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Z Gastroenterol 2019; 57(06): 753-766
DOI: 10.1055/a-0886-0081
Originalarbeit
© Georg Thieme Verlag KG Stuttgart · New York

Contrast-enhanced imaging in hepatic epithelioid hemangioendothelioma: retrospective study of 10 patients

Kontrastverstärkte Bildgebung des epithelioiden Hämangioendothelioms der Leber
Christoph Klinger
1   Department of Internal Medicine 1, Klinikum Ludwigsburg, Ludwigsburg, Germany
,
Gerhard Stuckmann
2   Department of Radiology, Kantonsspital Winterthur, Switzerland
,
Christoph F. Dietrich
3   Department of Internal Medicine 2, Caritas-Krankenhaus Bad Mergentheim, Bad Mergentheim, Germany
,
Annalisa Berzigotti
4   Swiss Liver Center, Hepatology, University Clinic for Visceral Surgery and Medicine, Inselspital, Department of Biomedical Research, University of Bern, Bern, Switzerland
,
Marius S. Horger
5   Department of Radiology, University of Tuebingen, Tuebingen, Germany
,
Ines Debove
6   Department of Neurology, Inselspital, University of Bern, Bern, Switzerland
,
Bryant J. Gilot
7   Department of Internal Medicine 1 – Center for Personalized Medicine, University of Tuebingen, Tuebingen, Germany
,
Jan Pauluschke-Fröhlich
8   Department of Women’s health, University of Tuebingen, Tuebingen, Germany
,
Tatjana Hoffmann
9   Department of Internal Medicine 1, University of Tuebingen, Tuebingen, Germany
,
Bence Sipos
10   Department of Pathology, University of Tuebingen, Tuebingen, Germany
,
Eckhart Fröhlich
9   Department of Internal Medicine 1, University of Tuebingen, Tuebingen, Germany
› Author Affiliations
Further Information

Publication History

02 January 2019

24 March 2019

Publication Date:
15 April 2019 (online)

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Abstract

Purpose The purpose of this study was to analyze imaging findings in hepatic epithelioid hemangioendothelioma (HEHE) with a particular focus on contrast-enhanced ultrasound (CEUS).

Materials and Methods This retrospective multicenter study included 10 patients with histologically proven HEHE from 5 European centers. All existing ultrasound images/videos were independently analyzed by 2 experienced examiners (DEGUM level III, internal medicine) using a standardized evaluation form. Patterns of contrast enhancement were correlated with computed tomography (CT), magnetic resonance imaging (MRI), and pathological findings.

Results B-mode ultrasound, CEUS, CT, and MRI were performed in 90 %, 70 %, 100 %, and 90 % of patients, respectively. Multifocal HEHE could be observed in 80 % with affection of both liver lobes in 70 %. Analysis of CEUS revealed 3 characteristic patterns that correlated well with contrast patterns on CT and MRI: (a) peripheral nodular enhancement with centripetal fill-in and wash-out in the portal venous and late venous phase (PVLP), (b) rim-like arterial enhancement with wash-out in the PVLP, and (c) inversed target sign with/without wash-out in the PVLP. Wash-out in the PVLP as a sign suspicious of malignancy was observed in 6/7 patients (85.7 %).

Conclusions Knowledge of the different characteristic CEUS patterns is of importance to avoid misdiagnosis due to resemblance of patterns A and B to the much more common focal liver lesions hemangioma and intrahepatic cholangiocarcinoma. Of importance, sonographers should be aware that wash-out in the PVLP might be absent in some patients.

Zusammenfassung

Ziel Analyse bildgebender Befunde des epithelioiden Hämangioendothelioms der Leber (HEHE) unter besonderer Berücksichtigung der Kontrastmittelsonografie (CEUS).

Material und Methode In diese retrospektive, multizentrische Studie wurden 10 Patienten mit histologisch gesichertem HEHE aus 5 europäischen Zentren eingeschlossen. Alle vorhandenen Ultraschallbilder/-videos wurden mithilfe eines standardisierten Auswertungsbogens unabhängig von zwei erfahrenen Untersuchern (DEGUM-Stufe III, Innere Medizin) analysiert. Die nachgewiesenen CEUS-Muster wurden mit Ergebnissen von Computertomografie (CT), Magnetresonanztomografie (MRT) und Pathologie korreliert.

Ergebnisse B-Bild-Sonografie, CEUS, CT und MRT wurden bei 90 %, 70 %, 100 % und 90 % der Patienten durchgeführt. Bei 80 % zeigt sich ein multifokales HEHE, in 70 % mit Beteiligung beider Leberlappen. Die Analyse der Kontrastmittelsonografie ergab drei charakteristische Muster, welche gut mit den CT- und MRT-Befunden korrelierten: A) Periphere noduläre Kontrastierung in der arteriellen Phase mit zentripetalem Anfluten und Auswaschphänomen in der portalvenösen und späten venösen Phase (PVSP) B) Ringförmige arterielle Kontrastierung mit Auswaschphänomen in der PVSP C) Inverses Target-Zeichen mit/ohne Auswaschphänomen in der PVSP. Ein Auswaschphänomen als Hinweis auf das Vorliegen einer malignen Läsion fand sich bei 6/7 Patienten (85,7 %).

Schlussfolgerungen Die Kenntnis der beschriebenen charakteristischen CEUS-Muster ist bedeutend zur Vermeidung von Fehlinterpretationen, da die CEUS-Muster A und B den sehr viel häufigeren fokalen Leberläsionen Hämangiom und intrahepatisches Cholangiokarzinom ähneln. Wichtig ist auch, dass ein für maligne Leberläsionen typisches Auswaschphänomen in der portalvenösen und späten venösen Phase fehlen kann.

Key words

contrast enhanced ultrasound - computed tomography - magnetic resonance imaging - vascular liver tumor - haemangioendothelioma - ultrasound

Schlüsselwörter

Kontrastmittelsonografie - Computertomografie - Magnetresonanztomografie - vaskulärer Lebertumor - Hämangioendotheliom - Ultraschall

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