Characterization of phenotypic spectrum of fetal heterotaxy syndrome by combining ultrasound and magnetic resonance imaging.

OBJECTIVE: Heterotaxy or isomerism of the atrial appendages is a congenital disorder with variable presentation, associated with both cardiac and non-cardiac anomalies, which may have a serious impact on fetal outcome. The aim of this exploratory study was to assess the value of fetal magnetic resonance imaging (MRI), as a complementary tool to ultrasound, for describing the morphological spectrum encountered in heterotaxy. METHODS: This retrospective study included 27 fetuses that underwent fetal MRI following prenatal suspicion of heterotaxy on ultrasound from 1998 to 2019 in a tertiary referral center. Heterotaxy was classified as left atrial isomerism (LAI) or right atrial isomerism (RAI) based on fetal echocardiography (FE) examination. In addition to routine prenatal ultrasound, fetal MRI was offered routinely to enhance the diagnosis of non-cardiac anomalies, which might have been missed on ultrasound. Prenatal findings on ultrasound, FE and MRI were reviewed systematically and compared with those of postnatal imaging and autopsy reports. RESULTS: Twenty-seven fetuses with heterotaxy and cardiovascular pathology, of which 19 (70%) had LAI and eight (30%) had RAI, were included. Seven (7/19 (37%)) fetuses with LAI had normal intracardiac anatomy, whereas all fetuses with RAI had a cardiac malformation. All 27 fetuses had non-cardiac anomalies on fetal MRI, including situs and splenic anomalies. In 12/19 (63%) fetuses with LAI, a specific abnormal configuration of the liver was observed on MRI. In three fetuses, fetal MRI revealed signs of total anomalous pulmonary venous connection obstruction. An abnormal bronchial tree pattern was suspected on prenatal MRI in 6/19 (32%) fetuses with LAI and 3/8 (38%) fetuses with RAI. CONCLUSIONS: Visualization on MRI of non-cardiac anomalies in fetuses with suspected heterotaxy is feasible and can assist the complex diagnosis of this condition, despite its limitations. This modality potentially enables differentiation of less severe cases from more complex ones, which may have a poorer prognosis. Fetal MRI can assist in prenatal counseling and planning postnatal management. © 2021 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Correlation of trastuzumab-based treatment with clinical characteristics and prognosis in HER2-positive gastric and gastroesophageal junction cancer: A retrospective single center analysis.

Attempts for identifying targeted therapy strategies in metastatic gastric and gastroesopheal junction cancer (upper-GI) revealed that the inhibition of human epidermal growth factor receptor-2 (HER2) by monoclonal antibody trastuzumab improves survival of these patients. Hence, adding trastuzumab to doublet chemotherapy has become the standard treatment in this setting. Although the patient survival is extended among clinical trials, the knowledge on the real-time setting is limited. With this retrospective, single center analysis of the patient data of the Medical University of Vienna, we sought to investigate the clinical characteristics and outcome of patients, who received trastuzumab-based chemotherapy for metastatic upper-GI tumor. All patients, who received trastzumab at least once were included to the analysis. Clinical and pathological data were recorded. This search revealed 33 patients. The demographic data was comparable with that of the previous clinical trials. Progression free survival (PFS) was 11 months, whereas overall survival (OS) was 21 months. OS was significantly associated with initially favorable response to treatment. Thirteen patients (39%) received trastuzumab as maintenance treatment with a median cycle number of 6. Toxicity profile was acceptable with only one patient detected to have cardiotoxicity. Taken together, trastuzumab based treatment induced a considerable PFS and OS in metastatic or advanced upper-GI tumors with acceptable toxicity profile. The maintenance therapy with trastuzumab was safe and effective in patients who had initially a favorable response to chemotherapy. The optimal duration of the maintenance therapy should be tested in future clinical trials.

[Incidental findings of liver, biliary system, pancreas and spleen in asymptomatic patients : Assessment and management recommendations]. / Zufallsbefunde von Leber, Gallensystem, Pankreas und Milz bei asymptomatischen Patienten : Bewertung und Managementempfehlung.

