Rituximab Monotherapy Is Effective as First-Line Treatment for Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) in CVID Patients.

Granulomatous lymphocytic interstitial lung disease (GLILD) represents a fatal immune dysregulatory complication in common variable immunodeficiency (CVID). Evidence-based diagnostic guidelines are lacking, and GLILD treatment consists in immunosuppressive drugs; nonetheless, therapeutical strategies are heterogeneous and essentially based on experts' opinions and data from small case series or case reports.We aimed to evaluate the efficacy and safety of first-line Rituximab monotherapy for CVID-related GLILD, by assessing symptoms and quality of life alterations, immunological parameters, pulmonary function tests, and lung computed tomography.All six GLILD patients received Rituximab infusions as a first-line treatment. Rituximab was administered at 375 mg/m2 monthly for six infusions followed by maintenance every 3 months; none of the patients experienced severe adverse events. Symptom burden and quality of life significantly improved in treated patients compared to a control group of CVID patients without GLILD. Rituximab treatment indirectly caused a trend toward reduced T-cell activation and exhaustion markers sCD25 and sTIM-3. Lung function improved in treated patients, with statistically significant increases in TLC and DLCO. Lung CT scan findings expressed by means of Baumann scoring system displayed a reduction in the entire cohort.In conclusion, first-line monotherapy with Rituximab displayed high efficacy in disease remission in all treated patients, with improvement of symptoms and amelioration of quality of life, as well as restoration of PFTs and lung CT scan findings.

Common and Uncommon CT Findings in CVID-Related GL-ILD: Correlations with Clinical Parameters, Therapeutic Decisions and Potential Implications in the Differential Diagnosis.

PURPOSE: To investigate computed tomography (CT) findings of Granulomatous Lymphocytic Interstitial Lung Disease (GL-ILD) in Common Variable Immunodeficiency (CVID), also in comparison with non-GL-ILD abnormalities, correlating GL-ILD features with functional/immunological parameters and looking for GL-ILD therapy predictive elements. METHODS: CT features of 38 GL-ILD and 38 matched non-GL-ILD subjects were retrospectively described. Correlations of GL-ILD features with functional/immunological features were assessed. A logistic regression was performed to find a predictive model of GL-ILD therapeutic decisions. RESULTS: Most common GL-ILD CT findings were bronchiectasis, non-perilymphatic nodules, consolidations, Ground Glass Opacities (GGO), bands and enlarged lymphnodes. GL-ILD was usually predominant in lower fields. Multiple small nodules (≤10 mm), consolidations, reticulations and fibrotic ILD are more indicative of GL-ILD. Bronchiectasis, GGO, Reticulations and fibrotic ILD correlated with decreased lung performance. Bronchiectasis, GGO and fibrotic ILD were associated with low IgA levels, whereas high CD4+ T cells percentage was related to GGO. Twenty out of 38 patients underwent GL-ILD therapy. A model combining Marginal Zone (MZ) B cells percentage, IgA levels, lower field consolidations and lymphnodes enlargement showed a good discriminatory capacity with regards to GL-ILD treatment. CONCLUSIONS: GL-ILD is a lower field predominant disease, commonly characterized by bronchiectasis, non-perilymphatic small nodules, consolidations, GGO and bands. Multiple small nodules, consolidations, reticulations and fibrotic ILD may suggest the presence of GL-ILD in CVID. MZ B cells percentage, IgA levels at diagnosis, lower field consolidations and mediastinal lymphnodes enlargement may predict the need of a specific GL-ILD therapy.

Endoscopic treatment of periampullary duodenal duplication cysts in children: Four case reports and review of the literature.

