Disease severity prognostication in primary sclerosing cholangitis: a validation of the Anali scores and comparison with the potential functional stricture.

OBJECTIVES: Our aim was twofold. First, to validate Anali scores with and without gadolinium (ANALIGd and ANALINoGd) in primary sclerosing cholangitis (PSC) patients. Second, to compare the ANALIs prognostic ability with the recently-proposed potential functional stricture (PFS). MATERIALS AND METHODS: This retrospective study included 123 patients with a mean age of 41.5 years, who underwent gadoxetic acid-enahnced MRI (GA-MRI). Five readers independently evaluated all images for calculation of ANALIGd and ANALINoGd scores based upon following criteria: intrahepatic bile duct change severity, hepatic dysmorphia, liver parenchymal heterogeneity, and portal hypertension. In addition, hepatobiliary contrast excretion into first-order bile ducts was evaluated on 20-minute hepatobiliary-phase (HBP) images to assess PFS. Inter- and intrareader agreement were calculated (Fleiss´and Cohen kappas). Kaplan-Meier curves were generated for survival analysis. ANALINoGd, ANALIGd, and PFS were correlated with clinical scores, labs and outcomes (Cox regression analysis). RESULTS: Inter-reader agreement was almost perfect (Ï° = 0.81) for PFS, but only moderate-(Ï° = 0.55) for binary ANALINoGd. For binary ANALIGd, the agreement was slightly better on HBP (Ï° = 0.64) than on arterial-phase (AP) (Ï° = 0.53). Univariate Cox regression showed that outcomes for decompensated cirrhosis, orthotopic liver transplantation or death significantly correlated with PFS (HR (hazard ratio) = 3.15, p < 0.001), ANALINoGd (HR = 6.42, p < 0.001), ANALIGdHBP (HR = 3.66, p < 0.001) and ANALIGdAP (HR = 3.79, p < 0.001). Multivariate analysis identified the PFS, all three ANALI scores, and Revised Mayo Risk Score as independent risk factors for outcomes (HR 3.12, p < 0.001; 6.12, p < 0.001; 3.56, p < 0.001;3.59, p < 0.001; and 4.13, p < 0.001, respectively). CONCLUSION: ANALINoGd and GA-MRI-derived ANALI scores and PFS could noninvasively predict outcomes in PSC patients. CLINICAL RELEVANCE STATEMENT: The combined use of Anali scores and the potential functional stricture (PFS), both derived from unenhanced-, and gadoxetic acid enhanced-MRI, could be applied as a diagnostic and prognostic imaging surrogate for counselling and monitoring primary sclerosing cholangitis patients. KEY POINTS: Primary sclerosing cholangitis patients require radiological monitoring to assess disease stability and for the presence and type of complications. A contrast-enhanced MRI algorithm based on potential functional stricture and ANALI scores risk-stratified these patients. Unenhanced ANALI score had a high negative predictive value, indicating some primary sclerosing cholangitis patients can undergo non-contrast MRI surveillance.

Multisocietal European consensus on the terminology, diagnosis, and management of patients with synchronous colorectal cancer and liver metastases: an E-AHPBA consensus in partnership with ESSO, ESCP, ESGAR, and CIRSE.

BACKGROUND: Contemporary management of patients with synchronous colorectal cancer and liver metastases is complex. The aim of this project was to provide a practical framework for care of patients with synchronous colorectal cancer and liver metastases, with a focus on terminology, diagnosis, and management. METHODS: This project was a multiorganizational, multidisciplinary consensus. The consensus group produced statements which focused on terminology, diagnosis, and management. Statements were refined during an online Delphi process, and those with 70 per cent agreement or above were reviewed at a final meeting. Iterations of the report were shared by electronic mail to arrive at a final agreed document comprising 12 key statements. RESULTS: Synchronous liver metastases are those detected at the time of presentation of the primary tumour. The term 'early metachronous metastases' applies to those absent at presentation but detected within 12 months of diagnosis of the primary tumour, the term 'late metachronous metastases' applies to those detected after 12 months. 'Disappearing metastases' applies to lesions that are no longer detectable on MRI after systemic chemotherapy. Guidance was provided on the recommended composition of tumour boards, and clinical assessment in emergency and elective settings. The consensus focused on treatment pathways, including systemic chemotherapy, synchronous surgery, and the staged approach with either colorectal or liver-directed surgery as first step. Management of pulmonary metastases and the role of minimally invasive surgery was discussed. CONCLUSION: The recommendations of this contemporary consensus provide information of practical value to clinicians managing patients with synchronous colorectal cancer and liver metastases.