CLINICAL ISSUE: The increased use of highly developed imaging procedures, such as multidetector-row computed tomography and magnetic resonance imaging has led to a substantial increase of asymptomatic and unexpected findings. STANDARD RADIOLOGICAL METHODS: Abdominal CT investigations are particularly affected with a large number of incidental findings. This valuable diagnostic procedure also entails the risk of complex and cost-intensive subsequent investigations with partly invasive procedures. ACHIEVEMENTS: For this reason radiologists are more often confronted with the difficult task of correctly assessing these lesions, to decide on the need for additional investigations and to inform the patient in detail about the clinical relevance. PRACTICAL RECOMMENDATIONS: The aims of this article are to describe the most common abdominal incidentalomas, to assist with the interpretation and differential diagnosis and to give recommendations for further management.

[Incidental findings of the kidneys, adrenal glands, adnexa uteri, gastrointestinal tract, mesentery and lymph nodes : Assessment and management recommendations]. / Zufallsbefunde von Niere, Nebenniere, Adnexen, Gastrointestinaltrakt, Mesenterium und Lymphknoten : Bewertung und Managementempfehlung.

CLINICAL ISSUE: Besides the upper abdominal parenchymal organs, the increasing application of cross-sectional imaging has also led to a rising number of incidental findings in the kidneys, adrenal glands, adnexa uteri, the gastrointestinal tract, mesentery and abdominal lymph nodes. STANDARD RADIOLOGICAL METHODS: Abdominal computed tomography investigations often show unexpected findings without any correlating symptoms. The growing clinical relevance is due to the large number of incidental findings as well as an increasing awareness of ethical and socioeconomic factors. ACHIEVEMENTS: When interpreting radiological findings not only morphological criteria but also individual risk factors of the patient and the clinical context are of great importance. PRACTICAL RECOMMENDATIONS: The aims of this article are the description and evaluation of frequent incidental findings detected by computed tomography and to provide information about management recommendations.

Impact of sarcopenia on outcome in patients with esophageal resection following neoadjuvant chemotherapy for esophageal cancer.

BACKGROUND: Nutritional status and body composition parameters such as sarcopenia are important risk factors for impaired outcome in patients with esophageal cancer. This study was conducted to evaluate the effect of sarcopenia on long-term outcome after esophageal resection following neoadjuvant treatment. METHODS: Skeletal muscle index (SMI) and body composition parameters were measured in patients receiving neoadjuvant treatment for locally advanced esophageal cancer. Endpoints included relapse-free survival (RFS) and overall survival (OS). RESULTS: The study included 130 patients. Sarcopenia was found in 80 patients (61.5%). Patients with squamous-cell cancer (SCC) showed a decreased median SMI of 48 (range 28.4-60.8) cm/m2 compared with that of patients with adenocarcinoma (AC) of 52 (range 34.4-74.2) cm/m2, P < 0.001. The presence of sarcopenia had a significant impact on patient outcome: HR 1.69 (1.04-2.75), P = 0.036. Median OS was 20.5 (7.36-33.64) versus 52.1 (13.55-90.65) months in sarcopenic and non-sarcopenic patients, respectively. Sarcopenia was identified as an independent risk factor: HR 1.72 (1.049-2.83), P = 0.032. CONCLUSION: Our data provide evidence that sarcopenia impacts long-term outcome after esophageal resection in patients who have undergone neoadjuvant therapy. Assessment of the body composition parameter can be a reasonable part of patient selection and may influence treatment methods.

ESGAR consensus statement on liver MR imaging and clinical use of liver-specific contrast agents.

OBJECTIVES: To develop a consensus and provide updated recommendations on liver MR imaging and the clinical use of liver-specific contrast agents. METHODS: The European Society of Gastrointestinal and Abdominal Radiology (ESGAR) formed a multinational European panel of experts, selected on the basis of a literature review and their leadership in the field of liver MR imaging. A modified Delphi process was adopted to draft a list of statements. Descriptive and Cronbach's statistics were used to rate levels of agreement and internal reliability of the consensus. RESULTS: Three Delphi rounds were conducted and 76 statements composed on MR technique (n = 17), clinical application of liver-specific contrast agents in benign, focal liver lesions (n = 7), malignant liver lesions in non-cirrhotic (n = 9) and in cirrhotic patients (n = 18), diffuse and vascular liver diseases (n = 12), and bile ducts (n = 13). The overall mean score of agreement was 4.84 (SD ±0.17). Full consensus was reached in 22 % of all statements in all working groups, with no full consensus reached on diffuse and vascular diseases. CONCLUSIONS: The consensus provided updated recommendations on the methodology, and clinical indications, of MRI with liver specific contrast agents in the study of liver diseases. KEY POINTS: • Liver-specific contrast agents are recommended in MRI of the liver. • The hepatobiliary phase improves the detection and characterization of hepatocellular lesions. • Liver-specific contrast agents can improve the detection of HCC.

