[Epithelioid hemangioendothelioma].
Hémangioendothéliome épithélioïde.
Centre de référence
Diagnosis
Diagnostic
Epithelioid hemangioendothelioma
Hémangioendothéliomes épithélioïdes
Management
Reference centers
Sarcoma
Sarcome
Traitement
Journal
Bulletin du cancer
ISSN: 1769-6917
Titre abrégé: Bull Cancer
Pays: France
ID NLM: 0072416
Informations de publication
Date de publication:
Jan 2019
Jan 2019
Historique:
received:
24
07
2018
revised:
28
10
2018
accepted:
01
11
2018
pubmed:
12
12
2018
medline:
5
2
2019
entrez:
12
12
2018
Statut:
ppublish
Résumé
We describe herein the inaugural manifestations, the radiological and histological diagnosis criteria for and the outcome of epithelioid hemangioendothelioma (EHE). Most of EHE (90%) display a specific reciprocal chromosomic translocation t(1;3)(p36;q23-25), which is associated with the synthesis of fusion protein WWTR1/CAMTA1. EHE are low-grade vascular sarcomas. EHE could be initially localized or multifocal. At localized stage, EHE are best treated with focal treatments. At multifocal stage, the upfront strategy is watchful follow-up. Some multifocal EHE display very indolent course with spontaneous stable disease for years or decades. In case of progressive multifocal EHE, there is no consensual treatment. Diagnostic and clinical management of EHE requires interdisciplinary expertise from labeled centers.
Identifiants
pubmed: 30527817
pii: S0007-4551(18)30340-0
doi: 10.1016/j.bulcan.2018.11.004
pii:
doi:
Types de publication
Journal Article
Review
Langues
fre
Sous-ensembles de citation
IM
Pagination
73-83Informations de copyright
Copyright © 2018 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.