Epithelioid hemangioendothelioma

被引：5

作者：

Cousin, Sophie ^{[1
]}

Le Loarer, Francois ^{[2
]}

Crombe, Amandine ^{[3
]}

Karanian, Marie ^{[4
]}

Minard-Colin, Veronique ^{[5
]}

Penel, Nicolas ^{[6
,7
]}

机构：

[1] Inst Bergonie, Dept Oncol Med, 229 Cours Argonne, F-33000 Bordeaux, France

[2] Inst Bergonie, Dept Anatomopathol, 229 Cours Argonne, F-33000 Bordeaux, France

[3] Inst Bergonie, Dept Imagerie Med, 229 Cours Argonne, F-33000 Bordeaux, France

[4] Ctr Leon Berard, Dept Anatomopathol, 28 Promenade Lea & Napoleon Bullukian, F-69008 Lyon, France

[5] Inst Gustave Roussy, Dept Oncol Pediat, 114 Rue Edouard Vaillant, F-94800 Villejuif, France

[6] Ctr Oscar Lambret, Dept Oncol Med, 3 Rue Frederic Combemale, F-59000 Lille, France

[7] Hop Claude Huriez, Serv Oncol Med, Rue Michel Polonowski, F-59000 Lille, France

来源：

BULLETIN DU CANCER | 2019年 / 106卷 / 01期

关键词：

Diagnosis; Epithelioid hemangioendothelioma; Management; Reference centers; Sarcoma; OF-THE-LITERATURE; LIVER-TRANSPLANTATION; IMAGING FINDINGS; SOFT-TISSUE; FUSION; EMPHASIS; MRI; CT;

D O I：

10.1016/j.bulcan.2018.11.004

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 ;

摘要：

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.

引用

页码：73 / 83

页数：11

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