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Il Giornale Italiano di Radiologia Medica 2019 Settembre-Ottobre;6(5):475-9

DOI: 10.23736/S2283-8376.19.00215-8

Copyright © 2019 EDIZIONI MINERVA MEDICA

language: English, Italian

Tips and tricks: how to identify epithelioid hemangioendothelioma of the liver and the spleen

Doriana VERGARA 1 , Maria MATTERA 2, Luca PAZIENZA 3, Filomena URBANO 3, Anna SIMEONE 3

1 Department of Advanced, Biomedical Sciences, Federico II University, Naples, Italy; 2 Department of Radiology, School of Medical Specialties, University of Foggia, Foggia, Italy; 3 Department of Diagnostic Imaging, Casa Sollievo della Sofferenza IRCCS, San Giovanni Rotondo, Foggia, Italy


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Epithelioid hemangioendothelioma is a rare vascular tumor that arises from endothelial cells with variable malignant potential. Hepatic involvement is typical in this uncommon disease. At the time of diagnosis most of patients are asymptomatic, with no known history of hepatic insult or systemic disease, and the diagnosis of hepatic epithelioid hemangioendothelioma (HEHE) occurs after accidental assessment of a liver lesion. Although confirmation of diagnosis always requires immunohistochemistry and histologic analysis, radiological imaging may be helpful in early detection of this tumor. For this purpose, several signs have been outlined to distinguish HEHE from other solid hepatic lesions, such as the halo sign, the target sign, the strip-like sign and the lollipop sign. The management of this uncommon disease, specially in patients with multiorgan involvement, is still debated.


KEY WORDS: Hemangioendothelioma, epithelioid; Liver; Spleen; Tomography, X-ray computed; Magnetic resonance imaging

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