from PART III - ORGAN-SPECIFIC CANCERS
Published online by Cambridge University Press: 18 May 2010
SCOPE
Soft-tissue sarcomas comprise roughly 1% of all malignant tumors and occur in about 8300 patients in the United States each year (1). In spite of progress in the diagnosis and treatment of sarcomas, still, more than 50% of patients die annually from this rare malignancy (2). Two thousand of these tumors arise in bone. The remainder mainly arise from mesodermal soft tissues anywhere in the body, with 60% of these soft-tissue sarcomas being found in the extremities. Most sarcomas are sporadic, but occasionally they are associated with trauma, foreign bodies, chemical carcinogens, radiation or genetic disorders such as neurofibromatosis, Gardner's syndrome, certain gene mutations or chromosomal abnormalities. A viral etiology exists for several animal sarcomas, with Kaposi's sarcoma being the most common virally induced sarcoma in humans.
At present, the relevant prognostic factors for soft-tissue sarcoma as defined in the stage classification system of the American Joint Committee on Cancer (AJCC) include grade, size and depth relative to the superficial investing muscular fascia (3). In addition to the aforementioned factors, large prospective databases relate the treatment and prognosis of soft-tissue sarcomas to location (4, 5), microscopic margin positivity (6), histopathologic subtype (7) and presentation status (primary tumor vs. local recurrence). Sarcomas are classified histologically as low or high grade, with high-grade tumors being more aggressive and therefore more likely to recur locally or distantly after resection of the primary. Tumors larger than 5 cm have a worse prognosis than tumors smaller than 5 cm.
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