5013 - Outcome of 98 patients with epithelioid sarcoma treated in curative intent: a retrospective study from the French Sarcoma Group (GSF-GETO)

Background

Epithelioid sarcoma (ES) is a rare soft-tissue sarcoma (STS) (1%). Few recent studies describe pathological and clinical characteristics of ES. Little is known about management and outcomes of patients with localized diseases. The objectives are to study tumor characteristics and clinical management of ES patients with local and locally-advanced disease.

Methods

This is a retrospective study from the nation-wide French sarcoma network (NetSarc). From 2000 to 2016, patients with confirmed ES by expert pathologists, and with a localized disease were included. R2 were excluded.

Results

A total of 98 patients were analysed from 13 centres. Median age was 38 years (range 9-90) with male predominance (56%). The median size was 4 cm (0.3-72). Most common location was distal upper limb (40%). Pre-surgical biopsy was performed in 46% of patients. R0 resection was obtained for 64 patients (79%), R1 for 17 patients (21%). Margins revision surgery has been performed in 51% of patients. Limb-sparing was possible in 73% patients and amputation in 27%. Twenty-five patients (26%) received neoadjuvant treatments mainly chemotherapy with doxorubin-based regimen or isolated limb perfusion in 8 patients (10%). After surgery, 40 patients (43%) received adjuvant treatment, mainly radiotherapy (93%) and 20% received chemotherapy. After a median follow-up of 66.2 months (IC 95 48.4-74.7), 52% patients relapsed : locoregional relapse for 25 patients (52%), distant metastases for 35 patients (75%). The median disease-free-survival (DFS) was 54.5 months (IC95 33.7-105.6). Five-year overall survival (OS) rate was 71% (IC95 59-80). By univariate analyses, locally advanced disease and size larger than 5 cm were identified as a poor-prognosis factors for DFS and OS. R1 surgical margins were prognostic for OS. Proximal location, necrosis, mitosis index had no significant prognostic impact.

Conclusions

ES have a high rate of recurrence, particularly as locoregional relapse. Locally advanced disease, size larger than 5 cm, R1 surgical margins were identified as a poor-prognosis factors.

Clinical trial identification

Editorial acknowledgement

Legal entity responsible for the study

Perrin Christophe.

Funding

Has not received any funding.

Disclosure

All authors have declared no conflicts of interest.