Epithelioid hemangioendothelioma

Sophie Cousin; François Le Loarer; Amandine Crombé; Marie Karanian; Véronique Minard-Colin; Nicolas Penel

doi:10.1016/j.bulcan.2018.11.004

Epithelioid hemangioendothelioma

Sophie Cousin, François Le Loarer, Amandine Crombé, Marie Karanian, Véronique Minard-Colin, Nicolas Penel

Research output: Contribution to journal › Short survey › peer-review

8 Citations (Scopus)

Abstract

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.

Translated title of the contribution	Hémangioendothéliome épithélioïde
Original language	English
Pages (from-to)	73-83
Number of pages	11
Journal	Bulletin du Cancer
Volume	106
Issue number	1
DOIs	https://doi.org/10.1016/j.bulcan.2018.11.004
Publication status	Published - 1 Jan 2019

Keywords

Diagnosis
Epithelioid hemangioendothelioma
Management
Reference centers
Sarcoma

Access to Document

10.1016/j.bulcan.2018.11.004

Cite this

@article{510c231904a34626af446bd9d03103b0,

title = "Epithelioid hemangioendothelioma",

abstract = "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.",

keywords = "Diagnosis, Epithelioid hemangioendothelioma, Management, Reference centers, Sarcoma",

author = "Sophie Cousin and {Le Loarer}, Fran{\c c}ois and Amandine Cromb{\'e} and Marie Karanian and V{\'e}ronique Minard-Colin and Nicolas Penel",

note = "Publisher Copyright: {\textcopyright} 2018 Soci{\'e}t{\'e} Fran{\c c}aise du Cancer",

year = "2019",

month = jan,

day = "1",

doi = "10.1016/j.bulcan.2018.11.004",

language = "English",

volume = "106",

pages = "73--83",

journal = "Bulletin du Cancer",

issn = "0007-4551",

number = "1",

}

TY - JOUR

T1 - Epithelioid hemangioendothelioma

AU - Cousin, Sophie

AU - Le Loarer, François

AU - Crombé, Amandine

AU - Karanian, Marie

AU - Minard-Colin, Véronique

AU - Penel, Nicolas

PY - 2019/1/1

Y1 - 2019/1/1

N2 - 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.

AB - 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.

KW - Diagnosis

KW - Epithelioid hemangioendothelioma

KW - Management

KW - Reference centers

KW - Sarcoma

UR - http://www.scopus.com/inward/record.url?scp=85057895116&partnerID=8YFLogxK

U2 - 10.1016/j.bulcan.2018.11.004

DO - 10.1016/j.bulcan.2018.11.004

M3 - Short survey

C2 - 30527817

AN - SCOPUS:85057895116

SN - 0007-4551

VL - 106

SP - 73

EP - 83

JO - Bulletin du Cancer

JF - Bulletin du Cancer

IS - 1

ER -

Epithelioid hemangioendothelioma

Abstract

Keywords

Access to Document

Other files and links

Cite this