Prognostic Factors for Survival in Patients with Epithelioid Sarcoma: 441 Cases from the SEER Database

  • Muhammad Umar Jawad
  • Jason Extein
  • Elijah S. Min
  • Sean P. ScullyEmail author
Original Article


Current stratification of prognosis in patients with epithelioid sarcoma (ES) is based largely on data reported by individual centers with a limited number of patients. We sought to identify the important prognostic parameters using the Surveillance, Epidemiology, and End Results (SEER) database. We identified 441 patients with ES in the database and extracted information regarding patient demographics and clinical characteristics. Kaplan-Meier, log-rank, and Cox regression were used for analysis. Disease-specific survival declined until 100 months after diagnosis after which survival was unrelated to epithelioid sarcoma. The overall incidence of ES during 2005 was 0.041 per 100,000. The reported incidence has increased since 1973, with an annual percentage change of 5.217%. On multivariate analysis, only age younger than 16 years, local stage of disease, or negative nodes and surgical resection of the tumor predicted better disease-specific survival. We observed no increase in survival by comparing decades of diagnosis since 1986. The SEER database shows only age younger than 16 years, negative nodes, or local stage of disease and operability of primary disease independently predict survival in patients with ES.

Level of Evidence: Level II, prognostic study. See Guidelines for Authors for a complete description of levels of evidence.


