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

This study was designed to evaluate the impact of multimodal management on a series of epithelioid sarcoma (ES) patients treated with curative intent.

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).

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).

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.