[Modern MRI of the small bowell]. / Moderne MRT des Dünndarms.

CLINICAL METHODOLOGICAL ISSUES: The radiological diagnostics of diseases of the small intestine have undergone a great change in the last two decades. Through rapid progress with new treatments and an increasing therapeutic focus on transmural healing, a complete evaluation of the gastrointestinal tract is now crucial. STANDARD RADIOLOGICAL METHODS: With the introduction of endoscopy, gastrointestinal imaging with a relatively high radiation exposure had only limited applications. The development of cross-sectional imaging allowed a much broader radiological evaluation of abdominal diseases. Due to rapid investigation techniques, excellent soft tissue contrast and the distinct advantage of eliminating exposure to radiation, magnetic resonance imaging (MRI) of the gastrointestinal tract has gained increasing importance. With sufficient filling of the intestinal lumen, simultaneous imaging of all the intestinal wall layers, the perienteric structures and associated abdominal pathologies is now possible. METHODOLOGICAL INNOVATIONS: New MR sequences, such as diffusion-weighted sequences, dynamic contrast-enhanced sequences and MR fluoroscopy, enable the detection of morphological changes, with additional characterization of affected bowel loops as well as the assessment of functional pathologies with dynamic information about intestinal motility disturbances. ACHIEVEMENTS: Recent guidelines of European radiological and gastroenterological organizations have confirmed the importance of cross-sectional imaging and particularly of MRI for diagnostics and follow-up in patients with Crohn's disease. Due to the possibility of assessment of all the layers of the intestinal wall and the presence of extramural complications, MRI has a significant impact on further therapeutic treatment in patients with inflammatory bowel disease. PRACTICAL RECOMMENDATIONS: Especially in patients with inflammatory bowel disease, MR enterography and MR enteroclysis should be the methods of choice for the evaluation of small bowel pathologies because of radiation issues and the great diagnostic value they provide. A variety of MR sequences has enabled not only the detection but also the characterization of pathological changes of the small intestine, which are vital for further treatment of these patients.

[Functional MR imaging of the liver]. / Funktionelle MR-Tomographie der Leber.

The diagnostics of diffuse liver disease traditionally rely on liver biopsies and histopathological analysis of tissue specimens. However, a liver biopsy is invasive and carries some non-negligible risks, especially for patients with decreased liver function and those requiring repeated follow-up examinations. Over the last decades, magnetic resonance imaging (MRI) has developed into a valuable tool for the non-invasive characterization of focal liver lesions and diseases of the bile ducts. Recently, several MRI methods have been developed and clinically evaluated that also allow the diagnostics and staging of diffuse liver diseases, e.g. non-alcoholic fatty liver disease, hepatitis, hepatic fibrosis, liver cirrhosis, hemochromatosis and hemosiderosis. The sequelae of diffuse liver diseases, such as a decreased liver functional reserve or portal hypertension, can also be detected and quantified by modern MRI methods. This article provides the reader with the basic principles of functional MRI of the liver and discusses the importance in a clinical context.

Intraductal papillary mucinous neoplasm of the pancreas in an adult patient with cystic fibrosis after double-lung transplantation.

We report on an adult patient with cystic fibrosis after double-lung transplantation under triple immunosuppression with non-specific abdominal symptoms and a pancreatic cystic tumor, resulting in the diagnosis of an intraductal papillary mucinous neoplasm (IPMN) of the pancreas. Pancreatic cysts in adult patients with cystic fibrosis, especially after transplantation, merit close attention and thorough investigation.

[Modern imaging of liver metastases]. / Moderne Bildgebung der Lebermetastasen.