BACKGROUND: Duodenal duplications are rare congenital anomalies of the gastrointestinal tract. As the periampullary variant is much rarer, literature is scant and only few authors have reported their experience in diagnosis and treatment, particularly with operative endoscopy. CASE SUMARY: To report our experience with the endoscopic treatment in a series of children with periampullary duodenal duplication cysts, focusing on the importance of obtaining an accurate preoperative anatomic assessment of the malformations. The pediatric periampullary duodenal duplication cyst literature is reviewed. We conducted a systematic review according to the PRISMA guidelines. The PubMed database was searched for original studies on "duodenal duplication", "periampullary duplication" or "endoscopic management" published since 1990, involving patients younger than 18 years of age. Eligible study designs were case report, case series and reviews. We analyzed the data and reported the results in table and text. Fifteen eligible articles met the inclusion criteria with 16 patients, and analysis was extended to our additional 4 cases. Median age at diagnosis was 13.5 years. Endoscopic treatment was performed in 10 (50%) patients, with only 2 registered complications. CONCLUSION: Periampullary duodenal duplication cysts in pediatric patients are very rare. Our experience suggests that an accurate preoperative assessment is critical. In the presence of sludge or stones inside the duplication, endoscopic retrograde cholangio-pancreatography is mandatory to demonstrate a communication with the biliary tree. Endoscopic treatment resulted in a safe, minimally invasive and effective treatment. In periampullary duodenal duplication cyst endoscopically treated children, long-term follow-up is still necessary considering the potential malignant transformation at the duplication site.

Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) in Common Variable Immunodeficiency (CVID): A Multicenter Retrospective Study of Patients From Italian PID Referral Centers.

Background: Granulomatous and Lymphocytic Interstitial Lung Diseases (GLILD) is a severe non-infectious complication of Common Variable Immunodeficiency (CVID), often associated with extrapulmonary involvement. Due to a poorly understood pathogenesis, GLILD diagnosis and management criteria still lack consensus. Accordingly, it is a relevant cause of long-term loss of respiratory function and is closely associated with a markedly reduced survival. The aim of this study was to describe clinical, immunological, laboratory and functional features of GLILD, whose combination in a predictive model might allow a timely diagnosis. Methods: In a multicenter retrospective cross-sectional study we enrolled 73 CVID patients with radiologic features of interstitial lung disease (ILD) associated to CVID (CVID-ILD) and 125 CVID patients without ILD (controls). Of the 73 CVID-ILD patients, 47 received a definite GLILD diagnosis while 26 received a clinical-radiologic diagnosis of CVID related ILD defined as uILD. Results: In GLILD group we found a higher prevalence of splenomegaly (84.8 vs. 39.2%), autoimmune cytopenia (59.6 vs. 6.4%) and bronchiectasis (72.3 vs. 28%), and lower IgA and IgG serum levels at CVID diagnosis. GLILD patients presented lower percentage of switched-memory B cells and marginal zone B cells, and a marked increase in the percentage of circulating CD21lo B cells (14.2 vs. 2.9%). GLILD patients also showed lower total lung capacity (TLC 87.5 vs. 5.0%) and gas transfer (DLCO 61.5 vs. 5.0%) percent of predicted. By univariate logistic regression analysis, we found IgG and IgA levels at CVID diagnosis, presence of splenomegaly and autoimmune cytopenia, CD21lo B cells percentage, TLC and DCLO percent of predicted to be associated to GLILD. The joint analysis of four variables (CD21lo B cells percentage, autoimmune cytopenia, splenomegaly and DLCO percent of predicted), together in a multiple logistic regression model, yielded an area under the ROC curve (AUC) of 0.98 (95% CI: 0.95-1.0). The AUC was only slightly modified when pooling together GLILD and uILD patients (0.92, 95% CI: 0.87-0.97). Conclusions: we propose the combination of two clinical parameters (splenomegaly and autoimmune cytopenia), one lung function index (DLCO%) and one immunologic variable (CD21lo%) as a promising tool for early identification of CVID patients with interstitial lung disease, limiting the use of aggressive diagnostic procedures.

Activated Phosphoinositide 3-Kinase Delta Syndrome 1: Clinical and Immunological Data from an Italian Cohort of Patients.

Activated phosphoinositide 3-kinase delta syndrome 1 (APDS-1) is a recently described inborn error of immunity caused by monoallelic gain-of-function mutations in the PIK3CD gene. We reviewed for the first time medical records and laboratory data of eight Italian APDS-1 patients. Recurrent sinopulmonary infections were the most common clinical feature at onset of disease. Seven patients presented lymphoproliferative disease, at onset or during follow-up, one of which resembled hemophagocytic lymphohistiocytosis (HLH). Genetic analysis of the PIK3CD gene revealed three novel mutations: functional testing confirmed their activating nature. In the remaining patients, the previously reported variants p.E1021K (n = 4) and p.E525A (n = 1) were identified. Six patients were started on immunoglobulin replacement treatment (IgRT). One patient successfully underwent hematopoietic stem cell transplantation (HSCT), with good chimerism and no GVHD at 21 months post-HSCT. APDS-1 is a combined immune deficiency with a wide variety of clinical manifestations and a complex immunological presentation. Besides IgRT, specific therapies targeting the PI3Kδ pathway will most likely become a valid aid for the amelioration of patients' clinical management and their quality of life.