The multi-societal European consensus on the terminology, diagnosis and management of patients with synchronous colorectal cancer and liver metastases: an E-AHPBA consensus in partnership with ESSO, ESCP, ESGAR, and CIRSE.

BACKGROUND: Contemporary management of patients with synchronous colorectal cancer and liver metastases is complex. The aim of this project was to provide a practical framework for care of patients with synchronous colorectal cancer and liver metastases with a focus on terminology, diagnosis and management. METHODS: This project was a multi-organisational, multidisciplinary consensus. The consensus group produced statements which focused on terminology, diagnosis and management. Statements were refined during an online Delphi process and those with 70% agreement or above were reviewed at a final meeting. Iterations of the report were shared by electronic mail to arrive at a final agreed document comprising twelve key statements. RESULTS: Synchronous liver metastases are those detected at the time of presentation of the primary tumour. The term "early metachronous metastases" applies to those absent at presentation but detected within 12 months of diagnosis of the primary tumour with "late metachronous metastases" applied to those detected after 12 months. Disappearing metastases applies to lesions which are no longer detectable on MR scan after systemic chemotherapy. Guidance was provided on the recommended composition of tumour boards and clinical assessment in emergency and elective settings. The consensus focused on treatment pathways including systemic chemotherapy, synchronous surgery and the staged approach with either colorectal or liver-directed surgery as first step. Management of pulmonary metastases and the role of minimally invasive surgery was discussed. CONCLUSIONS: The recommendations of this contemporary consensus provide information of practical value to clinicians managing patients with synchronous colorectal cancer and liver metastases.

Branch-duct intraductal papillary mucinous neoplasm (IPMN): Are cyst volumetry and other novel imaging features able to improve malignancy prediction compared to well-established resection criteria?

OBJECTIVES: Current guidelines base the management of intraductal papillary mucinous neoplasms (IPMN) on several well-established resection criteria (RC), including cyst size. However, malignancy may occur in small cysts. Since branch-duct (BD) IPMN are not perfect spheres, volumetric and morphologic analysis might better correlate with mucin production and grade of dysplasia. Nonetheless, their role in malignancy (high-grade dysplasia/invasive cancer) prediction has been poorly investigated. Previous studies evaluating RC also included patients with solid-mass-forming pancreatic cancer (PC), which may affect the RC yield. This study aimed to assess the role of volume, morphology, and other well-established RC in malignancy prediction in patients with BD- and mixed-type IPMN after excluding solid masses. METHODS: Retrospective ethical review-board-approved study of 106 patients (2008-2019) with histopathological diagnosis of BD- and mixed-type IPMN (without solid masses) and preoperative MRI available. Standard imaging and clinical features were collected, and the novel imaging features cyst-volume and elongation value [EV = 1 - (width/length)] calculated on T2-weighted images. Logistic regression analysis was performed. Statistical significance set at two-tails, p < 0.05. RESULTS: Neither volume (odds ratio (OR) = 1.01, 95% CI: 0.99-1.02, p = 0.12) nor EV (OR = 0.38, 95% CI: 0.02-5.93, p = 0.49) was associated with malignancy. Contrast-enhancing mural nodules (MN), main pancreatic duct (MPD) ≥ 5 mm, and elevated carbohydrate antigen (CA) 19-9 serum levels (> 37 µmol/L) were associated with malignancy (MN OR: 4.32, 95% CI: 1.18-15.76, p = 0.02; MPD ≥ 5 mm OR: 4.2, 95% CI: 1.34-13.1, p = 0.01; CA19-9 OR: 6.72; 95% CI: 1.89 - 23.89, p = 0.003). CONCLUSIONS: Volume and elongation value cannot predict malignancy in BD- and/or mixed-type IPMN. Mural nodules, MPD ≥ 5 mm and elevated CA19-9 serum levels are associated with higher malignancy risk even after the exclusion of solid masses. KEY POINTS: • Novel and well-established resection criteria for IPMN have been evaluated after excluding solid masses. • BD-IPMN volume and elongation value cannot predict malignancy. • Main pancreatic duct ≥ 5 mm, mural nodules, and elevated carbohydrate antigen 19-9 levels are associated with malignancy.

