To identify any clinical and pathologic features or 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.
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.
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.
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.