Paediatric MAS/HLH caused by a novel monoallelic activating mutation in p110δ.

This study provides evidence for the first time for APDS-1 presenting as MAS/HLH, with evident clinical implications in patient's management and prognosis.

PAX1 is essential for development and function of the human thymus.

We investigated the molecular and cellular basis of severe combined immunodeficiency (SCID) in six patients with otofaciocervical syndrome type 2 who failed to attain T cell reconstitution after allogeneic hematopoietic stem cell transplantation, despite successful engraftment in three of them. We identified rare biallelic PAX1 rare variants in all patients. We demonstrated that these mutant PAX1 proteins have an altered conformation and flexibility of the paired box domain and reduced transcriptional activity. We generated patient-derived induced pluripotent stem cells and differentiated them into thymic epithelial progenitor cells and found that they have an altered transcriptional profile, including for genes involved in the development of the thymus and other tissues derived from pharyngeal pouches. These results identify biallelic, loss-of-function PAX1 mutations as the cause of a syndromic form of SCID due to altered thymus development.

A new case report of severe mucopolysaccharidosis type VII: diagnosis, treatment with haematopoietic cell transplantation and prenatal diagnosis in a second pregnancy.

A new patient with severe mucopolysaccharidosis (MPS) type VII is reported. Non-immune hydrops fetalis (NIHF) was diagnosed during pregnancy. At birth, he showed generalized hydrops and dysmorphic features typical of MPS. Many diagnoses were excluded before reaching the diagnosis of MPS VII at 8 months of life. During the first year of life he had frequent respiratory infections associated with restrictive and obstructive bronchopneumopathy and underwent three surgical interventions: decompression of the spinal cord at the craniocervical junction, bilateral inguinal hernia, and bilateral clubfoot. At 14 months of life he underwent successful haematopoietic cell transplantation (HCT). During the following 10 months, his bronchopneumopathy progressively worsened, needing chronic pharmacological treatment and O2 administration. The patient died of respiratory insufficiency during a respiratory syncytial virus infection at 25 months of age. Molecular analysis showed the homozygous variant c.1617C > T, leading to the synonymous mutation p.Ser539=. This caused aberrant splicing with partial skipping of exon 10 (r.1616_1653del38) and complete skipping of exon 9 (r.1392_1476del85; r.1616_1653del38). No transcript of normal size was evident. The parents were both confirmed to be carriers. In a subsequent pregnancy, a prenatal diagnosis showed an affected fetus. Ultrasound examination before abortion showed NIHF. The skin and placenta examination by electron microscopy showed foamy intracytoplasmic vacuoles with a weakly electron-dense substrate. MPS VII is a very rare disease but it is possible that some cases go undiagnosed for several reasons, including that MPS VII, and other lysosomal storage diseases, are not included in the work-up for NIHF in many institutions, and the presence of anasarca at birth may be confounding for the recognition of the typical facial characteristics of the disease. This is the eighth patient affected by MPS VII who has undergone HCT. It is not possible to draw conclusions about the efficacy of HCT in MPS VII. Treatment with enzyme replacement is now available and will probably be beneficial for the patients who have a milder form with no or little cognitive involvement. Increased awareness among clinicians is needed for prompt diagnosis and to offer the correct treatment as early as possible.

Primary lymphoma of the distal radius of a child: imaging features.

Primary lymphoma of bone (PLB) is a rare entity, defined as a lymphoma confined to the bone without evidence of systemic involvement. The disease commonly affects middle-aged to elderly population and it accounts for less than 1% of all malignant lymphomas. We present a case of a 10-year-old child affected by PLB of the forearm and the frontal bone. Characteristic imaging features of PLB and the main differential diagnosis were discussed.

Comparative X-ray morphometry of prenatal osteogenesis imperfecta type 2 and thanatophoric dysplasia: a contribution to prenatal differential diagnosis.