Consensus report from the 9th International Forum for Liver Magnetic Resonance Imaging: applications of gadoxetic acid-enhanced imaging.

OBJECTIVES: The 9th International Forum for Liver Magnetic Resonance Imaging (MRI) was held in Singapore in September 2019, bringing together radiologists and allied specialists to discuss the latest developments in and formulate consensus statements for liver MRI, including the applications of gadoxetic acid-enhanced imaging. METHODS: As at previous Liver Forums, the meeting was held over 2 days. Presentations by the faculty on days 1 and 2 and breakout group discussions on day 1 were followed by delegate voting on consensus statements presented on day 2. Presentations and discussions centered on two main meeting themes relating to the use of gadoxetic acid-enhanced MRI in primary liver cancer and metastatic liver disease. RESULTS AND CONCLUSIONS: Gadoxetic acid-enhanced MRI offers the ability to monitor response to systemic therapy and to assist in pre-surgical/pre-interventional planning in liver metastases. In hepatocellular carcinoma, gadoxetic acid-enhanced MRI provides precise staging information for accurate treatment decision-making and follow-up post therapy. Gadoxetic acid-enhanced MRI also has potential, currently investigational, indications for the functional assessment of the liver and the biliary system. Additional voting sessions at the Liver Forum debated the role of multidisciplinary care in the management of patients with liver disease, evidence to support the use of abbreviated imaging protocols, and the importance of standardizing nomenclature in international guidelines in order to increase the sharing of scientific data and improve the communication between centers. KEY POINTS: • Gadoxetic acid-enhanced MRI is the preferred imaging method for pre-surgical or pre-interventional planning for liver metastases after systemic therapy. • Gadoxetic acid-enhanced MRI provides accurate staging of HCC before and after treatment with locoregional/biologic therapies. • Abbreviated protocols for gadoxetic acid-enhanced MRI offer potential time and cost savings, but more evidence is necessary. The use of gadoxetic acid-enhanced MRI for the assessment of liver and biliary function is under active investigation.

Inter-reader agreement of interpretation of radiological course of bile duct changes between serial follow-up magnetic resonance imaging/3D magnetic resonance cholangiopancreatography of patients with primary sclerosing cholangitis.

Objectives: Interpretation of MRI/MRCP in primary sclerosing cholangitis (PSC) at a single time point has low inter-reader agreement. Agreement of interpretation of the dynamic course of duct changes in follow-up MRI/MRCP is of clinical importance but remains unknown. Our aims are therefore to assess the inter-reader agreement of interpretation of the course of duct changes in PSC and investigate if elimination of 3 D MRCP affects inter-reader agreement.Materials and Methods: We studied 40 consecutive PSC-patients who underwent two liver MRI/MRCPs at two time points. Two readers independently evaluated the course of duct changes between the two time points in two imaging sets, one with and one without 3 D MRCP. The intraclass correlation coefficient (ICC) was calculated for evaluation of inter-reader and intra-reader agreement between the two time points and two imaging sets accordingly.Results: Inter-reader agreement of the interpretation of the course of duct changes between the two time points was poor (ICC up to 0.224). Elimination of 3 D MRCP neither improved inter-reader agreement which was again poor (ICC up to 0.26) nor did it change considerably the way readers interpret the course of ducts changes (ICC for intra-reader agreement between 0.809 and 0.978).Conclusions: Inter-reader agreement of the interpretation of radiological course of duct changes is poor in serial follow-up MRI/MRCP of PSC-patients. Elimination of 3 D MRCP does not increase inter-reader agreement but maintains an excellent intra-reader agreement for the interpretation of the dynamic course of bile duct changes.Key pointsInter-reader agreement of interpretation of radiological course of bile duct changes between serial follow-up MRI/MRCP examinations of patients with PSC is poor.Absence of 3D MRCP does not affect considerably the way readers interpret the radiological course of bile ducts changes.When MRCP is absent or of low quality, utilization of other sequences seems to be helpful as an alternative for bile duct evaluation.

Diagnosis, treatment and long-term outcome of autoimmune pancreatitis in Sweden.

