Computed Tomographic Features of Primary Small Cell Neuroendocrine Tumors of the Gallbladder.

PURPOSE: This study aimed to report the computed tomography (CT) imaging features of primary small cell neuroendocrine tumors of the gallbladder (PSCNETGs). MATERIALS AND METHODS: The CT examinations of 9 patients (5 women, 4 men; median age, 57 years) with histopathologically proven PSCNETG were reviewed. Computed tomographic images were analyzed with respect to morphologic features of primary tumors and accompanying lymph nodes. RESULTS: All PSCNETGs were visible on CT, with a median largest axial diameter of 60 mm (Q1, 30 mm; Q3 mm, 82; range, 25-86 mm). These tumors presented with extraluminal growth (8/9; 89%), heterogeneous enhancement (8/9; 89%), gallbladder replacement greater than 50% (5/9; 56%), hepatic metastases (5/9; 56%), and direct liver involvement by tumor (4/9; 44%). Enlarged lymph nodes were present in all patients (9/9; 100%) with a median largest axial diameter of 39 mm (Q1, 23 mm; Q3, 48 mm; range, 12-62 mm). Vessel encasement by lymph nodes was present in 6 (67%) of 9 patients. CONCLUSION: Primary small cell neuroendocrine tumors of the gallbladder predominantly presents as a large, heterogeneous gallbladder mass with extraluminal growth in association with large metastatic lymph nodes and intrahepatic metastases.

Budd-Chiari syndrome: an update on imaging features.

Budd-Chiari syndrome (BCS) is a rare cause of portal hypertension and liver failure. This condition is characterized by an impaired hepatic venous drainage. The diagnosis of BCS is based on imaging, which helps initiate treatment. Imaging findings can be categorized into direct and indirect signs. Direct signs are the hallmarks of BCS and consist of visualization of obstructive lesions of the hepatic veins or the upper portion of the inferior vena cava. Indirect signs, which are secondary to venous obstruction, correspond to intra- and extrahepatic collateral circulation, perfusion abnormalities, dysmorphy and signs of portal hypertension.

MR imaging features of liver involvement by Wilson disease in adult patients.

OBJECTIVE: To describe the magnetic resonance imaging (MRI) presentation of liver involvement in adult patients with Wilson disease (WD) and determine the most indicative appearance of this condition on MRI using a retrospective case-control study. MATERIALS AND METHODS: MRI examinations of 23 adult patients with WD (14 men, 9 women; mean age = 40.4 years) were analyzed by two blinded observers and compared to those obtained in 23 patients with chronic viral hepatitis (14 men, 9 women, mean age = 40.4 years) who were matched for age, gender and severity of chronic liver disease. Images were qualitatively and quantitatively analyzed with respect to imaging presentation. Comparisons were performed using univariate analysis. RESULTS: Honeycomb pattern of hepatic parenchyma was the most discriminating independent variable for the diagnosis of WD (odds ratio, 17.082; 95 % CI 2.092-139.497) (P = 0.0081) but had a sensitivity of 43 % (10/23; 95 % CI 23-66 %). Regular liver contours was the other variable that strongly correlated with the presence of liver involvement by WD (odds ratio, 11.939; 95 % CI 1.503-94.836) (P = 0.0190). CONCLUSION: The honeycomb pattern is the most discriminating independent variable for the diagnosis of liver involvement by WD but has limited sensitivity. Familiarity with this finding may clarify the cause of diffuse hepatic parenchymal abnormalities in patients with unknown WD.

MR imaging features of focal liver lesions in Wilson disease.

Hepatic involvement in Wilson disease (WD) manifests as a diffuse chronic disease in the majority of patients. However, in a subset of patients focal liver lesions may develop, presenting with a wide range of imaging features. The majority of focal liver lesions in patients with WD are benign nodules, but there are reports that have described malignant liver tumors or dysplastic nodules in these patients. Because of the possibility of malignant transformation of liver nodules, major concerns have been raised with respect to the management and follow-up of patients with WD in whom focal liver lesions have been identified. The assessment of liver involvement in patients with WD is generally performed with ultrasonography. However, ultrasonography conveys limited specificity so that magnetic resonance (MR) imaging is often performed to improve lesion characterization. This review was performed to illustrate the spectrum of MR imaging features of focal liver lesions that develop in patients with WD. It is assumed that familiarity with the MR imaging presentation of focal liver lesions in WD may help clarify the actual nature of hepatic nodules in patients with this condition.

Aetiological factors of Budd-Chiari syndrome in Algeria.

