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Portal hypertension in the absence of portal vein thrombosis and without cirrhosis, but with mild or moderate alterations of liver histology (eg, obliterative venopathy, nodular regenerative hyperplasia, or incomplete septal cirrhosis) is being increasingly recognised. Owing to the heterogeneity of causes and histological findings, a substantial number of terms have been used to describe such idiopathic non-cirrhotic portal hypertension. Patients with the same clinical and histological features exist, but without portal hypertension at the time of diagnosis. Therefore, improved criteria are needed to define this form of liver disease. Here, we propose the term porto-sinusoidal vascular disease, since all lesions found involve the portal venules or sinusoids. The definition of this entity is based on the characteristic absence of cirrhosis with or without signs of portal hypertension or histological lesions. The presence of known causes of liver disease does not rule out porto-sinusoidal vascular disease, but specific causes of vascular liver disease are excluded from its definition. The diagnosis of porto-sinusoidal vascular disease is based on liver biopsy and might include signs specific for portal hypertension with normal or mildly elevated liver stiffness values and no complete portal vein thrombosis. We provide simple diagnostic criteria, because agreement on a uniform nomenclature is an essential requirement for future collaborative studies.

Many management strategies exist for neuroendocrine liver metastases. These strategies range from surgery to ablation with various interventional radiology procedures, and include both regional and systemic therapy with diverse biological, cytotoxic, or targeted agents. A paucity of biological, molecular, and genomic information and an absence of data from rigorous trials limit the validity of many publications detailing management. This Review represents the views from an international conference, for which 15 expert working groups prepared evidence-based assessments addressing specific questions, and from which an independent jury derived final recommendations. The aim of the conference was to review the existing approaches to neuroendocrine liver metastases, assess the evidence on which management decisions were based, develop internationally acceptable recommendations for clinical practice (when evidence was available), and make recommendations for clinical and research endeavours. This report represents the final clinical statements and proposals for future research. © 2014 Elsevier Ltd.

Although liver transplantation is a widely accepted treatment for hepatocellular carcinoma (HCC), much controversy remains and there is no generally accepted set of guidelines. An international consensus conference was held on Dec 2–4, 2010, in Zurich, Switzerland, with the aim of reviewing current practice regarding liver transplantation in patients with HCC and to develop internationally accepted statements and guidelines. The format of the conference was based on the Danish model. 19 working groups of experts prepared evidence-based reviews according to the Oxford classification, and drafted recommendations answering 19 specific questions. An independent jury of nine members was appointed to review these submissions and make final recommendations, after debates with the experts and audience at the conference. This report presents the final 37 statements and recommendations, covering assessment of candidates for liver transplantation, criteria for listing in cirrhotic and non-cirrhotic patients, role of tumour downstaging, management of patients on the waiting list, role of living donation, and post-transplant management.

Purpose: The purpose of this study was to compare the value of contrast-enhanced computed tomography (CT) to that of magnetic resonance imaging obtained with extracellular contrast agent (ECA-MRI) for the diagnosis of a tumor capsule in hepatocellular carcinoma (HCC) using histopathologic findings as the standard of reference. Materials and methods: This retrospective study included patients with pathologically-proven resected HCCs with available preoperative contrast-enhanced CT and ECA-MRI examinations. Two blinded radiologists independently reviewed contrast-enhanced CT and ECA-MRI examinations to assess the presence of an enhancing capsule. The histopathological analysis of resected specimens was used as reference for the diagnosis of a tumor capsule. The sensitivity and specificity of CT and ECA-MRI for the diagnosis of a tumor capsule were determined, and an intra-individual comparison of imaging modalities was performed using McNemar test. Inter-reader agreement was assessed using Kappa test. Results: The study population included 199 patients (157 men, 42 women; mean age: 61.3 +/- 13.0 [SD] years) with 210 HCCs (mean size 56.7 +/- 43.7 [SD] mm). A tumor capsule was present in 157/210 (74.8%) HCCs at histopathologic analysis. Capsule enhancement was more frequently visualized on ECA-MRI (R1, 68.6%; R2, 71.9%) than on CT (R1, 44.3%, P < 0.001; R2, 47.6%, P < 0.001). The sensitivity of ECA-MRI was better for the diagnosis of histopathological tumor capsule (R1, 76.4%; R2, 79.6%; P < 0.001), while CT had a greater specificity (R1, 84.9%; R2, 83.0%; P < 0.001). Inter-reader agreement was moderate both on CT (kappa = 0.55; 95% confidence interval [CI]: 0.43-0.66) and ECA-MRI (kappa = 0.57; 95% CI: 0.45-0.70). Conclusion: Capsule enhancement was more frequently visualized on ECA-MRI than on CT. The sensitivity of ECA-MRI was greater than that of CT, but the specificity of CT was better than that of ECA-MRI. (c) 2021 Societe francaise de radiologie. Published by Elsevier Masson SAS. All rights reserved.