OBJECTIVE: The purpose of the paper was to assess the morphometric parameters to improve the specificity of the ultrasound (US) signs for the early differential diagnosis between two lethal dysplasias, as thanatophoric dysplasia (TD) and osteogenesis imperfecta type 2 (OI-2). METHOD: The diaphyseal length and the bowed shape of long bones associated with vertebral body dimension assessment were investigated in a group of 14 pregnancy terminations carried out in the time period 2007-2013. The definitive diagnosis was established after pregnancy termination by means of skeletal standardized X-rays, histopathology and gene analysis. RESULTS: TD and OI-2 long bones were significantly shorter than controls. No significant differences were observed between the two dysplasias. The bowing angle was higher in OI-2; a true angulation or eventually axial displacement was present only in the latter. Furthermore, they did not show any evidence of vertebral collapse. The thanatophoric dysplasia presented less bowed long bones, and never true angulation. The spine was steadily characterized by flattened anterior vertebral bodies. CONCLUSION: Long bone shortening is not a sufficient and accurate sign for early sonographic differential diagnosis between TD and OI-2. Angled diaphysis, axial diaphyseal displacement and a conserved vertebral body height in the prenatal period support the diagnosis of osteogenesis imperfecta type 2, while moderately regular bowed diaphysis associated with platyspondyly that of thanatophoric dysplasia.

Early Identification of Lung Fungal Infections in Chronic Granulomatous Disease (CGD) Using Multidetector Computer Tomography.

PURPOSE: The purpose of this study is to evaluate the possibility of early detection of pulmonary fungal infections by lung CT scan in chronic granulomatous disease (CGD). METHODS: A retrospective study on 14 patients affected with CGD for a total of 18 infectious episodes was performed. Revision of clinical data and CT scan analysis before and after treatment was performed. RESULTS: The presence of lung nodules <30 mm was evaluated in 18 infectious episodes in 14 patients. A total of 125 nodules in 18 CT scans were identified. Identification of the infectious agent through biopsy and in vitro culture resulted positive only in 3/18 cases. The remaining cases received clinical/radiologic diagnosis of suspected pulmonary fungal infection. In all cases, the introduction of empirical antifungal treatment resulted in reduction in size or complete resolution of the pulmonary lung nodules in all patients affected with CGD. CONCLUSIONS: Lung CT scan allows for early detection of pulmonary fungal infection in CGD. Pulmonary nodules (<30 mm), single or multiple, uni- or bilateral, with or without a halo sign may represent the first radiologic sign of pulmonary fungal infection in CGD.

Diffusion weighted MR and apparent diffusion coefficient measurement in classification and characterization of noncystic focal liver lesions: does a clinical role exist?

The objective of this study was to assess the clinical role of apparent diffusion coefficient (ADC) analysis in noncystic focal liver lesion (FLL) classification/characterization.Six hundred liver magnetic resonances with multi-b (b = 50, 400, 800 s/mm) diffusion-weighted imaging (DwI) were retrospectively reviewed. Mean ADC was measured in 388 lesions (195 benign and 193 malignant) excluding internal necrotic areas. Cystic benign lesions were excluded from analysis. Sensitivity and specificity in distinguishing benign from malignant lesions were calculated. Analysis of variance was performed to detect differences among subgroups of solid lesions.Mean ADC of malignant lesions was 0.980 × 10 mm/s, significantly (P < 0.05) lower than mean ADC of benign lesions (1.433 × 10 mm/s). Applying an ADC cutoff of 1.066 × 10 mm/s, specificity and sensitivity for malignancy were respectively 86.6% and 73.6%. Of all lesions, >1/3 (39.5%) presented values lower than 1 × 10 mm/s, with 90.0% chance of malignancy. Above 1.5 × 10 mm/s (about 20% of all lesions) chance of malignancy was 9.5%.DwI cannot assist in noncystic FLL characterization, but can help in FLL classification in about half the cases.

Congenital cystic lung disease: prenatal ultrasound and postnatal multidetector computer tomography evaluation. Correlation with surgical and pathological data.

