Title :

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

Author(s) :

Blay, J. Y. [Auteur]
Université Claude Bernard Lyon 1 [UCBL]
Centre Léon Bérard [Lyon]
Piperno-Neumann, S. [Auteur]
Institut Curie [Paris]
Watson, S. [Auteur]
Institut Curie [Paris]
Dufresne, A. [Auteur]
Université Claude Bernard Lyon 1 [UCBL]
Centre Léon Bérard [Lyon]
Valentin, T. [Auteur]
Institut Claudius Regaud [ICR]
Duffaud, F. [Auteur]
Hôpital de la Timone [CHU - APHM] [TIMONE]
Toulmonde, M. [Auteur]
Institut Bergonié [Bordeaux]
Italiano, A. [Auteur]
Institut Bergonié [Bordeaux]
Bertucci, F. [Auteur]
Institut Paoli-Calmettes [IPC]
Tlemsani, C. [Auteur]
Hôpital Cochin [AP-HP]
Firmin, N. [Auteur]
Institut de Recherche en Cancérologie de Montpellier [IRCM - U1194 Inserm - UM]
Bompas, E. [Auteur]
Institut de Cancérologie de l'Ouest [Angers/Nantes] [UNICANCER/ICO]
Perrin, C. [Auteur]
CRLCC Eugène Marquis [CRLCC]
Ropars, M. [Auteur]
CRLCC Eugène Marquis [CRLCC]
Saada-Bouzid, E. [Auteur]
Centre de Lutte contre le Cancer Antoine Lacassagne [Nice] [UNICANCER/CAL]
Dubray-Longeras, P. [Auteur]
Département d'oncologie médicale [Centre Georges-François Leclerc]
Hervieu, A. [Auteur]
Centre Jean Perrin [Clermont-Ferrand] [UNICANCER/CJP]
Lebbe, C. [Auteur]
Hopital Saint-Louis [AP-HP] [AP-HP]
Gantzer, J. [Auteur]
Les Hôpitaux Universitaires de Strasbourg [HUS]
Chaigneau, L. [Auteur]
Service d'Oncologie Médicale [CHRU Besançon]
Fiorenza, F. [Auteur]
Service d'Oncologie médicale [CHU Limoges]
Rios, M. [Auteur]
Institut de Cancérologie de Lorraine - Alexis Vautrin [Nancy] [UNICANCER/ICL]
Isambert, N. [Auteur]
Centre Régional de Lutte contre le cancer Georges-François Leclerc [Dijon] [UNICANCER/CRLCC-CGFL]
Soibinet, P. [Auteur]
Institut Jean Godinot [Reims]
Boudou-Roquette, P. [Auteur]
Hôpital Cochin [AP-HP]
Verret, B. [Auteur]
Institut Gustave Roussy [IGR]
Ferron, G. [Auteur]
Institut Claudius Regaud [ICR]
Ryckewaert, T. [Auteur]
Centre Hospitalier Régional Universitaire [CHU Lille] [CHRU Lille]
Lebellec, L. [Auteur]
Centre Hospitalier Régional Universitaire [CHU Lille] [CHRU Lille]
Brahmi, M. [Auteur]
Université Claude Bernard Lyon 1 [UCBL]
Centre Léon Bérard [Lyon]
Gouin, F. [Auteur]
Université Claude Bernard Lyon 1 [UCBL]
Centre Léon Bérard [Lyon]
Meeus, P. [Auteur]
Université Claude Bernard Lyon 1 [UCBL]
Centre Léon Bérard [Lyon]
Vaz, G. [Auteur]
Université Claude Bernard Lyon 1 [UCBL]
Centre Léon Bérard [Lyon]
Le Loarer, F. [Auteur]
Institut Bergonié [Bordeaux]
Karanian, M. [Auteur]
Université Claude Bernard Lyon 1 [UCBL]
Centre Léon Bérard [Lyon]
De Pinieux, G. [Auteur]
Centre Hospitalier Régional Universitaire de Tours [CHRU Tours]
Ducimetiere, F. [Auteur]
Université Claude Bernard Lyon 1 [UCBL]
Centre Léon Bérard [Lyon]
Chemin, C. [Auteur]
Université Claude Bernard Lyon 1 [UCBL]
Centre Léon Bérard [Lyon]
Morelle, M. [Auteur]
Université Claude Bernard Lyon 1 [UCBL]
Centre Léon Bérard [Lyon]
Le Cesne, A. [Auteur]
Institut Gustave Roussy [IGR]
Penel, Nicolas [Auteur]
METRICS : Evaluation des technologies de santé et des pratiques médicales - ULR 2694

Journal title :

European Journal of Cancer

Abbreviated title :

Eur J Cancer

Volume number :

192

Pages :

113262

Publication date :

2023-08-27

ISSN :

1879-0852

English keyword(s) :

Epithelioid hemangioendothe-lioma
Sarcoma
Rare cancers
Clinical practice guidelines
Survival
Reference centres

English abstract : [en]

Epithelioid hemangioendothelioma: a nationwide study Epithelioid hemangioendothelioma (EHE) is an ultrarare sarcoma whose natural history and treatment is not well defined. We report on the presentation and outcome of 267 ...
Show more >Epithelioid hemangioendothelioma: a nationwide study Epithelioid hemangioendothelioma (EHE) is an ultrarare sarcoma whose natural history and treatment is not well defined. We report on the presentation and outcome of 267 patients with EHE in the NETSARC+ network since 2010 in France. Patients and methods NETSARC (netsarc.org) is a network of 26 reference sarcoma centres with specialised multidisciplinary tumour boards (MDTB), funded by the French National Cancer Institute (NCI), Institut National du Cancer (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 with stable incidence 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 Two hundred and sixty-seven 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 tumour size was 37 mm (4–220). Forty-eight percent, 42%, and 10% were visceral, soft parts, or bone primaries. The most frequent sites were liver (28%), lung (13%). 40% were reported to have systemic (i.e. multifocal or metastatic disease) at diagnosis. With a median follow-up of 20 months, OS and PFS rates at 24 months were 82% and 67%, with 10-year 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 favourable survival in localised but not in patients with advanced stage at diagnosis. 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 a nationwide series over 12 years that most patients progressed but are still alive at 10 years, both in localised and metastatic stages.Show less >

Language :

Anglais

Audience :

Internationale

Popular science :

Non

Administrative institution(s) :

Université de Lille
CHU Lille

Collections :

• METRICS : Evaluation des technologies de santé et des pratiques médicales - ULR 2694

Submission date :

2024-01-20T22:09:19Z
2024-04-09T09:19:45Z