Differentiating IgG4-related sclerosing cholangiopathy from cholangiocarcinoma using CT and MRI: experience from a tertiary referring center

Purpose To compare the cross-sectional imaging findings of immunoglobulin G4-related sclerosing cholangiopathy (IgG4-SC) and cholangiocarcinoma (CCA). Methods Retrospective search of radiology and pathology databases identified 24 patients with IgG4-SC and over 500 patients with CCA from January 2009 to December 2016. Patients with no pre-treatment imaging studies available on PACS, non-contrasted imaging only, presence of mass lesions, metastatic disease or biliary stents were excluded. 17 patients with IgG4-SC and a selected group of 20 (age and gender matched) patients with CCA were obtained. Images were blinded and independently reviewed by two radiologists. Differences in proportions and means between groups were analyzed using Fishers and Mann–Whitney tests, respectively. Results Both readers identified a statistically significant difference in the presence of abrupt common bile duct narrowing between IgG4-SC and CCA (6.7% vs. 68.4%, p < 0.001; 33.3% vs. 75%, p = 0.019). No difference was seen in biliary wall thickening, wall enhancement, extrahepatic exclusive location of disease, or pancreatic duct dilation. Inter-observer variability was κ = 0.52. Total bilirubin and CA 19-9 were unable to differentiate between IgG4-SC and CCA. Serum IgG4 was positive in two of six IgG4-SC patients who were tested. Conclusion IgG4-SC and CCA share many clinical and imaging findings on CT and MRI. Abrupt bile duct cut sign strongly favors CCA. In the absence of this finding, IgG4-SC should be considered in the differential diagnosis in all cases of suspected extrahepatic CCA

Tumors in von Hippel–Lindau Syndrome: From Head to Toe—Comprehensive State-of-the-Art Review

Von Hippel–Lindau syndrome (VHL) is an autosomal-dominant hereditary tumor disease that arises owing to germline mutations in the VHL gene, located on the short arm of chromosome 3. Patients with VHL may develop multiple benign and malignant tumors involving various organ systems, including retinal hemangioblastomas (HBs), central nervous system (CNS) HBs, endolymphatic sac tumors, pancreatic neuroendocrine tumors, pancreatic cystadenomas, pancreatic cysts, clear cell renal cell carcinomas, renal cysts, pheochromocytomas, paragangliomas, and epididymal and broad ligament cystadenomas. The VHL/hypoxia-inducible factor pathway is believed to play a key role in the pathogenesis of VHL-related tumors. The diagnosis of VHL can be made clinically when the characteristic clinical history and findings have manifested, such as the presence of two or more CNS HBs. Genetic testing for heterozygous germline VHL mutation may also be used to confirm the diagnosis of VHL. Imaging plays an important role in the diagnosis and surveillance of patients with VHL. Familiarity with the clinical and imaging manifestations of the various VHL-related tumors is important for early detection and guiding appropriate management. The purpose of this article is to discuss the molecular cytogenetics and clinical manifestations of VHL, review the characteristic multimodality imaging features of the various VHL-related tumors affecting multiple organ systems, and discuss the latest advances in management of VHL, including current recommendations for surveillance and screening

Computerized Tomography of the Acute Left Upper Quadrant Pain

The purpose of this study was to evaluate the clinical utility of computerized tomography (CT) of the abdomen in the emergent setting of left upper quadrant pain. One hundred patients (average age: 45, range: 19–93 years, female: 57 %, male: 43 %) who presented to the emergency department (ED) and underwent CT scanning of abdomen with the given indication of left upper quadrant pain were included in this study. The results from CT examinations were compared to final diagnoses determined by either ED physician or clinician on a follow-up visit. Sensitivity of CT was 69 % (95 %CI: 52–83 %) for 39 patients who eventually were diagnosed with an acute abdominal abnormality. Twenty-seven patients had an acute abnormal finding on abdominal CT that represented the cause of the patient’s pain (positive predictive value of 100 %, 95 %CI: 87–100 %). Of the remaining 73 patients with negative CT report, 12 were diagnosed clinically (either in the ED or on follow-up visit to specialist) with a pathology that was undetectable on the CT imaging (negative predictive value of 83 %, 95 %CI: 73–91 %). None of the remaining 61 patients with negative CT were found to have pathology by clinical evaluation (specificity of 100 %, 95 %CI: 94–100 %). CT is a useful examination for patients with acute left upper quadrant pain in the emergency department setting with moderate sensitivity and excellent specificity