Background To investigate whether fractal analysis of perfusion differentiates hepatocellular adenoma (HCA) subtypes and hepatocellular carcinoma (HCC) in non-cirrhotic liver by quantifying perfusion chaos using four-dimensional dynamic contrast-enhanced magnetic resonance imaging (4D-DCE-MRI). Results A retrospective population of 63 patients (47 female) with histopathologically characterized HCA and HCC in non-cirrhotic livers was investigated. Our population consisted of 13 hepatocyte nuclear factor (HNF)-1α-inactivated (H-HCAs), 7 β-catenin-exon-3-mutated (bex3-HCAs), 27 inflammatory HCAs (I-HCAs), and 16 HCCs. Four-dimensional fractal analysis was applied to arterial, portal venous, and delayed phases of 4D-DCE-MRI and was performed in lesions as well as remote liver tissue. Diagnostic accuracy of fractal analysis was compared to qualitative MRI features alone and their combination using multi-class diagnostic accuracy testing including kappa-statistics and area under the receiver operating characteristic curve (AUC). Fractal analysis allowed quantification of perfusion chaos, which was significantly different between lesion subtypes (multi-class AUC = 0.90, p ex3-HCAs) and low-risk lesions (H-HCAs, I-HCAs) from sensitivity and specificity of 43% (95% confidence interval [CI] 23–66%) and 47% (CI 32–64%) for qualitative MRI features to 96% (CI 78–100%) and 68% (CI 51–81%), respectively, when adding fractal analysis. Conclusions Combining qualitative MRI features with fractal analysis allows identification of HCA subtypes and HCCs in patients with non-cirrhotic livers and improves differentiation of lesions with high and low risk for malignant transformation.

Background Hepatocellular adenomas (HCAs) are rare benign liver tumors. Guidelines recommend continued surveillance of patients diagnosed with HCAs, but these guidelines are mainly based on small studies or expert opinion. Purpose To analyze the long-term evolution of HCAs, including solitary and multiple lesions, and to identify predictive features of progression with MRI. Materials and Methods In a retrospective study, patients diagnosed with pathologically proven solitary or multiple HCAs between January 2004 and December 2015 were included; β-catenin-mutated HCAs and HCAs with foci of malignancy were considered to be at risk for progression. MRI examinations were analyzed, and tumor evolution was evaluated by using Response Evaluation Criteria in Solid Tumors, version 1.1. Student t, Mann-Whitney, χ2, Fisher exact, and McNemar tests were used, as appropriate. Results In total, 118 patients (mean age, 40 years ± 10 [standard deviation]; 108 women) were evaluated, including 41 with a solitary HCA (mean age, 40 years ± 14; 36 women) and 77 with multiple HCAs (mean age, 40 years ± 10; 72 women). At a median follow-up of 5 years, 37 of 41 (90%) patients with a solitary HCA and 55 of 77 (71%) patients with multiple HCAs showed stable or regressive disease. After resection of solitary HCAs, new lesions appeared in only two of 29 (7%) patients, both of whom had HCAs at risk of progression. In patients with multiple HCAs, hepatocyte nuclear factor 1α-inactivated HCAs showed a higher rate of progression compared with inflammatory HCAs (11 of 26 [42%] vs seven of 37 [19%], P = .04) despite lower use (28 of 32 patients [88%] vs 45 of 45 patients [100%]; P = .03) and shorter duration (mean, 12.0 years ± 7.5 vs 19.2 years ± 9.2; P = .001) of oral contraceptive intake. Conclusion Long-term MRI follow-up showed that 78% of hepatocellular adenomas had long-term stability or regression. After resection of solitary hepatocellular adenomas, new lesions occurred only in hepatocellular adenomas at risk of progression. Patients with multiple hepatocellular adenomas were more likely to show progressive disease, with hepatic nuclear factor 1α-inactivated hepatocellular adenomas being the most common subtype showing progression. © RSNA, 2020 Online supplemental material is available for this article.

