Annals of Surgical Oncology

, Volume 7, Issue 3, pp 218–225 | Cite as

Epithelioid sarcoma: the clinicopathological complexities of this rare soft tissue sarcoma

  • Andrew J. Spillane
  • J. Meirion Thomas
  • Cyril Fisher



Epithelioid sarcoma is a rare high grade soft tissue sarcoma with a known propensity for locoregional recurrence. The literature is limited on other characteristics such as frequency of multifocal disease at presentation, the relationship of presenting size of the primary lesion to prognosis, and the ability of current staging systems to predict prognosis.


Review of the Royal Marsden National Health Service Trust (RMH) experience of 37 cases over 21 years.


The mean age was 29 years, with male predominance (2.7:1), and distal limb locatinis were most common (56%). Five patients presented with multifocal local disease. Median follow-up was 88 months in the 19 patients still alive. The 5- and 10-year actuarial overall survival was 70% and 42%, respectively. Tumors deep to the investing fascia had a worse prognosis than superficial tumors. Regional metastasis events were also associated with significantly worse overall survival. Local recurrence, size of 5 cm or larger, and regional metastasis events were predictive of worse distant metastasis-free survival. Tumor size (<5 cm vs.≥5 cm), local recurrence events, sex, and site were not significant predictors of survival. The American Joint Committee on Cancer/International Union Against Cancer staging systems and the recently proposed RMH staging system of the Royal Marsden National Health Service Trust provided poor differentiation of prognosis in epithelioid sarcoma. The 5-year actuarial local recurrence rate was 35%. The 5-year actuarial regional nodal metastasis rate was 23%. The actuarial 5-year distant metastasis rate was 40%, with pleuropulmonary metastases the most common site of metastatic disease, and 35% of pleuropulmonary metastases presented with pleural effusion. Median post-distant metastasis survival was 8 months.


Epithelioid sarcoma has unusual clinical behavior compared with other high grade soft tissue sarcoma. It has a propensity for multifocal disease at presentation, local recurrence, regional metastasis, and particularly poor prognosis after regional or distant metastatic disease. Size and stage according to the American Joint Committee on Cancer/International Union Against Cancer are unreliable predictors of prognosis.