CLINICAL/METHODICAL ISSUE: The liver is the second most common location of metastases following the lymph nodes. The accurate characterization of focal liver lesions in oncology patients is especially important because of the high prevalence of benign liver lesions and the possibility of co-existing benign and malignant lesions. The exact interpretation of these lesions is crucial for therapeutic decisions and thus for the prognosis of the patient. STANDARD RADIOLOGICAL METHODS: It is essential to detect all focal liver lesions and to distinguish benign from malignant lesions, especially in the management of oncology patients. Numerous imaging modalities are available for these challenges in the daily routine. An extensive understanding of the advantages and limitations of the various imaging modalities and knowledge of the morphology and the typical and atypical appearances of the different metastases is important. METHODICAL INNOVATIONS: This review explains the radiological criteria for various metastases in different modalities. To evaluate the individual prognosis and risk assessment preoperatively, functional imaging is necessary. These personalized pretherapeutic diagnostics are discussed.

[Modern diagnostics of cystic liver lesions and hemangiomas]. / Moderne Diagnostik zystischer Leberläsionen und Hämangiome.

CLINICAL ISSUE: Cystic liver lesions incorporate a broad heterogeneous group of mostly benign but also malignant abnormalities. The radiological aim is the non-invasive diagnosis with the use of different imaging modalities to determine the type of lesion. STANDARD RADIOLOGICAL METHODS: The common generally asymptomatic incidental findings of cystic lesions on ultrasound, computed tomography (CT) and magnetic resonance imaging (MRI) must be classified on the basis of specific imaging features. Such a differentiation is essential because the clinical consequences and the appropriate therapy can vary depending on the underlying pathology. Due to the morphological overlap of many cystic lesions, conventional radiological methods are often insufficient. METHODICAL INNOVATIONS: The huge advances in cross-sectional imaging (multidetector CT, MRI with special sequences and different contrast agents and MR cholangiopancreatography) in combination with the clinical history usually enable a non-invasive diagnosis. Pathognomonic morphological and hemodynamic lesion features, as well as a knowledge of the pathomechanisms, help to differentiate this broad spectrum of entities. ACHIEVEMENTS: In this article the different entities of cystic liver lesions, together with the appropriate diagnostic method for detection and distinction and including their strengths and limitations, are demonstrated. PRACTICAL RECOMMENDATIONS: A well-founded knowledge about the development of various cystic liver lesions and the suitable choice of imaging method facilitate a non-invasive diagnosis.

[Focal nodular hyperplasia and hepatocellular adenoma]. / Fokale noduläre Hyperplasie und hepatozelluläres Adenom.

CLINICAL ISSUE: Focal nodular hyperplasia (FNH) and hepatocellular adenoma (HCA) are liver lesions of hepatocellular origin. The FNH is a commonly occurring hepatic lesion whereas HCA is very rare. Non-invasive differentiation between HCA subtypes and atypical FNH may pose a diagnostic challenge as both entities predominantly occur in middle-aged female patients. STANDARD RADIOLOGICAL METHODS: The conventional imaging modalities include ultrasound (US), computed tomography (CT) and magnetic resonance imaging (MRI). Distinguishing FNH from HCA is of great importance clinically as FNH is considered to be a benign lesion and needs no further management. In contrast HCA is considered to be a borderline tumor due to the risk of hemorrhage, growth and even malignant transformation and requires individualized management. METHODOLOGICAL INNOVATIONS: The above mentioned radiological procedures usually enable an accurate and certain diagnosis of a typical FNH to be achieved. In cases of atypical FNH, particularly in patients with a clinical history of malignancy, these imaging modalities are insufficient to establish a clear diagnosis. In this scenario, the use of modern hepatobiliary contrast-enhanced MRI will enable a differentiation between FNH and metastasis with a high sensitivity and specificity. Furthermore, it allows a differentiation of FNH from 90 % of adenoma subtypes. ACHIEVEMENTS: This article describes the histopathological and radiological features of these lesions and explains the advantages and limitations of various imaging modalities used for the diagnosis and differentiation of these entities. PRACTICAL RECOMMENDATIONS: The new classification of HCAs according to phenotype and genotype and their imaging features, as well as different enhancement patterns, are described. The correlation between HCA subtypes and their individual management are also discussed.

[Primary liver tumors : hepatocellular versus intrahepatic cholangiocellular carcinoma]. / Primäre Lebertumoren : Hepatozelluläres vs. intrahepatisches cholangiozelluläres Karzinom.