Focal nodular hyperplasia (FNH) is the second most common benign solid liver lesion after hemangioma, occurring more frequently in young women. The prime differential diagnoses include hepatocellular adenoma, hepatocellular carcinoma, and hypervascular metastasis. As the management of FNH is typically conservative, imaging plays a key role in diagnostic pathway, and misdiagnosis may have a major clinical effect. In this article, we describe the ultrasound, computed tomography, and magnetic resonance imaging features of FNH, underlining the importance of typical radiological features that allow a specific noninvasive diagnosis. We present a large spectrum of a typical imaging findings that FNH may present and discuss the up-to-date diagnostic strategy.

To report the association of a mosaic enhancement pattern on contrast-enhanced CT or MR imaging and hepatic sinusoidal dilatation (SD) with acute inflammatory conditions affecting extrahepatic organs. From 2007 to 2012, patients with acute inflammatory diseases who underwent contrast-enhanced CT and/or MRI of the liver with a mosaic enhancement pattern were selected. Clinico-biological and other imaging features were collected at diagnosis and during follow-up. Sixteen patients were included (15 women, median age 27 years; range 18–68). Five women (33 %) were receiving oral contraceptives. Acute inflammatory diseases included pyelonephritis (n = 10), pancreatitis (n = 2), pneumonia (n = 1), septicemia (n = 1), active Crohn's disease (n = 1), and infectious colitis (n = 1). Median white blood cell count was 13,250 cells/μL (range 11,500-18,000 cells/μL) and CRP level 94 mg/L (range 60–121 mg/L). Mosaic enhancement pattern was present in the whole liver and was prominent in the subcapsular areas. Four patients underwent liver biopsy confirming SD. Eleven patients underwent follow-up imaging showing normalized aspect in 9/11 patients after a median of 2 months. Acute diseases of extrahepatic organs, associated with a marked systemic inflammatory syndrome should be added to the list of conditions causing a reversible hepatic sinusoidal dilatation as manifested by a mosaic enhancement pattern on contrast-enhanced CT or MR imaging. • Acute extrahepatic infectious/inflammatory diseases are a cause of transient MP. • In most patients, MP was seen during both arterial and portal venous phase. • In all patients, the mosaic enhancement pattern was diffuse, but more conspicuous in subcapsular areas. • MP was no longer seen after resolution of the acute disease. • No liver biopsy should be performed.

PURPOSE:To analyze the signal intensity (SI) of benign hepatocellular lesions in high-b-value diffusion-weighted (DW) magnetic resonance (MR) images and to compare the apparent diffusion coefficient (ADC) values of focal nodular hyperplasias (FNHs) with those of hepatocellular adenomas (HCAs). MATERIALS AND METHODS: This retrospective study was approved by institutional review board, with waiver of informed consent. Inclusion criteria were consecutive patients with diagnosed FNH or HCA who underwent MR imaging with a DW sequence of the liver at three b values, 0, 150, and 600 sec/mm2. The final study population included 67 patients (seven men, 60 women) with 90 hepatocellular lesions (54 FNHs, 36 HCAs). The mean ADC was compared between the lesions and the liver. Receiver operating characteristic analysis was performed to evaluate the diagnostic value of ADC for differentiating HCAs and FNHs. RESULTS: The mean ADC value of all FNHs and HCAs was significantly lower than that of the liver (P=.004). An ADC ratio below 15% was observed in 50 of 54 (93%) FNHs and in 29 of 36 (81%) HCAs. The mean ADC value of FNHs was significantly higher than that of HCAs (P

