is located. The specific procedure is highly dependent on the patient. Radiation therapy can be used to achieve local control in the absence of a minimally invasive disease. Regional factors affect tumor dose and segmentation.

Report Highlights

Advanced FET-rearranged sarcomas - Current Market Trends

Advanced FET-rearranged sarcomas - Current & Forecasted Cases across the G8 Countries

Advanced FET-rearranged sarcomas - Market Opportunities and Sales Potential for Agents

Advanced FET-rearranged sarcomas - Patient-based Market Forecast to 2035

Advanced FET-rearranged sarcomas - Untapped Business Opportunities

Advanced FET-rearranged sarcomas - Product Positioning Vis-a-vis Competitors' Products

Advanced FET-rearranged sarcomas - KOLs Insight