General Information About Adult Soft Tissue Sarcoma

Incidence and Mortality
Risk Factors and Genetic Factors
Diagnosis
Prognostic Factors
Related Summaries

Estimated new cases and deaths from soft tissue sarcoma in the United States in 2012:[1]

• New cases: 11,280.
• Deaths: 3,900.

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]

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.[3-5]

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

• 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).

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.

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

• 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.

Although low-grade tumors are usually curable by surgery alone, higher-grade sarcomas (as determined by the mitotic index and by the presence of hemorrhage and necrosis) are associated with higher local-treatment failure rates and increased metastatic potential.

Note: Other PDQ summaries containing information about soft tissue sarcoma include:

• Childhood Soft Tissue Sarcoma Treatment
• Ewing Sarcoma Treatment
• Gastrointestinal Stromal Tumors Treatment
• Kaposi Sarcoma Treatment
• Uterine Sarcoma Treatment

References

1. American Cancer Society.: Cancer Facts and Figures 2012. Atlanta, Ga: American Cancer Society, 2012. Available online. Last accessed September 24, 2012. 
   
   
2. Wibmer C, Leithner A, Zielonke N, et al.: Increasing incidence rates of soft tissue sarcomas? A population-based epidemiologic study and literature review. Ann Oncol 21 (5): 1106-11, 2010.  [PUBMED Abstract]
   
   
3. Singer S, Nielsen T, Antonescu CR: Molecular biology of soft tissue sarcoma. In: DeVita VT Jr, Lawrence TS, Rosenberg SA: Cancer: Principles and Practice of Oncology. 9th ed. Philadelphia, Pa: Lippincott Williams & Wilkins, 2011, pp 1522-32. 
   
   
4. Singer S, Maki RG, O'Sullivan B: Soft tissue sarcoma. In: DeVita VT Jr, Lawrence TS, Rosenberg SA: Cancer: Principles and Practice of Oncology. 9th ed. Philadelphia, Pa: Lippincott Williams & Wilkins, 2011, pp 1533-77. 
   
   
5. Malawer MM, Helman LJ, O'Sullivan B: Sarcomas of bone. In: DeVita VT Jr, Lawrence TS, Rosenberg SA: Cancer: Principles and Practice of Oncology. 9th ed. Philadelphia, Pa: Lippincott Williams & Wilkins, 2011, pp 1578-1609. 
   
   
6. Gustafson P, Dreinhöfer KE, Rydholm A: Soft tissue sarcoma should be treated at a tumor center. A comparison of quality of surgery in 375 patients. Acta Orthop Scand 65 (1): 47-50, 1994.  [PUBMED Abstract]
   
   
7. Coindre JM, Terrier P, Guillou L, et al.: Predictive value of grade for metastasis development in the main histologic types of adult soft tissue sarcomas: a study of 1240 patients from the French Federation of Cancer Centers Sarcoma Group. Cancer 91 (10): 1914-26, 2001.  [PUBMED Abstract]
   
   
8. Vraa S, Keller J, Nielsen OS, et al.: Prognostic factors in soft tissue sarcomas: the Aarhus experience. Eur J Cancer 34 (12): 1876-82, 1998.  [PUBMED Abstract]