Transcatheter arterial chemoembolization (TACE) offers a survival benefit to patients with intermediate hepatocellular carcinoma (HCC). A widely accepted TACE regimen includes administration of doxorubicin-oil emulsion followed by gelatine sponge-conventional TACE. Recently, a drug-eluting bead (DC Bead) has been developed to enhance tumor drug delivery and reduce systemic availability. This randomized trial compares conventional TACE (cTACE) with TACE with DC Bead for the treatment of cirrhotic patients with HCC. Two hundred twelve patients with Child-Pugh A/B cirrhosis and large and/or multinodular, unresectable, N0, M0 HCCs were randomized to receive TACE with DC Bead loaded with doxorubicin or cTACE with doxorubicin. Randomization was stratified according to Child-Pugh status (A/B), performance status (ECOG 0/1), bilobar disease (yes/no), and prior curative treatment (yes/no). The primary endpoint was tumor response (EASL) at 6 months following independent, blinded review of MRI studies. The drug-eluting bead group showed higher rates of complete response, objective response, and disease control compared with the cTACE group (27% vs. 22%, 52% vs. 44%, and 63% vs. 52%, respectively). The hypothesis of superiority was not met (one-sided P = 0.11). However, patients with Child-Pugh B, ECOG 1, bilobar disease, and recurrent disease showed a significant increase in objective response (P = 0.038) compared to cTACE. DC Bead was associated with improved tolerability, with a significant reduction in serious liver toxicity (P < 0.001) and a significantly lower rate of doxorubicin-related side effects (P = 0.0001). TACE with DC Bead and doxorubicin is safe and effective in the treatment of HCC and offers a benefit to patients with more advanced disease.

OBJECTIVE: The objective of our study was to illustrate the imaging findings of Budd-Chiari syndrome, including CT, MRI, sonographic, and angiographic findings. CONCLUSION: The key imaging findings in Budd-Chiari syndrome are occlusion of the hepatic veins, inferior vena cava, or both; caudate lobe enlargement; inhomogeneous liver enhancement; and the presence of intrahepatic collateral vessels and hypervascular nodules. Awareness of these findings is important for early diagnosis and appropriate treatment.

Fibropolycystic liver disease encompasses a spectrum of related lesions of the liver and biliary tract that are caused by abnormal embryologic development of the ductal plates. These lesions (congenital hepatic fibrosis, biliary hamartomas, autosomal dominant polycystic disease, Caroli disease, choledochal cysts) can be clinically silent or can cause signs and symptoms such as cholangitis, portal hypertension, gastrointestinal bleeding, infections, and space-occupying masses. The different types of fibropolycystic liver disease demonstrate characteristic findings at computed tomography (CT) and magnetic resonance (MR) imaging. Patients with congenital hepatic fibrosis typically have imaging evidence of liver morphologic abnormalities, varices, splenomegaly, renal lesions, and other associated ductal plate abnormalities. Biliary hamartomas usually manifest as multiple cysts that are nearly uniform in size and measure up to 15 mm in diameter. Autosomal dominant polycystic disease typically manifests as an enlarged and diffusely cystic liver. In Caroli disease, cystic or fusiform dilatation of the intrahepatic ducts is seen, as well as the "central dot sign," which corresponds to a portal vein branch protruding into the lumen of a dilated bile duct. Choledochal cyst manifests as a fusiform or cystic dilatation of the extrahepatic bile duct. Awareness of these CT and MR imaging features is essential in detecting and differentiating between various fibropolycystic liver diseases and can assist in proper management.