INTRODUCTION: Autoimmune pancreatitis (AIP) is a pancreatic inflammatory process characterized by a strong inflammatory cell infiltration and two histopathologically distinct subtypes: type 1 and type 2. Diagnosis is often challenging and requires a combination of clinical, laboratory and imaging data. AIP can mimic pancreatic tumours leading to unnecessary resections if not correctly diagnosed. Short- and long-term outcomes of AIP have been poorly investigated so far and no large series have been previously reported from Sweden. METHODS: A single-centre, retrospective, cohort study of patients with histologically confirmed or highly probable diagnosis of AIP according to ICDC criteria. Demographic, clinical and radiological characteristics, type of treatment and its outcomes were collected and analysed. RESULTS: Seventy-one patients with AIP (87% with type 1), were evaluated at Karolinska University Hospital between 2004 and 2018; 49% males, mean age 49 years (range 44-53). Among them, 28% were histologically confirmed, 35% presented with jaundice, 22% with acute pancreatitis, 39% had non-specific symptoms such as weight loss or abdominal pain, 84% showed other organ involvement (OOI). Radiologically, 76% showed a focal pancreatic enlargement, 27% diffuse enlargement, 27% signs of acute pancreatitis and 10% of chronic pancreatitis. Overall, 58 patients (81%) underwent treatment with different medications: 46 (79%) cortisone, 7 (12%) azathioprine, 5 (8%) other immunosuppressive drugs. Twenty-six (36%) underwent biliary stenting and 12 (16%) were given surgery. In total, 47% of patients developed pancreatic exocrine insufficiency (PEI), of whom 76% had a severe form (faecal elastase-1 < 100 µg/g) and 21% of patients developed diabetes mellitus (pancreatic endocrine insufficiency), of whom 73% required insulin. CONCLUSIONS: AIP is a challenging disease for diagnosis and treatment. Cortisone treatment is generally successful and provides clinical remission in the large majority of patients (>90%). In the further course of the disease, a considerable number of patients develop PEI and diabetes. Only one-quarter of patients exhibit on imaging the characteristic "sausage-like" pancreas (diffuse enlargement), approximately three-quarters had a focal mass that could be misdiagnosed as pancreatic malignancy.

The Impact of a Hepatobiliary Multidisciplinary Team Assessment in Patients with Colorectal Cancer Liver Metastases: A Population-Based Study.

BACKGROUND: Assessing patients with colorectal cancer liver metastases (CRCLM) by a liver multidisciplinary team (MDT) results in higher resection rates and improved survival. The aim of this study was to evaluate the potentially improved resection rate in a defined cohort if all patients with CRCLM were evaluated by a liver MDT. PATIENTS AND METHODS: A retrospective analysis of patients diagnosed with colorectal cancer during 2008 in the greater Stockholm region was conducted. All patients with liver metastases (LM), detected during 5-year follow-up, were re-evaluated at a fictive liver MDT in which previous imaging studies, tumor characteristics, medical history, and patients' own treatment preferences were presented. Treatment decisions for each patient were compared to the original management. Odds ratios (ORs) and 95% confidence intervals were estimated for factors associated with referral to the liver MDT. RESULTS: Of 272 patients diagnosed with LM, 102 patients were discussed at an original liver MDT and 69 patients were eventually resected. At the fictive liver MDT, a further 22 patients were considered as resectable/potentially resectable, none previously assessed by a hepatobiliary surgeon. Factors influencing referral to liver MDT were age (OR 3.12, 1.72-5.65), American Society of Anaesthesiologists (ASA) score (OR 0.34, 0.18-0.63; ASA 2 vs. ASA 3), and number of LM (OR 0.10, 0.04-0.22; 1-5 LM vs. >10 LM), while gender (p = .194) and treatment at a teaching hospital (p = .838) were not. CONCLUSION: A meaningful number of patients with liver metastases are not managed according to best available evidence and the potential for higher resection rates is substantial. IMPLICATIONS FOR PRACTICE: Patients with liver metastatic colorectal cancer who are assessed at a hepatobiliary multidisciplinary meeting achieve higher resection rates and improved survival. Unfortunately, patients who may benefit from resection are not always properly referred. In this study, the potential improved resection rate was assessed by re-evaluating all patients with liver metastases from a population-based cohort, including patients with extrahepatic metastases and accounting for comorbidity and patients' own preferences towards treatment. An additional 12.9% of the patients were found to be potentially resectable. The results highlight the importance of all patients being evaluated in the setting of a hepatobiliary multidisciplinary meeting.

Survival Analysis and Risk for Progression of Intraductal Papillary Mucinous Neoplasia of the Pancreas (IPMN) Under Surveillance: A Single-Institution Experience.