AIM: To study the clinical presentation of Budd-Chiari syndrome (BCS) and identify the aetiologies of this disease in Algeria. METHODS: Patients with BCS, hospitalised in our unit from January 2004 until June 2010 were included and the aetiological factors were assessed. Patients presenting a BCS in the setting of advanced-stage cirrhosis or a liver transplantation were excluded from the study. The diagnosis was established when an obstruction of hepatic venous outflow (thrombosis, stenosis or compression) was demonstrated. We diagnosed myeloproliferative disease (MPD) by bone marrow biopsy and V617F JAK2 mutation. Anti-phospholipid syndrome (APLS) was detected by the presence of anticardiolipin antibodies, anti-ß2 glycoprotein antibodies and Lupus anticoagulant. We also detected paroxysmal nocturnal haemoglobinuria (PNH) by flow cytometry. Celiac disease and Behçet disease were systematically investigated in our patients. Hereditary anticoagulant protein deficiencies were also assessed. We tested our patients for the G20210A mutation at Beaujon Hospital. Imaging procedures were performed to determine a local cause of BCS, such as a hydatid cyst or a liver tumour. RESULTS: One hundred and fifteen patients were included. Mean follow up: 32.12 mo. Mean age: 34.41 years, M/F = 0.64. Chronic presentation was frequent: 63.5%. The revealing symptoms for the BCS were ascites (74.8%) and abdominal pain (42.6%). The most common site of thrombosis was the hepatic veins (72.2%). Involvement of the inferior vena cava alone was observed in 3 patients. According to the radiological investigations, BCS was primary in 94.7% of the cases (n = 109) and secondary in 5.2% (n = 6). An aetiology was identified in 77.4% of the patients (n = 89); it was multifactorial in 27% (n = 31). The predominant aetiology of BCS in our patients was a myeloproliferative disease, observed in 34.6% of cases. APLS was found in 21.7% and celiac disease in 11.4%. Other acquired conditions were: PNH (n = 4), systemic disease (n = 6) and inflammatory bowel disease (n = 5). Anticoagulant protein deficiency was diagnosed in 28% of the patients (n = 18), dominated by protein C deficiency (n = 13). Secondary BCS was caused by a compressing hydatic cyst (n = 5) and hepatocellular carcinoma (n = 1). CONCLUSION: The main aetiologic factor of BCS in Algeria is MPD. The frequency of celiac disease justifies its consideration when BCS is diagnosed in our region.

Budd-Chiari syndrome: a prospective analysis of hepatic vein obstruction on ultrasonography, multidetector-row computed tomography and MR imaging.

PURPOSE: The goal of this study was to prospectively describe the imaging presentation of hepatic vein (HV) obstruction in patients with Budd-Chiari syndrome (BCS) on duplex and color Doppler ultrasonography (DCD-US), multidetector-row computed tomography (MDCT) and magnetic resonance imaging (MRI). MATERIALS AND METHODS: A total of 176 patients with primary BCS (mean age, 33 years; 101 women) were prospectively included. BCS diagnosis was made by direct visualization of HV and/or upper portion of the inferior vena cava (IVC) obstruction on DCD-US and/or MDCT and/or MRI. Location (right, middle, and left HV), type (thrombus, stenosis, or both), and age (recent vs. long-standing) of HV obstruction were described on each imaging examination. RESULTS: HV obstruction was a constant (100%) finding and associated with IVC abnormalities in 51/176 (28.98%) patients. Obstruction of the three HVs was present in 158/176 (89.77%) patients. The prevalences of right, middle, and left HV thrombus were 151/169 (89.35%), 146/169 (86.39%), and 111/169 (65.68%), respectively. Long-standing HV thrombus was observed in more than 92% of patients on the three imaging methods. Agreement between DCD-US, MDCT, and MRI was perfect in the identification of long-standing HV thrombus (κ = 0.9); this agreement was slight to moderate in revealing the type of HV abnormality (i.e., fibrotic cord and non-visible HV). CONCLUSION: Our results indicate that BCS is a chronic and insidious disease, more often discovered at an advanced stage. These results should warrant further evaluation of screening strategies in patients with risk factors for BCS to identify the disease at an early stage.

Solid-pseudopapillary tumor of the pancreas: MR imaging findings in 21 patients.

PURPOSE: Solid-pseudopapillary tumor (SPT) of the pancreas is a rare, low-grade malignancy, which mostly occurs in adolescent and young adult females. The goal of this study was to retrospectively analyze the magnetic resonance (MR) imaging presentation of SPT of the pancreas. METHODS: We retrospectively reviewed the preoperative MR imaging examinations and the medical, surgical and histopathological records of 21 patients who underwent surgery for SPT of the pancreas. MR imaging included T1-weighted, T2-weighted, and gadolinium chelate-enhanced MR imaging. In addition, 10 patients had diffusion-weighted (DW) MR imaging. MR examinations were retrospectively reviewed for location, size, morphological features and signal intensity of the tumors. RESULTS: Nineteen women and 2 men (median age, 23 years; range, 14-59) were included. Seven patients (7/21; 33%) presented with abdominal symptoms. The median largest tumor diameter was 53mm (range, 32-141 mm). SPTs were located in the pancreatic head, body, and tail in 9 (9/21; 43%), 5 (5/21; 24%) and 7 (7/21, 33%) patients, respectively. All patients (21/21; 100%) had a single SPT. SPTs were more frequently oval (12/21; 57%), predominantly solid (12/21; 57%), fully encapsulated (16/21; 76%), larger than 30 mm (21/21; 100%), hypointense on T1-weighted MR images (21/21, 100%), hyperintense on T2-weighted MR images (21/21; 100%) and with an enhancing capsule after gadolinium-chelate administration (21/21; 100%). CONCLUSIONS: There is trend of appearance for SPT of the pancreas on MR imaging but that variations may be observed in a number of cases. SPT uniformly presents as a single, well-demarcated and encapsulated pancreatic mass.