∼10,400 cases/year and ∼3,700 deaths/year in the US Median age 40–60 years. Slight male predominance, more frequent among African-Americans. Genetics: NF-1, Retinoblastoma, Gardner’s syndrome, Li-Fraumeni syndrome. Environmental exposures: ionizing radiation, herbicides, thorotrast, chlorophenols, vinyl chloride, arsenic. Lower extremity (45%) > trunk (30%) > upper extremity (15%) > H&N (8%). Extremity = liposarcoma, MFH, synovial, fibrosarcoma, myxoid liposarcoma (upper medial thigh). Retroperitoneal = liposarcoma (fewer DM) > leiomyosarcoma (increased DM). H&N = MFH, usually high-grade (except myxoid MFH = intermediate grade). Frequency: MFH (20–30%), liposarcoma (10–20%), leiomyosarcoma (10–15%), fibrosarcoma (5–10%), synovial cell sarcoma (5–10%), rhabdomyosarcoma (5–10%), malignant peripheral nerve sheath tumor/malignant schwannoma (5%). Synovial sarcoma = usually high-grade, near (but not within) joints in tendon sheaths, bursae, and joint capsules. Grade based on cellularity, differentiation, pleomorphism, necrosis, #mitoses. Cytogenetics: See Appendix D.
KeywordsSynovial Sarcoma Malignant Peripheral Nerve Sheath Tumor Tendon Sheath Myxoid Liposarcoma Medial Thigh
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