Epithelioid sarcoma: a clinicopathologic review of 55 cases

Mayo Clin Proc. 1996 Jul;71(7):636-42. doi: 10.1016/S0025-6196(11)63000-0.


Objective: To identify any clinical and pathologic features of treatment modalities that are predictive of outcome in patients with epithelioid sarcoma, a rare slow-growing soft tissue tumor most commonly occurring in the distal extremities of young adults.

Design: We reviewed the institutional files for cases of epithelioid sarcoma for the period 1956 to 1991 and analyzed the effect of various factors on survival.

Material and methods: Fifty-five cases of epithelioid sarcoma were found, and the relevant clinical, pathologic, treatment, follow-up, and outcome features were assessed.

Results: All tumors were treated initially by operative resection. The recurrence rate progressively decreased with increasing aggressiveness of the initial operation; however, no difference was noted in metastatic rate. Overall, the recurrence rate was 38% and the metastatic rate was 47%. At the end of a mean follow-up of 102 months, 69% of patients had no evidence of disease, 27% had died of the disease, and 4% were alive with disease. Increasing tumor size, necrosis of more than 30%, and vascular invasion correlated significantly with a worse prognosis.

Conclusion: Epithelioid sarcoma should be considered a malignant neoplasm with a significant potential for aggressive behavior, and close follow-up of affected patients should be maintained for many years. Initial treatment should be aggressive in an attempt to prevent recurrence.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Child
  • Female
  • Humans
  • Immunohistochemistry
  • Male
  • Medical Records
  • Middle Aged
  • Necrosis
  • Neoplasm Invasiveness
  • Predictive Value of Tests
  • Prognosis
  • Retrospective Studies
  • Sarcoma* / pathology
  • Sarcoma* / therapy
  • Time Factors
  • Treatment Outcome