PURPOSE: While surveillance of the majority of patients with IPMN is considered best practice, consensus regarding the duration of follow-up is lacking. This study assessed the survival rate and risk for progression of IPMN under surveillance. METHODS: All patients diagnosed with and surveyed for IPMN between January 2008 and December 2013 were identified and assigned to two groups: patients without indication for surgery (Group 1), and patients whose IPMN required surgery but were inoperable for general reasons (Group 2). Disease progression and survival data were compared between both groups. RESULTS: In total 503 patients were identified, of whom 444 (88.3%) were followed up. Group 1 included 395 patients, and Group 2 had 49. In Group 1, IPMN-specific 1-, 5-, and 10-year survival rates were 100, 100, and 94.2%, respectively. Four patients died of associated or concomitant pancreatic cancer, and 230 patients (58.2%) experienced disease progression. The 1-, 4-, 10-year cumulative risk for progression and for surgery was 11.2, 70.6, 97.5, and 2.9, 26.2, 72.1%, respectively. In Group 2, the 1-, 5-, 10-year IPMN-specific survival rate was 90.7, 74.8, and 74.8%, respectively. CONCLUSIONS: This study confirmed the safety of surveillance for patients with IPMN who do not require surgery. However, the risk for disease progression and for surgery increases significantly over time. The study results support International and European guidelines not to discontinue IPMN surveillance and validate the European recommendation to intensify follow-up after 5 years. The fairly good prognosis of patients whose IPMN requires surgery but cannot undergo resection suggests a relatively indolent disease biology.

Pancreatic MRI for the surveillance of cystic neoplasms: comparison of a short with a comprehensive imaging protocol.

OBJECTIVES: The study aims were to evaluate: (1) whether a short-protocol (SP) MRI for the surveillance of pancreatic cystic neoplasms (PCN) provides equivalent clinical information as a comprehensive-protocol (CP), and (2) the cost reduction from substituting CP with SP for patient surveillance. METHODS: This retrospective study included 154 consecutive patients (median age: 66, 52 % men) with working-diagnosis of PCN and available contrast-enhanced MRI/MRCP. Three radiologists evaluated independently two imaging sets (SP/CP) per patient. The CP included: T2-weighted (HASTE/MRCP), DWI and T1-weighted (chemical-shift/pre-/post-contrast) images [acquisition time (AT) ≈ 35 min], whereas the SP included: T2-weighted HASTE and T1-weighted pre-contrast images (AT ≈ 8 min). Mean values of largest cyst/main pancreatic duct diameter (DC/DMPD) were compared. Agreement regarding presence/absence of cystic/MPD mural nodules (MNC/MNMPD), inter-observer agreement and cost differences between SP/CP were calculated. RESULTS: For DC and DMPD, mean values with SP/CP were 21.4/21.7 mm and 3.52/3.58 mm, while mean differences SP-CP were 0.3 mm (p = 0.02) and 0.06 mm (p = 0.12), respectively. For presence/absence of MNC and MNMPD, SP/CP coincided in 93 % and 98 % of cases, respectively. Inter-observer agreement was strong for SP/CP. SP-cost was 25 % of CP-cost. CONCLUSIONS: For the surveillance of PCN, short-protocol MRI provides information equivalent to the more time-consuming and costly comprehensive-protocol. KEY POINTS: • Pancreatic cystic neoplasms (PCN) are increasingly diagnosed in the general population. • Multiple imaging controls are recommended for the surveillance of patients with PCN. • Short and comprehensive MRI-protocols are equivalent for decision-making in PCN under surveillance. • Evaluation of imaging risk factors in PCNs is comparable with both MRI-protocols. • Use of the short MRI-protocol may rationalise healthcare resources.

Successful Hematopoietic Stem Cell Transplantation in a Patient with LPS-Responsive Beige-Like Anchor (LRBA) Gene Mutation.