CLINICAL ISSUE: Hepatocellular carcinoma (HCC) and intrahepatic cholangiocellular carcinoma (ICC) are the most commonly occurring and important primary liver tumors. Originating from one pluripotent liver stem cell both tumor entities can occur in a cirrhotic liver and also in patients without cirrhosis. Several risk factors have been identified as causative for both carcinomas; therefore, tumor screening is advantageous, especially for high-risk patients who could be diagnosed in an early stage to allow curative treatment. Surgical resection, interventional procedures and transplantation are available as curative treatment options when diagnosed in time. STANDARD RADIOLOGICAL METHODS: Common characteristic features and morphology in cross-sectional imaging by ultrasound (US), multidetector computed tomography (CT) and magnetic resonance imaging (MRI) as well as screening aspects are presented and discussed. METHODICAL INNOVATIONS: Recent findings show a better understanding of the carcinogenesis model of both liver tumors originating from one pluripotent liver stem cell. Further developments of modern cross-sectional imaging modalities, especially MRI in combination with diffusion-weighted imaging and intravenous administration of hepatocyte-specific contrast agents enable early detection, exact differentiation, staging and treatment evaluation of HCC and ICC ACHIEVEMENTS: In this article we discuss modern, multiparametric imaging modalities, which allow a complete and reliable diagnosis of the majority of these tumor entities. PRACTICAL RECOMMENDATIONS: Contrast-enhanced MRI, using hepatocyte-specific contrast agents, is currently the most accurate procedure for the noninvasive diagnosis and treatment evaluation of HCC and ICC.

Randomized multicentre trial of gadoxetic acid-enhanced MRI versus conventional MRI or CT in the staging of colorectal cancer liver metastases.

BACKGROUND: This multicentre international randomized trial compared the impact of gadoxetic acid-enhanced magnetic resonance imaging (MRI), MRI with extracellular contrast medium (ECCM-MRI) and contrast-enhanced computed tomography (CE-CT) as a first-line imaging method in patients with suspected colorectal cancer liver metastases (CRCLM). METHODS: Between October 2008 and September 2010, patients with suspected CRCLM were randomized to one of the three imaging modalities. The primary endpoint was the proportion of patients for whom further imaging after initial imaging was required for a confident diagnosis. Secondary variables included confidence in the therapeutic decision, intraoperative deviations from the initial imaging-based surgical plan as a result of additional operative findings, and diagnostic efficacy of the imaging modalities versus intraoperative and pathological extent of the disease. RESULTS: A total of 360 patients were enrolled. Efficacy was analysed in 342 patients (118, 112 and 112 with gadoxetic acid-enhanced MRI, ECCM-MRI and CE-CT respectively as the initial imaging procedure). Further imaging was required in 0 of 118, 19 (17.0 per cent) of 112 and 44 (39.3 per cent) of 112 patients respectively (P < 0.001). Diagnostic confidence was high or very high in 98.3 per cent of patients for gadoxetic acid-enhanced MRI, 85.7 per cent for ECCM-MRI and 65.2 per cent for CE-CT. Surgical plans were changed during surgery in 28, 32 and 47 per cent of patients in the respective groups. CONCLUSION: The diagnostic performance of gadoxetic acid-enhanced MRI was better than that of CE-CT and ECCM-MRI as the initial imaging modality. No further imaging was needed in the gadoxetic acid-enhanced MRI group and comparison of diagnostic efficacy parameters demonstrated the diagnostic superiority of gadoxetic acid-enhanced MRI. REGISTRATION NUMBER: NCT00764621(http://clinicaltrials.gov); EudraCT number: 2008-000583-16 (https://eudract.ema.europa.eu/).

Diagnostic workup of primary sclerosing cholangitis: the benefit of adding gadoxetic acid-enhanced T1-weighted magnetic resonance cholangiography to conventional T2-weighted magnetic resonance cholangiography.

