Abstract 1927P
Background
Epithelioid hemangioendothelioma (EHE) is an ultrarare sarcoma whose natural history and treatment are not well defined. We report on the presentation and outcome of the nationwide exhaustive series of 267 patients with EHE in the NETSARC+ network since 2010 in France.
Methods
NETSARC (netsarc.org) is a network of 26 reference sarcoma centers with specialized multidisciplinary tumor boards (MDTB), funded by the French NCI (INCA). Since 2010, presentation to an MDTB and second pathological review are mandatory for sarcoma patients. Patients’ characteristics are collected in a nationwide database regularly monitored. Exhaustivity in the collection of cases was reached since 2013. The characteristics of patients with EHE at diagnosis are presented as well as progression-free survival (PFS), overall survival (OS), and outcome under treatment.
Results
267 patients with EHE were included in the NETSARC+ database since 2010. Median age in the series was 51 (range 10-90) years, 58% were women. Median tumor size was 37mm (4-220). 48%, 42%, and 10% were visceral, soft parts, or bone primaries. The most frequent sites were liver (28%), lung (13%). 40% were reported as metastatic at diagnosis. With a median follow-up of 20 months, OS and PFS rates at 24 months were 82% and 67%, with 10-years projected OS and PFS of 62% and 21% respectively. Male and M+ patients at diagnosis had a significantly worse OS, but not PFS. Local treatment was associated with a favorable survival in localized but not in advanced stage. For 23 patients receiving medical treatment, PFS and OS were 50.2% and 33.2% at 60 months were respectively.
Conclusions
EHE is a frequently metastatic sarcoma at diagnosis with a unique natural history. This study shows in an exhaustive nationwide series over 12 years that most patients progressed but are still alive at 10 years, both in localized and metastatic stages.
Clinical trial identification
Editorial acknowledgement
Legal entity responsible for the study
Centre Leon Bérard for NETSARC+ network.
Funding
NetSARC+ (INCA & DGOS) and RREPS (INCA & DGOS), RESOS (INCA & DGOS), LYRICAN+ (INCA-DGOS-INSERM 12563), Eurosarc (FP7-278742), InterSARC (INCA), LabEx DEvweCAN (ANR-10-LABX-0061), EURACAN (EC 739521), Association DAM’s, la Fondation ARC, Infosarcome, Ligue de L’Ain contre le Cancer, La Ligue contre le Cancer funded this study.
Disclosure
All authors have declared no conflicts of interest.
Resources from the same session
1930P - phase II trial of avelumab in combination with gemcitabine in advanced leiomyosarcoma as a second-line treatment (KCSG UN18-06)
Presenter: Young Saing Kim
Session: Poster session 15
1931P - Ultra-rare sarcomas have worse survival compared to non-ultra-rare sarcomas: A national cancer registry study
Presenter: MIng-Jing Lee
Session: Poster session 15
1932P - First in human trial of CEB-01 for retroperitoneal soft tissue sarcoma
Presenter: Jose Antonio González
Session: Poster session 15
1933P - Perioperative chemotherapy could reduce the risk of recurrence in resected leiomyosarcoma of the vena cava (LMS-VC): A retrospective bi-centric series on 41 patients
Presenter: Thibaud Bertrand
Session: Poster session 15
1934P - Pharmacokinetics of unesbulin in a phase Ib study of patients with advanced leiomyosarcoma
Presenter: Brian Van Tine
Session: Poster session 15
1935P - Exploration of tertiary lymphatic structure in soft tissue sarcoma for the prognostic and immunotherapy response
Presenter: Wang Xiang-Xu
Session: Poster session 15
1936P - Targeted therapy of desmoplastic small round cell tumor guided by multilayered molecular profiling
Presenter: Marcus Renner
Session: Poster session 15
1937P - KM-subtraction meta-analysis of randomized controlled trials of adjuvant chemotherapy for localized resectable soft-tissue sarcoma
Presenter: Qin Jian Low
Session: Poster session 15
1938P - Assessment of clinical benefit of cancer drugs recommended in National Comprehensive Cancer Network (NCCN) guidelines for advanced soft tissue sarcomas (STS) and bone sarcomas (BS)
Presenter: Maria Aguado Sorolla
Session: Poster session 15
1939P - phase I clinical results of SQ3370, a doxorubicin-based click chemistry therapeutic in advanced solid tumor patients
Presenter: Sant Chawla
Session: Poster session 15