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

• DOI: 10.1016/j.ejca.2023.113262
• Publication Date: 2023-07-29T15:28:51Z
• Authors: JY Blay; S. Piperno-Neumann; S. Watson; A. Dufresne; T. Valentin; F. Duffaud; M. Toulmonde; A. Italiano; F. Bertucci; C. Tlemsani; N. Firmin; E. Bompas; C. Perrin; M. Ropars; E. Saada-Bouzid; P. Dubray-Longeras; A. Hervieu; C. Lebbe; J. Gantzer; L. Chaigneau; F. Fiorenza; M. Rios; N. Isambert; P. Soibinet; P. Boudou-Roquette; B. Verret; G. Ferron; T. Ryckewaert; L. Lebellec; M. Brahmi; F. Gouin; P. Meeus; G. Vaz; F. Le Loarer; M. Karanian; G. De Pinieux; F. Ducimetiere; C. Chemin; M. Morelle; A. Le Cesne; N. Penel
• Keywords: Male; Adult; Aged, 80 and over; Adolescent; Databases, Factual; Sarcoma; Neoplasms, Second Primary; Middle Aged; 3. Good health; Young Adult; Liver; Humans; Hemangioendothelioma, Epithelioid; Female; France; Child; Aged

A NATIONWIDE STUDY: Epithelioid hemangioendothelioma (EHE) is an ultrarare sarcoma whose natural history and treatment not well defined. We report on the presentation outcome of 267 patients with EHE in NETSARC+ network since 2010 France.NETSARC (netsarc.org) a 26 reference centres specialised multidisciplinary tumour boards (MDTB), funded by French National Cancer Institute (NCI), Institut du (INCA). Since 2010, to MDTB second pathological review are mandatory for patients. Patients' characteristics collected nationwide database regularly monitored stable incidence 2013. The at diagnosis presented as progression-free survival (PFS), overall (OS), under treatment.Two hundred sixty-seven were included 2010. Median age series was 51 (range 10-90) years, 58% women. size 37 mm (4-220). Forty-eight percent, 42%, 10% visceral, soft parts, or bone primaries. most frequent sites liver (28%), lung (13%). 40% reported have systemic (i.e. multifocal metastatic disease) diagnosis. With median follow-up 20 months, OS PFS rates 24 months 82% 67%, 10-year projected 62% 21% respectively. Male M+ had significantly worse OS, but PFS. Local associated favourable localised advanced stage For 23 receiving medical treatment, 50.2% 33.2% 60 respectively.EHE frequently unique history. This study shows over 12 years that progressed still alive 10 both stages.