Epithelioid sarcoma: need for a multimodal approach to maximize the chances of curative conservative treatment.

OBJECTIVE: This study was designed to evaluate the impact of multimodal management on a series of epithelioid sarcoma (ES) patients treated with curative intent.
METHODS: Data were collected on 69 consecutive patients treated from 1982 to 2012. Univariate and multivariate analyses were performed for tumor control and overall survival (OS).
RESULTS: In total, 54 (78 %) patients had localized ES (M0 group). In the M0 group, 85 % of patients received multimodal management (surgery n = 50, radiotherapy n = 37, chemotherapy n = 30). Among 42 patients with limb ES, 9 (21 %) underwent amputation, and isolated limb perfusion (ILP) was required in 17 (40.5 %) to allow conservative management. Among the 45 patients who underwent conservative surgery, flap reconstructions were required in 13 (28.8 %). The median follow-up was 5.7 years. The 5-year actuarial OS rates were 54, 62, and 24 % in the entire group and the M0 and M1 groups, respectively. In the M0 group, the 5-year actuarial distant control, local control (LC), and locoregional control rates were 67, 75, and 66 %, respectively. Prognostic factors for poor OS in the multivariate analysis were tumors that were deep to the fascia (p = 0.04) and grade 3 (p = 0.005). In the univariate analysis, age <30 years (p = 0.04), the T2 stage (p = 0.04), and mass presentation (p = 0.03) correlated with decreased LC, whereas patients who underwent ILP had a significantly higher LC rate (hazard ratio 3; 95 % confidence interval 0.9-9.4; p = 0.05).
CONCLUSIONS: Multimodal management including ILP and flap reconstruction is necessary to achieve optimal conservative LC. High rates of metastasis and lymphatic spread require innovative systemic treatments.