PURPOSE: To retrospectively investigate the prevalence, MR findings, natural history, and association with other hepatic lesions of focal nodular hyperplasia (FNH)-like lesions in a cohort of consecutive patients with cavernous transformation of the portal vein (CTPV). MATERIALS AND METHODS: This retrospective IRB-approved study comprised 58 patients (32 men, 26 women; average age, 50 years) with CTPV who underwent liver MR imaging between 2000 and 2008. MR images were assessed by two radiologists in consensus for the presence of (a) FNH-like lesions and other liver lesions, and (b) other imaging findings. Patients were assigned to a stable or progressive clinical course based on lesion characteristics at follow-up. RESULTS: Twelve of 58 patients (21%) had 38 FNH-like lesions (average size, 1.3 cm). Common findings of FNH-like lesions were isointensity on T2-weighted images (82%), intense and homogeneous enhancement during the arterial phase, and lack of washout during the hepatic venous and interstitial phases (100%). FNH-like lesions were found with other benign liver lesions (1 hemangioma, 1 adenoma) in two patients. Three (25%) patients with FNH-like lesions showed a progressive clinical course. CONCLUSION: FNH-like lesions are commonly detected in patients with CTPV. Most lesions demonstrate benign imaging findings and stable clinical course.

Benign hepatic vascular neoplasms and vascular pseudolesions are commonly encountered in magnetic resonance (MR) imaging. Most of these benign lesions demonstrate not only arterial-phase hyperintensity, but also persistent enhancement on venous and delayed imaging, unlike most malignant hepatic masses. These features, along with other MR findings and morphological characteristics, may allow for more confident diagnosis and distinction from hepatic malignancy. The objective of our study was to illustrate the MR imaging findings of hepatic lesions that demonstrate both early arterial and sustained enhancement on hepatic venous and delayed phase.

G. Brancatelli, MD; M. Midiri, MD; R. Lagalla, MD Department of Radiology, Policlinico Universitario, Via del Vespro 127, 90127 Palermo, Italy M. P. Federle, MD Department of Radiology, Abdominal Imaging Offi ces, University of Pittsburgh Medical Center, Room 4660, CHP, MT, 200 Lothrop Street, Pittsburgh, PA 15213, USA V. Vilgrain, MD Department of Radiology, Hospital Beaujon, Avenue Du General Leclerc 100, 92118 Clichy, France L. Grazioli, MD Department of Radiology, University of Brescia, Spedali Civili di Brescia, Piazzale Spedali Civili 1, 25023 Brescia, Italy CONTENTS

To evaluate the computed tomographic (CT) findings in adult patients with pathologically proved congenital hepatic fibrosis.This was a retrospective review of congenital hepatic fibrosis cases identified at two institutions over the course of 8 years. Eight men and 10 women with an age range of 22-72 years (mean age, 39 years) were included. Contrast material-enhanced and unenhanced CT scans were obtained through the liver in all patients. Two radiologists evaluated size of and morphologic findings (atrophy or hypertrophy localized according to hepatic segments) in the liver; increased diameter or number of hepatic arteries at the hilum; presence of hepatic nodules, varices, spontaneous splenorenal shunts, and splenomegaly; and association with other hepatic ductal plate malformations and renal abnormalities.Sixteen patients had morphologic abnormalities in the liver, 15 had splenomegaly (three underwent splenectomy for portal hypertension), and 14 had varices or spontaneous splenorenal shunts. An enlarged hepatic artery and a tangle of abnormally enlarged arterial vessels were identified in five and four patients, respectively, and four of these nine patients had large benign regenerative nodules. Ten patients had renal abnormalities and nine had an associated ductal plate malformation.This retrospective study shows that certain findings (ie, liver morphologic and associated ductal plate abnormalities, varices, splenomegaly, and renal abnormalities) are frequently observed in combination in patients with congenital hepatic fibrosis.