T1 Mapping for Diagnosis of Mild Chronic Pancreatitis

Purpose To determine if the T1 relaxation time of the pancreas can detect parenchymal changes in mild chronic pancreatitis (CP). Materials and Methods This Institutional Review Board (IRB)-approved, Health Insurance Portability and Accountability Act (HIPAA)-compliant retrospective study analyzed 98 patients with suspected mild CP. Patients were grouped as normal (n = 53) or mild CP (n = 45) based on history, presenting symptomatology, and concordant findings on both the secretin-enhanced magnetic resonance cholangiopancreatography (S-MRCP) and endoscopic retrograde cholangiopancreatography (ERCP). T1 maps were obtained in all patients using the same 3D gradient echo technique on the same 3T scanner. T1 relaxation times, fat signal fraction (FSF), and anterior–posterior (AP) diameter were correlated with the clinical diagnosis of CP. Results There was a significant difference (P < 0.0001) in the T1 relaxation times between the control (mean = 797 msec, 95% confidence interval [CI]: 730, 865) and mild CP group (mean = 1099 msec, 95% CI: 1032, 1166). A T1 relaxation time threshold value of 900 msec was 80% sensitive (95% CI: 65, 90) and 69% specific (95% CI: 56, 82) for the diagnosis of mild CP (area under the curve [AUC]: 0.81). Multiple regression analysis showed that T1 relaxation time was the only statistically significant variable correlating with the diagnosis of CP (P < 0.0001). T1 relaxation times showed a weak positive correlation with the pancreatic FSF (ρ = 0.33, P = 0.01) in the control group, but not in the mild CP group. Conclusion The T1 relaxation time of the pancreatic parenchyma was significantly increased in patients with mild CP. Therefore, T1 mapping might be used as a practical quantitative imaging technique for the evaluation of suspected mild CP

Detection of Exocrine Dysfunction by MRI in Patients with Early Chronic Pancreatitis

Purpose To determine if T1-weighted MR signal of the pancreas can be used to detect early CP. Methods A retrospective analysis was performed on 51 suspected CP patients, who had both secretin-enhanced magnetic resonance cholangiopancreatography (S-MRCP) and an intraductal secretin stimulation test (IDST). There were 29 patients in normal and 22 patients in the low bicarbonate group. Bicarbonate level, total pancreatic juice volume, and excretory flow rate were recorded during IDST. Signal intensity ratio of pancreas (SIR), fat signal fraction, pancreatograms findings, and grade of duodenal filling were recorded on S-MRCP by two blinded radiologists. Results There was a significant difference in the signal intensity ratio of the pancreas to spleen (SIRp/s) between the normal and low bicarbonate groups (p < 0.0001). A significant positive correlation was found between pancreatic fluid bicarbonate level and SIRp/s (p < 0.0001). SIRp/s of 1.2 yielded sensitivity of 77% and specificity of 83% for detection of pancreatic exocrine dysfunction (AUC: 0.89). Conclusion T1-weighted MR signal of the pancreas has a high sensitivity and specificity for the detection of parenchymal abnormalities related to exocrine dysfunction and can therefore be helpful in evaluation of suspected early CP

Comparison of hepatic MDCT, MRI, and DSA to explant pathology for the detection and treatment planning of hepatocellular carcinoma

BACKGROUND/AIMS: The diagnosis and treatment plan for hepatocellular carcinoma (HCC) can be made from radiologic imaging. However, lesion detection may vary depending on the imaging modality. This study aims to evaluate the sensitivities of hepatic multidetector computed tomography (MDCT), magnetic resonance imaging (MRI), and digital subtraction angiography (DSA) in the detection of HCC and the consequent management impact on potential liver transplant patients. METHODS: One hundred and sixteen HCC lesions were analyzed in 41 patients who received an orthotopic liver transplant (OLT). All of the patients underwent pretransplantation hepatic DSA, MDCT, and/or MRI. The imaging results were independently reviewed retrospectively in a blinded fashion by two interventional and two abdominal radiologists. The liver explant pathology was used as the gold standard for assessing each imaging modality. RESULTS: The sensitivity for overall HCC detection was higher for cross-sectional imaging using MRI (51.5%, 95% confidence interval [CI]=36.2-58.4%) and MDCT (49.8%, 95% CI=43.7-55.9%) than for DSA (41.7%, 95% CI=36.2-47.3%) (P=0.05). The difference in false-positive rate was not statistically significant between MRI (22%), MDCT (29%), and DSA (29%) (P=0.67). The sensitivity was significantly higher for detecting right lobe lesions than left lobe lesions for all modalities (MRI: 56.1% vs. 43.1%, MDCT: 55.0% vs. 42.0%, and DSA: 46.9% vs. 33.9%; all P<0.01). The sensitivities of the three imaging modalities were also higher for lesions ≥2 cm vs. <2 cm (MRI: 73.4% vs. 32.7%, MDCT: 66.9% vs. 33.8%, and DSA: 62.2% vs. 24.1%; all P<0.01). The interobserver correlation was rated as very good to excellent. CONCLUSION: The sensitivity for detecting HCC is higher for MRI and MDCT than for DSA, and so cross-sectional imaging modalities should be used to evaluate OLT candidacy

Intestinal and multivisceral transplantation

Intestinal transplantation is carried out in only a handful of centers in the world. However, patients with such transplantation may be seen at almost any institution and radiologists should be familiar with the expected normal anatomy and complications of intestinal transplantation and its variants. In this paper, we discuss the anatomy and complications following intestinal and multivisceral transplantations. We review the literature and present the findings in a cohort of 46 patients who underwent these procedures at our institution. It is important not to misinterpret the normal adaptation of the transplanted intestine for disease. Some of the complications, including infections, vascular complications, and recurrent tumor, are conclusively diagnosed on imaging studies. Imaging studies may raise the diagnosis of complications such as rejection, graft-versus-host disease, posttransplant lymphoproliferative disease, and sclerosing peritonitis