TY - JOUR
T1 - Epithelioid hemangio-endothelioma (EHE) in NETSARC
T2 - The nationwide series of 267 patients over 12 years
AU - on behalf of the NETSARC/REPPS/RESOS and French Sarcoma Group–Groupe d′Etude des Tumeurs Osseuses (GSF-GETO) networks
AU - Blay, J. Y.
AU - Piperno-Neumann, S.
AU - Watson, S.
AU - Dufresne, A.
AU - Valentin, T.
AU - Duffaud, F.
AU - Toulmonde, M.
AU - Italiano, A.
AU - Bertucci, F.
AU - Tlemsani, C.
AU - Firmin, N.
AU - Bompas, E.
AU - Perrin, C.
AU - Ropars, M.
AU - Saada-Bouzid, E.
AU - Dubray-Longeras, P.
AU - Hervieu, A.
AU - Lebbe, C.
AU - Gantzer, J.
AU - Chaigneau, L.
AU - Fiorenza, F.
AU - Rios, M.
AU - Isambert, N.
AU - Soibinet, P.
AU - Boudou-Roquette, P.
AU - Verret, B.
AU - Ferron, G.
AU - Ryckewaert, T.
AU - Lebellec, L.
AU - Brahmi, M.
AU - Gouin, F.
AU - Meeus, P.
AU - Vaz, G.
AU - Le Loarer, F.
AU - Karanian, M.
AU - De Pinieux, G.
AU - Ducimetiere, F.
AU - Chemin, C.
AU - Morelle, M.
AU - Le Cesne, A.
AU - Penel, N.
N1 - Publisher Copyright:
© 2023 The Author(s)
PY - 2023/10/1
Y1 - 2023/10/1
N2 - 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.
AB - 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.
KW - Clinical practice guidelines
KW - Epithelioid hemangioendothelioma
KW - Rare cancers
KW - Reference centres
KW - Sarcoma
KW - Survival
UR - http://www.scopus.com/inward/record.url?scp=85171600871&partnerID=8YFLogxK
U2 - 10.1016/j.ejca.2023.113262
DO - 10.1016/j.ejca.2023.113262
M3 - Article
C2 - 37625241
AN - SCOPUS:85171600871
SN - 0959-8049
VL - 192
JO - European Journal of Cancer
JF - European Journal of Cancer
M1 - 113262
ER -