PURPOSE: Autosomal recessive mutations in LRBA, encoding for LPS-responsive beige-like anchor protein, were described in patients with a common variable immunodeficiency (CVID)-like disease characterized by hypogammaglobulinemia, autoimmune cytopenias, and enteropathy. Here, we detail the clinical, immunological, and genetic features of a patient with severe autoimmune manifestations. METHODS: Whole exome sequencing was performed to establish a molecular diagnosis. Evaluation of lymphocyte subsets was performed for immunological characterization. Medical files were reviewed to collect clinical and immunological data. RESULTS: A 7-year-old boy, born to consanguineous parents, presented with autoimmune hemolytic anemia, hepatosplenomegaly, autoimmune thyroiditis, and severe autoimmune gastrointestinal manifestations. Immunological investigations revealed low immunoglobulin levels and low numbers of B and NK cells. Treatment included immunoglobulin replacement and immunosuppressive therapy. Seven years after disease onset, the patient developed severe neurological symptoms resembling acute disseminated encephalomyelitis, prompting allogeneic hematopoietic stem cell transplantation (HSCT) with the HLA-identical mother as donor. Whole exome sequencing of the patient uncovered a homozygous 1 bp deletion in LRBA (c.7162delA:p.T2388Pfs*7). Importantly, during 2 years of follow-up post-HSCT, marked clinical improvement and recovery of immune function was observed. CONCLUSIONS: Our data suggest a beneficial effect of HSCT in patients with LRBA deficiency.

A phase I dose escalation trial of AXP107-11, a novel multi-component crystalline form of genistein, in combination with gemcitabine in chemotherapy-naive patients with unresectable pancreatic cancer.

BACKGROUND: AXP107-11 is a novel, multi-component crystalline form of the naturally occurring compound genistein. AXP107-11 has improved physiochemical properties and oral bioavailability compared to the natural form of genistein, and it is possible that combining AXP107-11 with chemotherapy may increase the effect and reduce chemoresistance. The purpose of this dose escalation phase Ib study was to assess the safety, maximum tolerated dose (MTD) and pharmacokinetics (PK) of AXP107-11 in combination with gemcitabine in treatment-naïve patients with inoperable pancreatic carcinoma. PATIENTS AND METHODS: AXP107-11 was given orally in escalating doses (400 mg-1600 mg daily) in combination with standard gemcitabine treatment (1000 mg/m(2)/week) for the first seven of eight weeks and thereafter for a maximum of four × four-week treatment cycles. PK, safety, MTD and efficacy of AXP107-11 in combination with gemcitabine were evaluated. RESULTS: Sixteen patients were enrolled and received AXP107-11. The maximum concentration in serum of unconjugated (free) genistein was 1 µM. Neither dose-limiting toxicities (DLTs) nor signs of hematological or non-hematological toxicities related to AXP107-11 were observed over a period ranging from 0.7 to 13.2 months. The median overall survival time was 4.9 months (range 1.5-19.5 months). Seven patients (44%) survived longer than six months and 19% were alive at the one-year follow-up. CONCLUSION: Treatment of pancreatic cancer patients with AXP107-11 in combination with gemcitabine resulted in a favorable PK-profile with high serum levels without signs of either hematological or non-hematological toxicity. Accordingly, we suggest further studies with AXP107-11 in pancreatic cancer patients.

Perfusion computed tomography for detection of hepatocellular carcinoma in patients with liver cirrhosis.

PURPOSE: To evaluate the diagnostic performance of dynamic perfusion CT (P-CT) for detection of hepatocellular carcinoma (HCC) in the cirrhotic liver. MATERIALS AND METHODS: Twenty-six cirrhotic patients (19 men, aged 69 ± 10 years) with suspicion of HCC prospectively underwent P-CT of the liver using the 4D spiral-mode (100/80 kV; 150/175mAs/rot) of a dual-source system. Two readers assessed: (1) arterial liver-perfusion (ALP), portal-venous liver-perfusion (PLP) and hepatic perfusion-index (HPI) maps alone; and (2) side-by-side with maximum-intensity-projections of arterial time-points (art-MIP) for detection of HCC using histopathology and imaging follow-up as standard of reference. Another reader quantitatively assessed perfusion maps of detected lesions. RESULTS: A total of 48 HCCs in 21/26 (81%) patients with a mean size of 20 ± 10 mm were detected by histopathology (9/48, 19%) or imaging follow-up (39/48, 81%). Detection rates (Reader1/Reader2) of HPI maps and side-by-side analysis of HPI combined with arterial MIP were 92/88% and 98/96%, respectively. Positive-predictive values were 63/63% and 68/71%, respectively. A cut-off value of ≥85% HPI and ≥99% HPI yielded a sensitivity and specificity of 100%, respectively, for detection of HCC. CONCLUSION: P-CT shows a high sensitivity for detection of HCC in the cirrhotic liver. Quantitative assessment has the potential to reduce false-positive findings improving the specificity of HCC diagnosis. KEY POINTS: • Visual analysis of perfusion maps shows good sensitivity for detection of HCC. • Additional assessment of anatomical arterial MIPs further improves detection rates of HCC. • Quantitative perfusion analysis has the potential to reduce false-positive findings. • In cirrhotic livers, a hepatic-perfusion-index ≥ 9 9% might be specific for HCC.

