Sarcomas of Soft Tissue
Summary of Key Points
Incidence and Epidemiology
• In the United States, there are 11,280 new cases annually; fewer than 1% of cancer diagnoses.
• No specific etiologic agent is identifiable in the majority of cases.
• Occasional cases are related to previous radiation, chemical exposure, alkylating chemotherapeutic agents, or chronic lymphedema.
• Genetic conditions related to soft-tissue sarcoma include neurofibromatosis, tuberous sclerosis, basal cell nevus syndrome, Gardner syndrome, and Li-Fraumeni syndrome.
Diagnosis and Evaluation of Extent of Disease
• Core-needle biopsy (large lesions) or excisional biopsy (small lesions).
• Pathological review of histologic subtype, grade, and assessment of margins (excisional biopsies).
• Magnetic resonance imaging or computed tomography (CT) of primary site.
• Chest x-ray for low-grade tumors and high-grade T1 lesions, chest CT for high-grade T2 tumors.
Prognostic Factors
• High-grade histology, deep location, and T2 tumor size are independent adverse prognostic factors for distant metastasis and survival.
• Presentation with recurrent disease and positive surgical margin (gross or microscopic) are independent adverse prognostic factors for local recurrence.
• Individual patient prognosis may be estimated by using a nomogram or newer techniques.
