Gorham-Stout Syndrome with Chylothorax in a Six-Year-Old Boy

WOS: 000291250000012PubMed ID: 21188554Gorham-Stout syndrome, also called "disappearing bone disease, diffuse cystic angiomatosis of bone, disseminated lymphangiomatosis, Gorham's vanishing bone disease, phantom bone disease or idiopathic massive osteolysis, is a rare disease of unknown etiology and pathogenesis. It is characterized by rapidly progressive localized massive osteolysis associated with proliferation of vascular structures of benign origin in which the absence of new bone formation is representative. When it is complicated by chylothorax, the prognosis is poor. The authors report a 6-year-old boy with Gorham-Stout syndrome who presented with pleural effusion showing features of chylothorax, who responsed poorly to currently available therapeutic modalities

Intravoxel Incoherent Motion Parameters for Assessing the Efficiency of Locoregional Bridging Treatments before Liver Transplantation

1st International Transplant Network Congress -- OCT 17-21, 2018 -- Antalya, TURKEYWOS: 000487349900059PubMed: 31474296Objective. To evaluate the diagnostic accuracy of Intravoxel Incoherent Motion (IVIM) parameters for assessment of tumor response after locoregional treatment (LRT) of hepatocellular carcinoma (HCC). Methods. Fifteen patients with HCC who had undergone LRTs (11 transarterial radioembolization, 4 transarterial chemoembolization) were included. in addition to routine upper abdominal magnetic resonance imaging sequences, IVIM with 16 different b values and conventional diffusion weighted imaging with 3 different b factors were obtained immediately before and 8 weeks after LRTs. Magnetic resonance imaging response was evaluated according to modified Response Evaluation Criteria in Solid Tumors (mRECIST) and HCCs were categorized into 2 subgroups, responders and nonresponders. Quantitatively, the number of diffusion-changes were calculated with apparent diffusion coefficient (ADC) and IVIM parameters, including mean D (true diffusion coefficient), pseudo-diffusion coefficient associated with blood flow, and f (perfusion fraction) values. Subsequently, the pre- and post-treatment parameters were compared using the Mann-Whitney U test. Results. Considering all HCCs, a significant decrease was observed according to mRECIST criteria (-38.43 +/- 16.49). the ADC and D values after LRTs were significantly higher than those of the preceding ones. the f values after LRTs were significantly lower than those of pre-treatment. in the responders group, ADC and D values were significantly increased and f values were significantly decreased after LRTs. No difference of statistical significance was achieved in the nonresponders group. Conclusions. ADC values and IVIM parameters appear to reflect the response of LRTs as effectively as those of mRECIST. This promises new horizons in the management of pretransplant patients, especially in renal insufficiency clinical settings, owing to the elimination of contrast media administration

Focal xanthogranulomatous pyelonephritis mimicking adrenal mass: Case report

Ksantogranülomatöz pyelonefrit (KGP) etiyolojisi tam olarak bilinmeyen ve nadir görülen kronik renal inflamasyondur. Tipik olarak taşa bağlı obstrüksiyon zemininde gelişir. Ultrasonografi (USG) bulgularının nonspesifik olması sebebiyle bilgisayarlı tomografi (BT) ve Manyetik Rezonans Görüntüleme (MRG) hastalığın tanısı ve preoperatif planlamada ekstrarenal uzanımının gösterilmesinde seçilebilecek diagnostik modalitelerdir. Ancak fokal tutulum gösteren KGP'i böbrek tümörlerinden ayırt etmek zor olabilir ve bazen tanı yalnızca nefrektomi sonrası konulabilir. Bu yazımızda adrenal kitleyi taklit eden ve histopatolojik olarak ksantogranulomatöz pyelonefrit tanısı alan olgu sunulmuştur.Xanthogranulomatous pyelonephritis is a rare form of chronic renal infection which is unknown etiology exactly. Xanthogranulomatous pyelonephritis is typically associated with an obstructing calculus. Because of the nonspecific findings of ultrasonography, computed tomography and magnetic resonance imaging can be selected diagnostic modalities to show the extrarenal extension of the disease. However, it may be difficult to distinguish focal involvement of xanthogranulomatous pyelonephritis from renal tumors and sometimes diagnosis can only be made after nephrectomy. In this article we present a case of xanthogranulomatous pyelonephritis mimicking adrenal mass and histopathologically diagnosed

The Performance of Radiographic Criteria for Bone Malignancy When Applied to Computed Tomography and Magnetic Resonance Imaging

WOS: 000429481400015PubMed ID: 30479294Background: The conventional radiologic features that differentiate benign from malignant bone lesions were originally described using radiography (x-ray [XR]). When evaluating sectional imaging studies such as magnetic resonance imaging (MRI) and computed tomography (CT), one may apply these principles to identify malignant bone lesions. The aim of this study was to evaluate the performances of these radiographic features for detecting malignity when applied to CT and MRI. Materials and Methods: This retrospective study was approved by our institutional ethical board. Thirty-nine patients with histopathologic proof of a high-grade bone malignancy and preoperative imaging data obtained with a minimum of two different modalities were included in the study. Four radiologists reviewed the images and scored the lesions for distinctness of margins, presence and type of periosteal reaction, matrix mineralization, and presence of soft tissue mass. The average score for each modality was then tested for accuracy with regard to the histopathology. Results: When lesion margins were considered, XR was the best modality to detect a high-grade malignancy. MRI, especially post-contrast T1-weighted sequence, was the least helpful in this regard. There was no significant difference between CT and XR and between CT and MRI. When the periosteal reaction was considered, XR was the best modality to detect the malignant type of periosteal reaction. In this regard, MRI and CT were misleading; either by not detecting or undergrading periosteal reaction. MRI was the best modality to detect soft tissue mass. Conclusion: Conventional imaging criteria for bone malignancy can be misleading when applied to MRI or CT. When cross-sectional imaging features contradict those from XR, the latter should be the guide for clinical management

