Background: Epithelioid sarcoma is a rare histologic subtype of sarcom
a. The clinical behavior and prognostic factors influencing survival i
n this disease are examined. Methods: A review of clinicopathologic fe
atures of patients with epithelioid sarcoma prospectively followed bet
ween July 1982 and July 1995 at Memorial Sloan-Kettering Cancer Center
was performed. Kaplan-Meier and log-rank analysis were used. Results:
Eleven men (69%) and five women (31%) were treated during this period
. Mean age at diagnosis was 33 years, and length of symptoms before di
agnosis was 18 months. Tumors presented in the trunk in 44% of patient
s, the lower extremity in 31%, and the upper extremity in 25%. Median
follow-up time was 45 months. At least one local recurrence was experi
enced by 69% of patients. Metastases to regional lymph nodes during th
e course of the disease developed in 44% of patients and to the lungs
in 44%. Median survival was 88.8 months, with a 66% 5-year survival ra
te. Pulmonary metastasis was correlated with decreased survival. Concl
usions: A delay in diagnosis of epithelioid sarcoma is common. Epithel
ioid sarcoma differs from other sarcoma subtypes in propensity for nod
al spread and local recurrence. Careful follow-up evaluating local rec
urrence, nodal spread, and pulmonary metastases is warranted.