Abstract 5013
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.
Resources from the same session
2640 - Health-related quality of life issues of patients affected by desmoid-type fibromatosis; experiences from two countries
Presenter: Milea Timbergen
Session: Poster Display session 1
Resources:
Abstract
1269 - One-year follow-up results of eribulin for soft-tissue sarcoma including rare subtypes in a real-world observational study in Japan
Presenter: Shunji Takahashi
Session: Poster Display session 1
Resources:
Abstract
2868 - Prevalence of chemotherapy use and its impact on overall survival in patients with bone- and soft tissue sarcomas -A population-based analysis of 3746 patients
Presenter: Herbert Loong
Session: Poster Display session 1
Resources:
Abstract
3042 - Clinical course and therapeutic management of classical and endemic Kaposi’s Sarcoma (C/E KS)
Presenter: Lina Benajiba
Session: Poster Display session 1
Resources:
Abstract
3141 - The final outcomes of study on combined therapy of adult patients with localized synovial sarcoma
Presenter: Katarzyna Kozak
Session: Poster Display session 1
Resources:
Abstract
5449 - Real-world Outcomes of Patients with Locally Advanced or Metastatic Epithelioid Sarcoma
Presenter: Mrinal Gounder
Session: Poster Display session 1
Resources:
Abstract
4465 - SAKK 57/16 Nab-Paclitaxel And Gemcitabine in soft tissue sarcoma (NAPAGE): results from the phase I part of a phase I/II trial
Presenter: Antonia Digklia
Session: Poster Display session 1
Resources:
Abstract
5614 - Comparison of filgrastim and pegfilgrastim prophylaxis in sarcoma patients receiving highly myelosuppressive chemotherapy.
Presenter: Paolo Tarantino
Session: Poster Display session 1
Resources:
Abstract
1033 - Access to clinical trials for soft tissue sarcoma patients in Western and Eastern Europe
Presenter: Vasilii Ostafiichuk
Session: Poster Display session 1
Resources:
Abstract
4094 - Treatment outcomes for adult patients with localized osteosarcoma treated with chemotherapy without methotrexate
Presenter: Marília Silva
Session: Poster Display session 1
Resources:
Abstract