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

Titre traduit de la contribution: Hémangioendothéliome épithélioïde

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

Résultats de recherche: Contribution à un journal › Brève enquête › Revue par des pairs

8 Citations (Scopus)

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.

Titre traduit de la contribution	Hémangioendothéliome épithélioïde
langue originale	Anglais
Pages (de - à)	73-83
Nombre de pages	11
journal	Bulletin du Cancer
Volume	106
Numéro de publication	1
Les DOIs	https://doi.org/10.1016/j.bulcan.2018.11.004
état	Publié - 1 janv. 2019

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10.1016/j.bulcan.2018.11.004

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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",

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AU - Le Loarer, François

AU - Crombé, Amandine

AU - Karanian, Marie

AU - Minard-Colin, Véronique

AU - Penel, Nicolas

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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.

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