The effect of smoking on clinical and radiographic variables, and acute phase reactants in patients with ankylosing spondylitis.

To investigate the association between smoking and clinical, inflammatory and radiographic parameters in patients with ankylosing spondylitis (AS). One hundred and six tumour necrosis factor inhibitor naïve patients with AS were included in the study. The erythrocyte sedimentation rate, C-reactive protein, Bath AS Disease Activity Index (BASDAI), Bath AS Functional Index (BASFI) and modified Stroke AS Spine Score (mSASSS) were assessed cross-sectionally for each patient. Smoking history was obtained, and smoking pack years were calculated. Current smokers had significantly higher BASDAI (p < 0.001) and a trend for higher BASFI (p = 0.059). Ever smokers had significantly higher BASFI (p = 0.035) and a trend for higher mSASSS (p = 0.063) compared to never smokers. Pack years (smoking intensity) were positively correlated with duration of inflammatory back pain (r = 0.628, p < 0.001), BASFI (r = 0.443, p < 0.001) and mSASSS (r = 0.683, p < 0.001). Multivariate regression analyses showed that current smoking was independently associated with a higher BASDAI score [regression coefficient (B) = 14.75, p < 0.001] and increasing pack years were independently associated with higher mSASSS (B = 0.26, p = 0.005). In patients with AS, current smoking was strongly and independently associated with higher disease activity, and cumulative smoking exposure with more radiographic spinal damage. In AS smokers, smoking cessation should be strongly recommended.

Comparison of preoperative conference-based diagnosis with histology of cystic tumors of the pancreas.

BACKGROUND: Diagnostic errors in the evaluation of cystic neoplasms of the pancreas (PCNs) are quite common. Few data are available regarding the impact of these errors on clinical management. The aim of this study was to determine the accuracy of a pancreatic multidisciplinary conference in diagnosing PCNs, to assess the potential risk of misdiagnosis, and to evaluate the clinical impact of these errors. METHODS: A retrospective consecutive series of patients undergoing surgery for PCNs at Karolinska University Hospital between 2004 and 2012 was analyzed. RESULTS: During the study period, a total of 141 patients had undergone pancreatic resection for PCN. The overall accuracy of the preoperative diagnosis was 60.9 %. The rate of concordance between preoperative diagnosis and histology was similar for asymptomatic and symptomatic lesions (62.8 vs. 59.1 %; p = NS). The rate of correct diagnosis increased over time (54.5 % in 2004-2006, 61.7 % in 2007-2012, 63.5 % in 2010-2012). Univariate analysis identified the location of the lesion (diffuse pancreatic involvement) and a mucinous nature of the lesion as factors conducive to a correct diagnosis. Reevaluation of the original indication for surgery in light of the exact diagnosis showed that a surgical procedure should not have been performed in 12 patients (8.5 %). CONCLUSIONS: This study confirms that diagnostic errors are fairly common in the preoperative assessment of PCNs, but the errors are clinically relevant in <10 % of patients.

Time-resolved computed tomography of the liver: retrospective, multi-phase image reconstruction derived from volumetric perfusion imaging.

OBJECTIVE: To assess feasibility and image quality (IQ) of a new post-processing algorithm for retrospective extraction of an optimised multi-phase CT (time-resolved CT) of the liver from volumetric perfusion imaging. METHODS: Sixteen patients underwent clinically indicated perfusion CT using 4D spiral mode of dual-source 128-slice CT. Three image sets were reconstructed: motion-corrected and noise-reduced (MCNR) images derived from 4D raw data; maximum and average intensity projections (time MIP/AVG) of the arterial/portal/portal-venous phases and all phases (total MIP/ AVG) derived from retrospective fusion of dedicated MCNR split series. Two readers assessed the IQ, detection rate and evaluation time; one reader assessed image noise and lesion-to-liver contrast. RESULTS: Time-resolved CT was feasible in all patients. Each post-processing step yielded a significant reduction of image noise and evaluation time, maintaining lesion-to-liver contrast. Time MIPs/AVGs showed the highest overall IQ without relevant motion artefacts and best depiction of arterial and portal/portal-venous phases respectively. Time MIPs demonstrated a significantly higher detection rate for arterialised liver lesions than total MIPs/AVGs and the raw data series. CONCLUSION: Time-resolved CT allows data from volumetric perfusion imaging to be condensed into an optimised multi-phase liver CT, yielding a superior IQ and higher detection rate for arterialised liver lesions than the raw data series. KEY POINTS: • Four-dimensional computed tomography is limited by motion artefacts and poor image quality. • Time-resolved-CT facilitates 4D-CT data visualisation, segmentation and analysis by condensing raw data. • Time-resolved CT demonstrates better image quality than raw data images. • Time-resolved CT improves detection of arterialised liver lesions in cirrhotic patients.

