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Adult Soft Tissue Sarcoma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - General Information About Adult Soft Tissue Sarcoma

Incidence and Mortality

Estimated new cases and deaths from soft tissue sarcoma in the United States in 2013:[1]

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  • New cases: 11,410.
  • Deaths: 4,390.

Soft tissue sarcomas are malignant tumors that arise in any of the mesodermal tissues of the extremities (50%), trunk and retroperitoneum (40%), or head and neck (10%). The reported international incidence rates range from 1.8 to 5 per 100,000 per year.[2]

Risk Factors and Genetic Factors

The risk of sporadic soft tissue sarcomas is increased by prior radiation therapy and, in the case of lymphangiosarcoma, by chronic lymphedema. The chemicals Thorotrast, vinyl chloride, and arsenic are also established carcinogens for hepatic angiosarcomas.[3,4,5]

Soft tissue sarcomas occur with greater frequency in patients with the following inherited syndromes:[3,4,5]

  • Nevoid basal cell carcinoma syndrome (Gorlin syndrome: PTC gene mutation).
  • Gardner syndrome (APC mutation).
  • Li-Fraumeni syndrome (p53 mutation).
  • Tuberous sclerosis (Bourneville disease: TSC1 or TSC2 mutation).
  • von Recklinghausen disease (neurofibromatosis type 1: NF1 mutation).
  • Werner syndrome (adult progeria: WRN mutation).

Diagnosis

Soft tissue sarcomas may be heterogeneous, so adequate tissue should be obtained via either core-needle or incisional biopsy for microscopic examination to determine histologic type and tumor grade. Careful planning of the initial biopsy is important to avoid compromising subsequent curative resection. Since the selection of treatment is determined by the grade of the tumor, it is essential to have a careful review of the biopsy tissue by a pathologist who is experienced in diagnosing sarcomas. Complete staging and treatment planning by a multidisciplinary team of cancer specialists is required to determine the optimal treatment for patients with this disease.

There is evidence that at least some favorable clinical outcomes may be associated with referral to a specialized sarcoma treatment center. In a population-based consecutive series of 375 soft tissue sarcoma patients in Sweden, local recurrence rates of resected tumors were higher in patients who were not referred to the specialized center: in 35 of 78 (45%) patients not referred; in 24 of 102 (24%) patients referred after initial surgery or incisional biopsy; and in 36 of 195 (18%) patients referred prior to any surgical procedure (P = .0001 for the difference between those never referred vs. those referred prior to any surgical procedure).[6][Level of evidence: 3iDii] However, there were no statistically significant differences in death from sarcoma between the groups of patients.

Prognostic Factors

The prognosis for patients with adult soft tissue sarcomas depends on several factors, including:[3,4,5,7]

  • Patient's age.
  • Size, histologic grade, mitotic activity, and stage of the tumor.

Factors associated with a poorer prognosis include the following:[8]

  • Age older than 60 years.
  • Tumors larger than 5 cm in greatest dimension.
  • High-grade histology with high mitotic activity.
1|2

WebMD Public Information from the National Cancer Institute

Last Updated: February 25, 2014
This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.
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