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
3245 - BCR-ABL transcript variant’s significance in chronic myeloid leukemia in chronic phase: Institutional experience from a developing country
Presenter: Siva Prasad
Session: Poster Display session 1
Resources:
Abstract
2831 - Interleukin-6 as a Predictive Marker for Early Response to Induction Chemotherapy in Acute Myeloblastic Leukaemia
Presenter: Salah Khallaf
Session: Poster Display session 1
Resources:
Abstract
6014 - Impact of FLT3-ITD mutation on post-transplant outcome of adult AML is modified by the concomitant NPM1 mutation and pre-transplant remission status: A report from Taiwan Bone Marrow Transplant Registry (TBMTR)
Presenter: Su-peng Yeh
Session: Poster Display session 1
Resources:
Abstract
2914 - Efficacy endpoints studied in clinical trials for early-onset leukaemia
Presenter: Dylan Said
Session: Poster Display session 1
Resources:
Abstract
4691 - High triglyceride is a major risk factor of DIC and differentiation syndrome in acute promyelocytic leukemia
Presenter: Tomohiro Yamakawa
Session: Poster Display session 1
Resources:
Abstract
4395 - DREAMM 4: A Phase I/II single-arm open-label study to explore safety and clinical activity of belantamab mafodotin (GSK2857916) administered in combination with pembrolizumab in patients with relapsed/refractory multiple myeloma (RRMM)
Presenter: Suzanne Trudel
Session: Poster Display session 1
Resources:
Abstract
2808 - A Phase 1 Study of HMPL-523, a Selective Oral Anti-Spleen Tyrosine Kinase Inhibitor, in Patients with Relapsed or Refractory Lymphoma
Presenter: Nathan Fowler
Session: Poster Display session 1
Resources:
Abstract
4403 - A Phase 1 Study of HMPL-689, a Selective Oral Phosphoinositide 3-Kinase-Delta Inhibitor, in Patients with Relapsed or Refractory Lymphoma
Presenter: Jonathon Cohen
Session: Poster Display session 1
Resources:
Abstract
3357 - A global patient-driven Facebook study in a very rare sarcoma: Health-related quality of life in Epithelioid Hemangioendothelioma (EHE) patients
Presenter: Marije Weidema
Session: Poster Display session 1
Resources:
Abstract
2475 - Qualitative study of patients’ experiences of living with and beyond a soft tissue sarcoma diagnosis: the impact of sarcoma specialist services
Presenter: Ana Martins
Session: Poster Display session 1
Resources:
Abstract