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

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.

Guidelines and Good Clinical Practice Recommendations for Contrast Enhanced Ultrasound (CEUS) in the Liver – Update 2020 – WFUMB in Cooperation with EFSUMB, AFSUMB, AIUM, and FLAUS

“…They are almost always hyperenhancing in the PVP and LP. Thrombosed hemangiomas show lack of enhancement and can be confused with malignancy if only identified during the later CEUS phases [76,96].…”

“…▪ Hepatic epithelioid hemangioendothelioma (HEHE) often manifests as multinodular FLL. On CEUS, HEHE shows rim-like or heterogeneous hyperenhancement in the AP and hypoenhancement in the PVP and LP, a sign of malignancy [96,116,117]. Some patients show centrally located unenhanced areas.…”

“…Since antiangiogenic treatments frequently induce necrosis without causing tumor shrinkage, functional imaging techniques are particularly suitable for the early assessment of response, a task for which both the RECIST and World Health Organization (WHO) size criteria [247,248] are unsatisfactory. Studies of various types of tumors such as HCC, gastrointestinal stromal tumor (GIST) or renal cell carcinoma (RCC) treated with antiangiogenic therapies have confirmed that DCEUS may allow early prediction of response to treatment [91,96,[249][250][251][252][253][254][255][256][257][258][259][260][261][262]. The first multicenter study including more than 500 patients in 19 centers [263] correlated DCEUS with progression free survival (PFS) and overall survival (OS).…”

Hepatic Epithelioid Hemangioendothelioma: Difficult Differential Diagnosis from Angiosarcoma

“…Imaging studies sometimes reveal similar findings between hepatic EHE and angiosarcoma. Hepatic EHE showed three patterns in contrast-enhanced imaging: peripheral nodular enhancement with centripetal fill-in and wash-out during the portal venous and late venous phase (PVLP), rim-like arterial enhancement with wash-out during the PVLP, and inversed target sign with/without wash-out during the PVLP [7]. Moreover, hepatic EHE tends to be multiple and located in the periphery [3].…”

Guidelines and Good Clinical Practice Recommendations for Contrast-Enhanced Ultrasound (CEUS) in the Liver–Update 2020 WFUMB in Cooperation with EFSUMB, AFSUMB, AIUM, and FLAUS

“…They are almost always hyper-enhancing in the PVP and LP. Thrombosed hemangiomas exhibit a lack of enhancement and can be confused with malignancy if only identified during the later CEUS phases (Dietrich et al 2007;Klinger et al 2019).…”