Biologic Features of Adult Soft Tissue Sarcomas
The exception to the biologic features of adult soft tissue sarcomas is a low (less than 1%) risk of metastasis for small, low-grade lesions, as this is inconsistent with the established clinical behavior of these tumors.
Correct Biologic Features of Adult Soft Tissue Sarcomas
Mutations of p53 are commonly found in metastatic liposarcoma, representing one of the genetic alterations that characterize aggressive soft tissue sarcomas 1
Small, low-grade soft tissue sarcomas actually have a significant risk of metastasis, not less than 1%. According to clinical guidelines, even intermediate-high grade, deep tumors with a diameter of >5 cm have substantial metastatic potential, necessitating consideration of adjuvant treatments 1
Recurrent disease occurs in at least 33% of patients with soft tissue sarcomas, highlighting the aggressive nature of these tumors and the need for close surveillance following initial treatment 1
Lymph node metastasis occurs in less than 3% of patients with soft tissue sarcomas, as these tumors predominantly spread hematogenously rather than through lymphatic channels 1
Mutations of p53 are characteristic of Li-Fraumeni syndrome, a hereditary cancer predisposition syndrome associated with the development of various malignancies including soft tissue sarcomas 1
Molecular and Genetic Characteristics
Soft tissue sarcomas can be classified into two major genetic groups: those with complex unbalanced karyotypes and those with specific chromosomal translocations 1
Specific fusion gene transcripts resulting from chromosomal translocations provide useful diagnostic and prognostic information in certain sarcoma subtypes (e.g., EWS-ATF1 in clear cell sarcoma, TLS-CHOP in myxoid liposarcoma) 1
Molecular testing, while promising, should be considered an ancillary technique and interpreted in the context of morphologic features due to technical limitations 1
Prognostic Factors
Tumor grade, margin status, and tumor size are the most important prognostic factors for soft tissue sarcomas 2
The Federation of Cancer Centers Sarcoma Group (FNCLCC) grading system, based on tumor differentiation, mitosis count, and tumor necrosis, shows increased ability to predict distant metastasis development and tumor mortality 1
High-risk features include grade 2-3, deep location, and size >5 cm, which may warrant consideration of adjuvant therapy 1, 3
Treatment Implications
Surgery remains the standard primary treatment for most sarcomas, with wide excision being the standard surgical procedure 1
Radiation therapy is standard treatment for intermediate-high grade, deep tumors with a diameter of >5 cm 1, 3
Regional hyperthermia in combination with systemic chemotherapy has shown improvement in local progression-free survival and disease-free survival in patients with high-risk soft tissue sarcomas 4, 3