Sentinel Node Soft Tissue Sarcoma Distant Disease Annual Percentage Change Sentinel Node Dissection 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. 1.
    American Joint Committee on Cancer. AJCC Cancer Staging Manual. 6th ed. New York, NY: Springer; 2003.Google Scholar
  2. 2.
    Bach PB, Guadagnoli E, Schrag D, Schussler N, Warren JL. Patient demographic and socioeconomic characteristics in the SEER-Medicare database applications and limitations. Med Care. 2002;40(8 suppl):IV-19–25.Google Scholar
  3. 3.
    Baratti D, Pennacchioli E, Casali PG, Bertulli R, Lozza L, Olmi P, Collini P, Radaelli S, Fiore M, Gronchi A. Epithelioid sarcoma: prognostic factors and survival in a series of patients treated at a single institution. Ann Surg Oncol. 2007;14:3542–3551.PubMedCrossRefGoogle Scholar
  4. 4.
    Bliss BO, Reed RJ. Large cell sarcomas of tendon sheath. Am J Clin Pathol. 1968;49:776–781.PubMedGoogle Scholar
  5. 5.
    Bos GD, Pritchard DJ, Reiman HM, Dobyns JH, Ilstrup DM, Landon GC. Epithelioid sarcoma: an analysis of fifty-one cases. J Bone Joint Surg Am. 1988;70:862–870.PubMedGoogle Scholar
  6. 6.
    Callister MD, Ballo MT, Pisters PW, Patel SR, Feig BW, Pollock RE, Benjamin RS, Zagars GK. Epithelioid sarcoma: results of conservative surgery and radiotherapy. Int J Radiat Oncol Biol Phys. 2001;51:384–391.PubMedGoogle Scholar
  7. 7.
    Casanova M, Ferrari A, Collini P, Bisogno G, Alaggio R, Cecchetto G, Gronchi A, Meazza C, Garaventa A, Di Cataldo A, Carli M. Epithelioid sarcoma in children and adolescents: a report from the Italian Soft Tissue Sarcoma Committee. Cancer. 2006;106:708–717.PubMedCrossRefGoogle Scholar
  8. 8.
    Chase DR, Enzinger FM. Epithelioid sarcoma: diagnosis, prognostic indicators, and treatment. Am J Surg Pathol. 1985;9:241–263.PubMedCrossRefGoogle Scholar
  9. 9.
    Cheung MC, Perez EA, Molina MA, Jin X, Gutierrez JC, Franceschi D, Livingstone AS, Koniaris LG. Defining the role of surgery for primary gastrointestinal tract melanoma. J Gastrointest Surg. 2008;12:731–738.PubMedCrossRefGoogle Scholar
  10. 10.
    Cooper GS, Virnig B, Klabunde CN, Schussler N, Freeman J, Warren JL. Use of SEER-Medicare data for measuring cancer surgery. Med Care. 2002;40(8 suppl):IV-43–48.Google Scholar
  11. 11.
    de Visscher SA, van Ginkel RJ, Wobbes T, Veth RP, Ten Heuvel SE, Suurmeijer AJ, Hoekstra HJ. Epithelioid sarcoma: still an only surgically curable disease. Cancer. 2006;107:606–612.PubMedCrossRefGoogle Scholar
  12. 12.
    Earle CC, Nattinger AB, Potosky AL, Lang K, Mallick R, Berger M, Warren JL. Identifying cancer relapse using SEER-Medicare data. Med Care. 2002;40(8 suppl):IV-75–81.Google Scholar
  13. 13.
    Evans HL, Baer SC. Epithelioid sarcoma: a clinicopathologic and prognostic study of 26 cases. Semin Diagn Pathol. 1993;10:286–291.PubMedGoogle Scholar
  14. 14.
    Fong Y, Coit DG, Woodruff JM, Brennan MF. Lymph node metastasis from soft tissue sarcoma in adults: analysis of data from a prospective database of 1772 sarcoma patients. Ann Surg. 1993;217:72–77.PubMedCrossRefGoogle Scholar
  15. 15.
    Gloeckler Ries LA, Reichman ME, Lewis DR, Hankey BF, Edwards BK. Cancer survival and incidence from the Surveillance, Epidemiology, and End Results (SEER) program. Oncologist. 2003;8:541–552.PubMedCrossRefGoogle Scholar
  16. 16.
    Gross E, Rao BN, Pappo A, Bowman L, Shearer P, Kaste S, Greenwald C, Michalkiewicz E, Pratt C. Epithelioid sarcoma in children. J Pediatr Surg. 1996;31:1663–1665.PubMedCrossRefGoogle Scholar
  17. 17.
    Gutierrez JC, De Oliveira LO, Perez EA, Rocha-Lima C, Livingstone AS, Koniaris LG. Optimizing diagnosis, staging, and management of gastrointestinal stromal tumors. J Am Coll Surg. 2007;205:479–491 (Quiz 524).PubMedCrossRefGoogle Scholar
  18. 18.
    Gutierrez JC, Fischer AC, Sola JE, Perez EA, Koniaris LG. Markedly improving survival of neuroblastoma: a 30-year analysis of 1,646 patients. Pediatr Surg Int. 2007;23:637–646.PubMedCrossRefGoogle Scholar
  19. 19.
    Gutierrez JC, Franceschi D, Koniaris LG. How many lymph nodes properly stage a periampullary malignancy? J Gastrointest Surg. 2008;12:77–85.PubMedCrossRefGoogle Scholar
  20. 20.
    Gutierrez JC, Perez EA, Franceschi D, Moffat FL Jr, Livingstone AS, Koniaris LG. Outcomes for soft-tissue sarcoma in 8249 cases from a large state cancer registry. J Surg Res. 2007;141:105–114.PubMedCrossRefGoogle Scholar
  21. 21.
    Halling AC, Wollan PC, Pritchard DJ, Vlasak R, Nascimento AG. Epithelioid sarcoma: a clinicopathologic review of 55 cases. Mayo Clin Proc. 1996;71:636–642.PubMedCrossRefGoogle Scholar
  22. 22.
    Hayat MJ, Howlader N, Reichman ME, Edwards BK. Cancer statistics, trends, and multiple primary cancer analyses from the Surveillance, Epidemiology, and End Results (SEER) Program. Oncologist. 2007;12:20–37.PubMedCrossRefGoogle Scholar
  23. 23.
    Herr MJ, Harmsen WS, Amadio PC, Scully SP. Epithelioid sarcoma of the hand. Clin Orthop Relat Res. 2005;431:193–200.PubMedCrossRefGoogle Scholar
  24. 24.
    Hodgson N, Koniaris LG, Livingstone AS, Franceschi D. Gastric carcinoids: a temporal increase with proton pump introduction. Surg Endosc. 2005;19:1610–1612.PubMedCrossRefGoogle Scholar