Malignant versus benign mediastinal lesions: quantitative assessment with diffusion weighted MR imaging," Eur Radiol

Abstract Objectives We aimed to evaluate the performance of diffusion-weighted magnetic resonance imaging in differentiating malignant from benign mediastinal lesions. Methods Fifty-three mediastinal lesions were examined with T1-and T2-weighted (W) conventional images. Then, two diffusion-weighted images were obtained with b=0 and 1000 s/mm² values and apparent diffusion coefficients (ADC) were calculated. The statistical significance of differences between measurements was tested using the Student-t test. Results The mean ADC of malignant lesions was significantly lower than that of the benign masses (p<0.001). The cut-off value of ≤1.39×10 −3 mm²/s indicated a malignant lesion with a sensitivity of 95% and specificity of 87%. Conclusion Diffusion-weighted imaging may be helpful in differentiating benign from malignant mediastinal masses

Liver Apparent Diffusion Coefficient Changes during Telaprevir-Based Therapy for Chronic Hepatitis C

Background: Diffusion-weighted imaging (DWI) has become an established diagnostic modality for the evaluation of liver parenchymal changes in diseases such as diffuse liver fibrosis. Aims: To evaluate the parenchymal apparent diffusion coefficient value (ADC) changes using diffusion-weighted imaging (DWI) during telaprevir-based triple therapy. Study Design: Diagnostic accuracy study. Methods: Seventeen patients with chronic hepatitis C virus (HCV) virus and twenty-five normal volunteers were included. All of the patients took 12-weeks of telaprevir-based triple therapy followed by 12-weeks of PEGylated interferon and ribavirin therapy. They were examined before treatment (BT), as well as 12-weeks (W12) and 24-weeks (W24) after treatment by 3 Tesla magnetic resonance imaging (MRI). DWI was obtained using a breath-hold single-shot echo-planar spin echo sequence. Histopathologically, liver fibrosis was classified in accordance with the modified Knodell score described by Ishak. Quantitatively, liver ADCs were compared between patients and normal volunteers to detect the contribution of DWI in the detection of fibrosis. In addition, liver ADCs were compared during the therapy to analyze the effect of antiviral medication on liver parenchyma. Results: The liver ADC values of fibrotic liver parenchyma were significantly lower than those of the healthy liver parenchyma (p<0.001). However, we were not able to reach a sufficiently discriminative threshold value. The ADC values showed a declining trend with increasing fibrotic stage. No statistically significant correlation (p=0.204) was observed. Compared with those before treatment, the liver ADC values after telaprevir-based triple therapy were significantly decreased at W12. A significant increase in the liver ADC values was also observed after the cessation of telaprevir therapy at W24 with a return to initial values. Conclusion: Liver ADC values appear to indicate the present but not the stage of liver fibrosis. DWI may be a helpful research tool for the assessment of antiviral drug effects

Value of intravoxel incoherent motion for hepatocellular carcinoma grading

Background: To evaluate the diagnostic accuracy of intravoxel incoherent motion (IVIM) parameters in estimation of hepatocellular carcinoma (HCC) grading. Materials and methods: Twenty-nine patients with histopathologically diagnosed as 42 HCC at explant were included in this retrospective study. All patients were examined by 1.5T magnetic resonance imaging with the use of 4-channel phased array body coil. In addition to routine pre- and postcontrast sequences, IVIM (16 different b factors varying from 0 to 1300 s/mm2) and conventional diffusion-weighted imaging (3 different b factors of 50, 400, 800 s/mm2) were obtained with single-shot echo planar spin echo sequence. Apparent diffusion coefficient (ADC) and IVIM parameters including mean D (true diffusion coefficient), D* (pseudo-diffusion coefficient associated with blood flow), and f (perfusion fraction) values were calculated. Histopathologically, HCC was classified as low (grade 1, 2) and high (grade 3, 4) grade in accordance with the Edmondson-Steiner score. Quantitatively, ADC, D, D*, and f values were compared between the low- and high-grade groups by Student t test. The relationship between the parameters and histologic grade was analyzed using the Spearman's correlation test. To evaluate the diagnostic performance of the parameters, receiver operating characteristic analysis was performed. Results: High-grade HCCs had significantly lower ADC and D values than low grade groups (P = .005 and P = .026, retrospectively); ADC and D values were inversely correlated with tumor grade (r = –0.519, P = .011, r = –0.510, P = .026, respectively). High-grade HCCs had significantly higher f values when compared with the low-grade group (P = .005). The f values were positively correlated with tumor grade (r = 0.548, P = .007). The best discriminative parameter was f value. Cut-off value of 32% of f values showed sensitivity of 75.6% and a specificity of 73.5%. Conclusion: ADC values and IVIM parameters such as f values appear to reflect the grade of HCCs