1927P - Epithelioid hemangio-endothelioma (EHE) in NETSARC: The nationwide series of 267 patients over 12 years

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.