  25. 25.
    International Agency for Research on Cancer. International Classification of Diseases for Oncology. 3rd ed. Geneva, Switzerland: World Health Organization; 2000.Google Scholar
  26. 26.
    Lee DJ, Voti L, Mackinnon J, Koniaris LG, Fleming LE, Huang Y, Wohler B, Franceschi D, Dietz NA, Sherman R, Soler-Vila H. Gender- and race-specific comparison of tobacco-associated cancer incidence trends in Florida with SEER regional cancer incidence data. Cancer Causes Control. 2008;19:711–723.PubMedCrossRefGoogle Scholar
  27. 27.
    Livi L, Shah N, Paiar F, Fisher C, Judson I, Moskovic E, Thomas M, Harmer C. Treatment of epithelioid sarcoma at the Royal Marsden Hospital. Sarcoma. 2003;7:149–152.PubMedCrossRefGoogle Scholar
  28. 28.
    Matsushita Y, Ahmed AR, Kawaguchi N, Matsumoto S, Manabe J. Epithelioid sarcoma of the extremities: a dismal long-term outcome. J Orthop Sci. 2002;7:462–466.PubMedCrossRefGoogle Scholar
  29. 29.
    Merrill RM, Dearden KA. How representative are the surveillance, epidemiology, and end results (SEER) program cancer data of the United States? Cancer Causes Control. 2004;15:1027–1034.PubMedCrossRefGoogle Scholar
  30. 30.
    National Cancer Institute. Available at: Accessed December 1, 2008.
  31. 31.
    Perez EA, Gutierrez JC, Jin X, Lee DJ, Rocha-Lima C, Livingstone AS, Franceschi D, Koniaris LG. Surgical outcomes of gastrointestinal sarcoma including gastrointestinal stromal tumors: a population-based examination. J Gastrointest Surg. 2007;11:114–125.PubMedCrossRefGoogle Scholar
  32. 32.
    Perez EA, Livingstone AS, Franceschi D, Rocha-Lima C, Lee DJ, Hodgson N, Jorda M, Koniaris LG. Current incidence and outcomes of gastrointestinal mesenchymal tumors including gastrointestinal stromal tumors. J Am Coll Surg. 2006;202:623–629.PubMedCrossRefGoogle Scholar
  33. 33.
    Petrelli NJ. SEER data: it can be thought provoking, but where do we go from here? Ann Surg Oncol. 2007;14:2173–2174.PubMedCrossRefGoogle Scholar
  34. 34.
    Potosky AL, Warren JL, Riedel ER, Klabunde CN, Earle CC, Begg CB. Measuring complications of cancer treatment using the SEER-Medicare data. Med Care. 2002;40(8 suppl):IV-62–68.Google Scholar
  35. 35.
    Prat J, Woodruff JM, Marcove RC. Epithelioid sarcoma: an analysis of 22 cases indicating the prognostic significance of vascular invasion and regional lymph node metastasis. Cancer. 1978;41:1472–1487.PubMedCrossRefGoogle Scholar
  36. 36.
    Ross HM, Lewis JJ, Woodruff JM, Brennan MF. Epithelioid sarcoma: clinical behavior and prognostic factors of survival. Ann Surg Oncol. 1997;4:491–495.PubMedCrossRefGoogle Scholar
  37. 37.
    Schrag D, Bach PB, Dahlman C, Warren JL. Identifying and measuring hospital characteristics using the SEER-Medicare data and other claims-based sources. Med Care. 2002;40(8 suppl):IV-96–103.Google Scholar
  38. 38.
    Shwartz M, Pliskin JS, Grondahl HG, Boffa J. Use of the Kaplan-Meier estimate to reduce biases in estimating the rate of caries progression. Community Dent Oral Epidemiol. 1984;12:103–108.PubMedCrossRefGoogle Scholar
  39. 39.
    Spillane AJ, Thomas JM, Fisher C. Epithelioid sarcoma: the clinicopathological complexities of this rare soft tissue sarcoma. Ann Surg Oncol. 2000;7:218–225.PubMedCrossRefGoogle Scholar
  40. 40.
    Virnig BA, Warren JL, Cooper GS, Klabunde CN, Schussler N, Freeman J. Studying radiation therapy using SEER-Medicare-linked data. Med Care. 2002;40(8 suppl):IV-49–54.Google Scholar
  41. 41.
    Warren JL, Harlan LC, Fahey A, Virnig BA, Freeman JL, Klabunde CN, Cooper GS, Knopf KB. Utility of the SEER-Medicare data to identify chemotherapy use. Med Care. 2002;40(8 suppl):IV-55–61.Google Scholar
  42. 42.
    Warren JL, Klabunde CN, Schrag D, Bach PB, Riley GF. Overview of the SEER-Medicare data: content, research applications, and generalizability to the United States elderly population. Med Care. 2002;40(8 suppl):IV-3–18.Google Scholar
  43. 43.
    Wingo PA, Jamison PM, Hiatt RA, Weir HK, Gargiullo PM, Hutton M, Lee NC, Hall HI. Building the infrastructure for nationwide cancer surveillance and control: a comparison between the National Program of Cancer Registries (NPCR) and the Surveillance, Epidemiology, and End Results (SEER) Program (United States). Cancer Causes Control. 2003;14:175-193.PubMedCrossRefGoogle Scholar
  44. 44.
    Wolf PS, Flum DR, Tanas MR, Rubin BP, Mann GN. Epithelioid sarcoma: the University of Washington experience. Am J Surg. 2008;196:407–412.PubMedCrossRefGoogle Scholar

Copyright information

© The Association of Bone and Joint Surgeons 2009

Authors and Affiliations

  • Muhammad Umar Jawad
    • 1
  • Jason Extein
    • 2
  • Elijah S. Min
    • 3
  • Sean P. Scully
    • 1
    Email author
  1. 1.Department of OrthopaedicsUniversity of Miami Miller School of Medicine, University of Miami HospitalMiamiUSA
  2. 2.Department of SurgeryWeill Cornell Medical CollegeNew YorkUSA
  3. 3.Ross University School of MedicineNorth BrunswickUSA

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