Extremity and Truncal Soft Tissue Sarcoma: Risk Assessment and Multidisciplinary Management

Soft tissue sarcomas (STS) are a heterogeneous group of malignant tumors with over 150 different histological and molecular types that account for about 1% of all tumors.1 Extremities are the most common site of STS occurrence, accounting for around 40% to 60% of all cases, with lower limbs being involved 3 times more frequently than the upper limbs. Approximately 13% to 19% of STS occur in the trunk.2,3 Any of the STS histological types can occur in these anatomical sites, and undifferentiated pleomorphic sarcoma, liposarcoma, leiomyosarcoma, myxofibrosarcoma, and synovial sarcoma are the most prevalent ones.3, 4, 5, 6, 7 The histological type carries both prognostic and predictive implications. Certain histotypes, such as myxofibrosarcoma, exhibit a higher propensity for local recurrence (LR),8 while others like synovial sarcoma, angiosarcoma, and leiomyosarcoma are associated with a higher risk of metastatic disease.8,9 Furthermore, some histological types such as myxoid liposarcoma and synovial sarcoma have been found to be more sensitive to chemotherapy, while others like clear cell sarcoma and alveolar soft part sarcoma have shown no response to conventional systemic treatment. Similar variability across histotypes is observed with radiotherapy.8,10 Extremity and truncal STS typically have a better prognosis than tumors of the retroperitoneum or pelvis, with high-grade sarcoma representing roughly 75% of cases in the extremity and trunk.4,11 In patients with truncal and extremity sarcomas, the risk of tumor-related death is mostly associated with the development of distant metastases (DM), which for most histotypes occurs most commonly in the lungs.

留言 (0)

沒有登入
gif