Oral Manganese Chloride Tetrahydrate: A Novel Magnetic Resonance Liver Imaging Agent for Patients With Renal Impairment: Efficacy, Safety, and Clinical Implication.

ABSTRACT: Manganese-based contrast agents (MBCAs) show promise to complement gadolinium-based contrast agents (GBCAs) in magnetic resonance imaging (MRI) of the liver. Management of patients with focal liver lesions and severely impaired renal function uses unenhanced liver MRI or GBCA-enhanced MRI. However, unenhanced MRI risks reducing patient's survival.Gadolinium-based contrast agents, which help to detect and visualize liver lesions, are associated with increased risk of nephrogenic systemic fibrosis in renally impaired patients, a severe adverse event (AE) with potentially fatal outcome. Therefore, use of GBCA in patients with impaired renal function requires careful consideration. Other concerns are related to tissue deposition in the brain and other organs due to lack of gadolinium clearance, which could lead to concerns also for other patient populations, for example, those exposed to multiple procedures with GBCA. Of particular concern are the linear chelates that remain available for liver MRI, where there is no replacement technology. This has highlighted the urgency for safer alternatives.An alternative may be the drug candidate Ascelia-MBCA (ACE-MBCA, Orviglance), oral manganese chloride tetrahydrate. This candidate effectively visualizes and detects focal liver lesions, as demonstrated in 8 clinical studies on 201 adults (healthy or with known or suspected focal liver lesions). ACE-MBCA has a low and transient systemic exposure, which is likely the reason for its beneficial safety profile. The AEs were primarily mild and transient, and related to the gastrointestinal tract. This new, orally administered product may offer a simple imaging approach, allowing appropriate patient management in renally impaired patients when use of GBCA requires careful consideration.In this review, we highlight the clinical development of ACE-MBCA-a novel, liver-specific contrast agent. We begin with a brief overview of manganese properties, addressing the need for MBCAs and describing their optimal properties. We then review key findings on the novel agent and how this allows high-quality MRIs that are comparable to GBCA and superior to unenhanced MRI. Lastly, we provide our view of future perspectives that could advance the field of liver imaging, addressing the medical needs of patients with focal liver lesions and severe renal impairment.Our review suggests that ACE-MBCA is a promising, effective, and well-tolerated new tool in the radiologist's toolbox.

Autoimmune Pancreatitis Type 1 with Biliary, Nasal, Testicular, and Pulmonary Involvement: A Case Report and a Systematic Review.

INTRODUCTION: Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated condition associated with fibroinflammatory lesions that can occur at almost any anatomical site. It often presents as a multiorgan disease that may mimic malignancy, infection, or other immune-mediated conditions. Autoimmune pancreatitis (AIP) type 1 is the most prominent manifestation of IgG4-RD in the digestive tract, with common extra-pancreatic inflammation. We present the first patient with AIP and involvement of the testicles and nasal cavity. PATIENT AND METHODS: A case of a patient with AIP type 1 and other organ involvement (bile ducts, testicles, nasal polyps, and lungs) is described. Additionally, a systematic review of AIP type 1 with testicular and nasal involvement was conducted. RESULTS: The systematic review found two cases of AIP type 1 with testicular involvement and 143 cases with AIP type 1 with nasal cavity involvement. None of them had both testicular and nasal involvement. CONCLUSIONS: This is the first case of AIP type 1 with other organ involvement, including testicular and nasal involvement, to be described. The number of patients with nasal and testicular involvement described in the literature is low. Creating awareness of this rare clinical condition is necessary, especially due to the very effective available treatment with corticosteroids and rituximab.