AIM: To evaluate the value of gadoxetic acid-enhanced T1-weighted (T1W) magnetic resonance cholangiography (MRC) versus conventional T2-weighted (T2W) MRC compared to endoscopic retrograde cholangiopancreatography (ERCP) in patients with primary sclerosing cholangitis (PSC). MATERIALS AND METHODS: Based on T1W MRC, PSC patients were classified into a regular (RG) and a delayed (DG) excreting group, with an absence of gadoxetic acid in the common bile duct at 20 min. Beading, pruning, and gradation of central bile duct stenosis, evaluated by T1W and T2W MRC, were compared to ERCP. Liver parenchymal enhancement was measured in both study groups and compared to a reference group (n = 20) without a history of liver disease. Two readers performed all measurements. RESULTS: Based on beading and pruning of the peripheral bile ducts, sensitivities, specificities, and accuracies for reader 1 were 0.17/0.43, 0/0.17, and 0.15/0.31 for T1W MRC, and 0.83/0.86, 1/0.83, and 0.85/0.85 for T2W MRC (p = 0.004). For reader 2 sensitivities, specificities, and accuracies were 0.25/0.57, 0/0.33, and 0.23/0.46 for T1W MRC, and 0.92/1, 1/0.83, and 0.92/0.92 for T2W MRC (p = 0.012). Compared to ERCP, central bile duct stenoses were significantly overestimated (p < 0.001) by T2W MRC. A significantly lower parenchymal enhancement was found in the DG (n = 7) compared to the RG (n = 13), and compared to the reference group (p < 0.001). CONCLUSION: The combined performance of T2W and T1W MRC may provide a comprehensive imaging workup of PSC, including morphological and functional information resulting in optimal management.

Imaging techniques for assessment of inflammatory bowel disease: joint ECCO and ESGAR evidence-based consensus guidelines.

The management of patients with IBD requires evaluation with objective tools, both at the time of diagnosis and throughout the course of the disease, to determine the location, extension, activity and severity of inflammatory lesions, as well as, the potential existence of complications. Whereas endoscopy is a well-established and uniformly performed diagnostic examination, the implementation of radiologic techniques for assessment of IBD is still heterogeneous; variations in technical aspects and the degrees of experience and preferences exist across countries in Europe. ECCO and ESGAR scientific societies jointly elaborated a consensus to establish standards for imaging in IBD using magnetic resonance imaging, computed tomography, ultrasonography, and including also other radiologic procedures such as conventional radiology or nuclear medicine examinations for different clinical situations that include general principles, upper GI tract, colon and rectum, perineum, liver and biliary tract, emergency situation, and the postoperative setting. The statements and general recommendations of this consensus are based on the highest level of evidence available, but significant gaps remain in certain areas such as the comparison of diagnostic accuracy between different techniques, the value for therapeutic monitoring, and the prognostic implications of particular findings.

Preoperative evaluation of colorectal liver metastases: comparison between gadoxetic acid-enhanced 3.0-T MRI and contrast-enhanced MDCT with histopathological correlation.

OBJECTIVES: The aim of this prospective study was to compare the diagnostic performance of 64-row MDCT and gadoxetic-acid-enhanced MRI at 3.0 T in patients with colorectal liver metastases in correlation with histopathological findings. METHODS: Lesions detected at MDCT and MRI were interpreted by three blinded readers and compared with histopathological workup as the term of reference. Two subgroups of lesions were additionally evaluated: (1) metastases smaller than 10 mm and (2) lesions in patients with and without steatosis of the liver, assessed histopathologically. RESULTS: Surgery and histopathological workup revealed 81 colorectal liver metastases in 35 patients and diffuse metastatic involvement in 3 patients. In a lesion-by-lesion analysis, significant sensitivity differences could only be found for reader 1 (P = 0.035) and reader 3 (P = 0.003). For segment-based evaluation, MRI was more sensitive only for reader 3 (P = 0.012). The number of false-positive results ranged from 3 to 12 for MDCT and 8 to 11 for MRI evaluation. In the group of small lesions, the sensitivity differed significantly between both methods (P = 0.003). In patients with hepatic steatosis, MRI showed a trend toward better performance than MDCT, but without statistical performance. CONCLUSIONS: The 3.0-T MRI with liver-specific contrast agents is the preferred investigation in the preoperative setting, especially for the assessment of small colorectal liver metastases. KEY POINTS: • Potential surgical treatment requires accurate radiological assessment of colorectal liver metastases • Magnetic resonance imaging with gadoxetic acid is the preferred imaging investigation. • MRI is better than multidetector CT for detecting small liver metastases.