PURPOSE: The aim of this study was to evaluate the diagnostic accuracy of postnatal multidetector computed tomography (MDCT) compared with prenatal ultrasound (US), surgical findings, and histology, in 33 patients with congenital cystic lung disease. METHODS: Thirty-three patients, 17 males and 16 females, were evaluated by MDCT. Twenty-seven of these patients underwent prenatal US between week 18 and 22, and between week 32 and 35 of gestation. Lung lobectomy, segmentectomy, atypical resection, lesion resection were performed in 31 patients and surgical specimens were analysed. RESULTS: Prenatal US and MDCT correctly diagnosed 76.9 and 94 % of the lesions, respectively. Disagreement occurred in six lesions with prenatal US and in two lesions with MDCT. No statistically significant differences were observed between the two techniques (P = 0.122). CONCLUSIONS: As most surgeons consider the surgical resection of these lesions mandatory, our study underscores the essential role of imaging, in particular CT, in providing invaluable preoperative information on congenital cystic lung diseases recognised in uterus.

Prenatal presentation and postnatal evolution of a patient with Jansen metaphyseal dysplasia with a novel missense mutation in PTH1R.

Wave-shaped ribs were detected at prenatal ultrasound in a 20(+1) week female fetus. At birth, skeletal radiographs showed marked hypomineralization and suggested hypophosphatasia. However, elevated blood calcium and alkaline phosphatase excluded hypophosphatasia and raised the possibility of Jansen metaphyseal dysplasia. Molecular analysis of the PTH/PTHrP receptor gene (PTH1R) showed heterozygosity for a previously undescribed transversion variant (c.1373T>A), which predicts p.Ile458Lys. In vitro evaluation of wild type and mutant PTH/PTHrP receptors supported the pathogenic role of the p.Ile458Lys substitution, and confirmed the diagnosis of Jansen metaphyseal dysplasia. This disorder may present prenatally with wavy ribs and in the newborn with hypomineralization, and may therefore be confused with hypophosphatasia. The mottled metaphyseal lesions typically associated with this disease appear only in childhood.

MR Imaging of Hepatocellular Adenomas and Differential Diagnosis Dilemma.

HEPATOCELLULAR ADENOMAS (HCAS) ARE CURRENTLY CATEGORIZED INTO DISTINCT GENETIC AND PATHOLOGIC SUBTYPES AS FOLLOWS: inflammatory hepatocellular adenoma, hepatocyte-nuclear-factor-1-alpha (HNF-1 α -mutated) hepatocellular adenoma, and ß -catenin-mutated hepatocellular adenomas; the fourth, defined as unclassified subtype, encompasses HCAs without any genetic abnormalities. This classification has accepted management implications due to different risks of haemorrhage and malignant transformation of the four subtypes. Imaging guided biopsy and/or surgical resection very important in obtaining definitive characterization; nevertheless, MRI with intra-extravascular and hepatobiliary (dual phase) agents, is an important tool not only in differential subtypes definition but even in surveillance with early identification of complications and discovery of some signs of HCA malignant degeneration. Inflammation, abnormal rich vascularisation, peliotic areas, and abundant fatty infiltration are pathologic findings differently present in the HCA subtypes and they may be detected by multiparametric MRI approach. Lesion enlargement and heterogeneity of signal intensity and of contrast enhancement are signs to be considered in malignant transformation. The purpose of this paper is to present the state of the art of MRI in the diagnosis of HCA and subtype characterization, with particular regard to morphologic and functional information available with dual phase contrast agents, and to discuss differential diagnosis with the most common benign and malignant lesions mimicking HCAs.

Hepatocellular adenoma and focal nodular hyperplasia: value of gadoxetic acid-enhanced MR imaging in differential diagnosis.

PURPOSE: To retrospectively evaluate the utility of gadoxetic acid-enhanced magnetic resonance (MR) imaging in the differential diagnosis of hepatocellular adenoma (HCA) and focal nodular hyperplasia (FNH). MATERIALS AND METHODS: This study had institutional review board approval; the requirement for informed consent was waived. Eighty-two patients (58 patients with FNH and 24 patients with HCAs) with 111 lesions were included in the study. There were 74 female patients and eight male patients (mean age, 41.9 years±13.2 [standard deviation]; age range, 11-78 years). Two readers reviewed all images in terms of signal intensity (SI) features on unenhanced, dynamic, and hepatobiliary phase images. For quantitative analysis, contrast enhancement ratio (CER), lesion-to-liver contrast (LLC), and SI ratio on dynamic and hepatobiliary phase images were calculated. RESULTS: The CER of FNH in the arterial phase (mean, 94.3%±33.2) was significantly higher than that of HCAs (mean, 59.3%±28.1) (P<.0001). During the hepatobiliary phase, the LLC of FNH showed minimally positive values (mean, 0.05±0.01) and that of HCAs demonstrated strong negative values (mean, -0.67±0.24) (P<.0001). The area under the receiver operating characteristic curve of the hepatobiliary phase SI ratio for differentiation of the two tumors was 0.97, and a sensitivity of 92% and specificity of 91% were found with a cutoff value of 0.87. Among six FNH lesions that showed atypical hypointensity during the hepatobiliary phase, four had a large central scar, one contained a substantial fat component, and one had abundant radiating fibrous septa. Three HCAs were isointense during the hepatobiliary phase owing to severe hepatic steatosis. CONCLUSION: Gadoxetic acid-enhanced MR imaging facilitates the differentiation of FNH from HCA.

