Epithelioid sarcoma: Clinical behavior and prognostic factors of survival

Abstract

Background: Epithelioid sarcoma is a rare histologic subtype of sarcoma. The clinical behavior and prognostic factors influencing survival in this disease are examined. Methods: A review of clinicopathologic features of patients with epithelioid sarcoma prospectively followed between 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 diagnosis was 18 months. Tumors presented in the trunk in 44% of patients, the lower extremity in 31%, and the upper extremity in 25%. Median follow-up time was 45 months. At least one local recurrence was experienced by 69% of patients. Metastases to regional lymph nodes during the 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 rate. Pulmonary metastasis was correlated with decreased survival. Conclusions: A delay in diagnosis of epithelioid sarcoma is common. Epithelioid sarcoma differs from other sarcoma subtypes in propensity for nodal spread and local recurrence. Careful follow-up evaluating local recurrence, nodal spread, and pulmonary metastases is warranted.