Key Words

Soft tissue sarcoma Epithelioid sarcoma Survival 


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Enzinger FM. Epithelioid sarcoma: a sarcoma simulating a granuloma or a carcinoma.Cancer 1970;26:1029–41.PubMedCrossRefGoogle Scholar
  2. 2.
    Fisher C: Epithelioid sarcoma: the spectrum of ultrastructural differentiation in seven immunohistochemically defined cases,Hum Pathol 1988;19:265–75.PubMedGoogle Scholar
  3. 3.
    Smith MEF, Brown JI, Fisher C: Epithelioid sarcoma: presence of vascular-endothelial cadherin and lack of epithelial cadherin.Histopathology 1998;33:425–31.PubMedCrossRefGoogle Scholar
  4. 4.
    Chapter 38. Malignant soft tissue tumours of uncertain type. In: Enzinger FM, Weiss SW, eds.Soft Tissue Tumours, 3rd ed. St. Louis: Mosby, 1995:1067-93.Google Scholar
  5. 5.
    Chase DR, Enzinger FM: Epithelioid sarcoma: diagnosis, prognostic indicators, and treatment.Am J Surg Pathol 1985;9:241–61.PubMedCrossRefGoogle Scholar
  6. 6.
    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;70:862–70.Google Scholar
  7. 7.
    Evans HL, Baer SC: Epithelioid sarcoma: a clinicopathologic and prognostic study of 26 cases.Semin Diagn Pathol 1993;10:286–91.PubMedGoogle Scholar
  8. 8.
    Ross HM, Lewis JJ, Woodruff JM, Brennan MF: Epithelioid sarcoma: clinical behavior and prognostic factors of survival.Ann Surg Oncol 1997;4:491–5.PubMedCrossRefGoogle Scholar
  9. 9.
    Fleming ID, Cooper JS, Henson DE, aet al., deds. Soft tissue sarcoma. In:American Joint Committee on Cancer Staging Manual, 5th ed. Philadelphia: Lippincott-Raven, 1977:149-56.Google Scholar
  10. 10.
    Sobin LH, Wittekind CH, edsUICC TNM Classification of Malignant Tumours, 5th ed. New York: John Wiley, 1997:101–9.Google Scholar
  11. 11.
    Ramanathan RC, A’Hern R, Fisher C, Thomas JM: Modified staging system for extremity soft tissue sarcomas.Ann Surg Oncol 1999;6:57–69.PubMedCrossRefGoogle Scholar
  12. 12.
    Kaplan EL, Meier P: Nonparametric estimation from incomplete observations.J Am Stat Soc 1958;53:457–81.Google Scholar
  13. 13.
    Cox DR: Regression models and life tables (with discussion). J R Stat Soc B 1972;34:187–220.Google Scholar
  14. 14.
    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;41:1472–87.PubMedCrossRefGoogle Scholar
  15. 15.
    Brennan MF: Staging of soft tissue sarcoma (editorial).Ann Surg Oncol 1999;6:8–9.PubMedCrossRefGoogle Scholar
  16. 16.
    Pisters P, Leung D, Woodruff J, Shi W, Brennan MF: Analysis of prognostic factors in 1041 patients with localized soft tissue sarcomas of the extremities.J Clin Oncol 1996;14:1679–89.PubMedGoogle Scholar
  17. 17.
    Pitcher ME, Fish S, Thomas JM: Management of soft tissue sarcoma.Br J Surg 1994;81:1136–9.PubMedGoogle Scholar
  18. 18.
    Fong Y, Coit DG, Woodruff JF, Brennan MF: Lymph node metastasis, from soft tissue sarcoma in adults.Ann Surg 1992;217:72–7.CrossRefGoogle Scholar
  19. 19.
    Suit HD, Russell WO, Martin RG: Management of patients with sarcoma of soft tissues in an extremity.Cancer 1973;31:1247–55.PubMedCrossRefGoogle Scholar
  20. 20.
    Suit HD, Russell WO, Martin RG: Sarcoma of soft tissue: clinical and histopathologic parameters and response to treatment.Cancer 1975;35:1478–83.PubMedCrossRefGoogle Scholar
  21. 21.
    Rosenberg SA, Tepper J, Glatstein E, et al. The treatment of soft-tissue sarcomas of the extremities: prospective randomized evaluations of (1) limb-sparing surgery plus radiation therapy compared with amputation and (2) the role of adjuvant chemotherapy.Ann Surg 1982;196:305–15.PubMedCrossRefGoogle Scholar
  22. 22.
    Shimm DS, Suit HD: Radiation therapy of epithelioid sarcoma.Cancer 1983;52:1022–5.PubMedCrossRefGoogle Scholar
  23. 23.
    Whitworth PW, Pollock RE, Mansfield PF, Couture J, Romsdahl MM: Extremity epithelioid sarcoma: amputation vs local resection.Arch Surg 1991;126:1485–9.PubMedGoogle Scholar
  24. 24.
    Santoro A, Tursz T, Mouridsen H, et al. Doxorubicin versus CY-VADIC versus doxorubicin plus ifosfamide in first-line treatment of advanced soft tissue sarcomas: a randomized study of the European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group.J Clin Oncol 1995;13:7:1537–45.PubMedGoogle Scholar

Copyright information

© The Society of Surgical Oncology, Inc. 2000

Authors and Affiliations

  • Andrew J. Spillane
    • 2
  • J. Meirion Thomas
    • 2
  • Cyril Fisher
    • 1
  1. 1.Department of Anatomical PathologyRoyal Marsden HospitalLondonEngland
  2. 2.Sarcoma UnitRoyal Marsden HospitalLondonUK

Personalised recommendations