A review of the actual knowledge of the processes governing growth and development of long bones.

Autoptic samples of human bones (from 8 weeks of gestation to 12 years of age) and a second group of serial, skeletal x-rays (required for pathologies not related to bone dysplasia in children from 4 months to 17 years of age) provided the material for the analysis of the physes normal growth mechanism presented in this review. Before the appearance of the ossification centers epiphyseal growth rests exclusively on chondrocytes proliferation (interstitial growth), without any detectable differentiated cellular organization. When endochondral ossification starts a defined spatial disposition of chondrocytes and a corresponding organization of the intercellular matrix is set up, so that it is possible to identify a growth vector corresponding to the columns of piled chondrocytes with direction from hypertrophic toward the proliferative cell layers. The complexity of the tubular bones growth process is well represented by the spatial arrangement of the growth vectors. In the late epiphyseal growth another mechanism is active in addition to endochondral ossification, namely, articular cartilage interstitial growth and subchondral remodelling. The knowledge of the normal mode of organization of the physis and its temporal sequence can help to better understand of the deviaton from the normal development of metaphyseal and epiphyseal dysplasias.

Solid hypervascular liver lesions: accurate identification of true benign lesions on enhanced dynamic and hepatobiliary phase magnetic resonance imaging after gadobenate dimeglumine administration.

PURPOSE: To evaluate hepatobiliary phase magnetic resonance imaging with gadobenate dimeglumine for differentiation of benign hypervascular liver lesions from malignant or high-risk lesions. METHODS AND MATERIALS: Retrospective assessment was performed of 550 patients with 910 hypervascular lesions (302 focal nodular hyperplasia [FNH], 82 nodular regenerative hyperplasia [NRH], 59 hepatic adenoma or liver adenomatosis [HA/LA], 329 hepatocellular carcinomas [HCC], 12 fibrolamellar-HCC [FL-HCC], 21 peripheral cholangiocarcinomas [PCC], 105 metastases). Imaging was performed before and during the arterial, portal-venous, equilibrium, and hepatobiliary phases after gadobenate dimeglumine administration (0.05 mmol/kg). Histologic confirmation was available for ≥1 lesion per patient, except for patients with suspected FNH (diagnosis based on characteristic enhancement/follow-up). Lesion differentiation (benign/malignant) on the basis of contrast washout and lesion enhancement (hypo-/iso-/hyperintensity) was assessed (sensitivity, specificity, accuracy, PPV, and NPV) relative to histology or final diagnosis. RESULTS: On portal-venous or equilibrium phase images, washout was not seen for 208 of 526 (39.5%) malignant (HCC, FL-HCC, PCC, metastases) and high-risk (HA/LA) lesions. Conversely, only 5 of 384 (1.3%) true benign lesions (FNH/NRH) showed washout. Taking washout as indicating malignancy, the sensitivity, specificity, and accuracy for malignant lesion identification during these phases was 61.8%, 98.7%, and 77.4%. On hepatobiliary phase images, 289 of 302 FNH, 82 of 82 NRH, 1 of 59 HA or LA, 62 of 341 HCC or FL-HCC, and 2 of 105 metastases were hyperintense or isointense. Taking iso- or hyperintensity as an indication for lesion benignity, the sensitivity, specificity, accuracy, PPV, and NPV for benign lesion identification was 96.6%, 87.6%, 91.4%, 85.1%, and 97.3%, respectively. CONCLUSIONS: Hepatobiliary phase imaging with gadobenate dimeglumine is accurate for distinguishing benign lesions from malignant or high-risk lesions. Biopsy should be considered for hypointense lesions on hepatobiliary phase images after gadobenate dimeglumine.