Epidemiology

Incidence and Prevalence of Soft Tissue Sarcoma

• The estimates for new cases of and annual deaths from soft tissue sarcoma in adults and children in the United States were 13,040 and 5,150, respectively.¹
• Reported international incidence of soft tissue sarcoma ranges between 1.8 and 5 cases per 100,000 individuals per year.²
• Soft tissue sarcomas may arise in the extremities (50%), torso and retroperitoneum (40%), or head and neck (10%).³
• Sarcomas account for over 15% of all childhood cancers and 1% of all adult cancers.
• About 58% of sarcomas are localized at diagnosis, 18% are regional, and 14% are distant or metastatic.

Risk Factors for Soft Tissue Sarcoma^4-6

• The risk of sporadic soft tissue sarcomas is increased by previous radiation therapy.
• Chronic lymphedema is associated with increased risk of lymphangiosarcoma.
• Hepatic angiosarcoma is associated with exposure to thorium dioxide (Thorotrast), vinyl chloride, and arsenic.
• HIV and human herpes 8 increase the risk of Kaposi sarcoma.
• Several inherited syndromes are associated with an increased risk of soft tissue sarcoma:
  • Gardner syndrome (APC mutation)
  • Li-Fraumeni syndrome (TP53 mutation)
  • Nevoid basal cell carcinoma syndrome (Gorlin syndrome: PTC mutation)
  • Tuberous sclerosis (Bourneville disease: TSC1 or TSC2 mutations)
  • von Recklinghausen disease (neurofibromatosis type 1: NF1 mutation)
  • Werner syndrome (adult progeria: WRN mutation)

Prognosis

• Median overall survival for patients with metastatic soft tissue sarcoma is 12 to 16 months.⁷
• Up to 50% of high-risk patients with high-grade soft tissue sarcoma develop metastases and die from their disease.⁸
• Soft tissue sarcoma is one of the top five causes of cancer-related deaths in patients under 20 years old.⁹

National Cancer Institute 5-year Relative Survival Rates:¹⁰

• 65% for all sarcoma patients, regardless of stage
• 81% for localized sarcomas
• 58% for regional sarcomas
• 16% for distant sarcomas

Memorial Sloan-Kettering Cancer Center 5-year Observed Survival Rates for Sarcomas of the Arms and Legs:¹¹

• 90% for stage I sarcomas
• 81% for stage II sarcomas
• 56% for stage III sarcomas
• No data available for stage IV sarcomas

The 10-year survival rates for soft tissue sarcoma are only slightly worse than the 5-year survival rates.

Factors that indicate a better prognosis include:

• Tumors less than 5 cm
• Low-grade tumors
• Superficial tumors
• Certain types of sarcomas, such as liposarcomas
• Earlier stage at diagnosis
• Sarcomas of the arms and legs
• Negative surgical margins
• Patients under 60 years old

REFERENCES

 

1. American Cancer Society: Cancer Facts and Figures 2018. Atlanta, GA: American Cancer Society, 2018. https://www.cancer.org/content/dam/cancer-org/research/cancer-facts-and-statistics/annual-cancer-facts-and-figures/2018/cancer-facts-and-figures-2018.pdf. Accessed March 14, 2019.
2. PDQ Cancer Information Treatment (PDQ). Health Professional Version. Adult Soft Tissue Carcinoma Treatment (PDQ). https://www.ncbi.nlm.nih.gov/books/NBK66046/. Bethesda (MD): National Cancer Institute (US); 2002-2019 Jan 2019. Accessed March 14, 2019.
3. 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. 2010;21:1106-1111.
4. 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 1522-1532.
5. 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 1533-1577.
6. 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.
7. Tap W, Jones R, Van Tine B, et al. Olaratumab and doxorubicin versus doxorubicin alone for treatment of soft-tissue sarcoma: an open-label phase 1b and randomized phase 2 trial. Lancet. 2016;388:488-497.
8. In G, Hu J, Tseng W. Treatment of advanced, metastatic soft tissue sarcoma: latest evidence and clinical consideration. Ther Adv Med Oncol. 2017;9:533-550.
9. Siegel R, Miller K, Jemal A. Cancer statistics, 2016. CA Cancer J Clin. 2016;66:7-30.
10. SEER Cancer Stat Facts: Soft Tissue including Heart Cancer. National Cancer Institute. Bethesda, MD. https://seer.cancer.gov/statfacts/html/soft.html. Accessed March 14, 2019.
11. American Cancer Society. Survival by Stage of Soft Tissue Sarcoma. https://www.cancer.org/cancer/soft-tissue-sarcoma/detection-diagnosis-staging/survival-rates.html#references. Accessed March 14, 2019.