HEOCHROMOCYTOMA AND PARAganglioma are tumors of the autonomic nervous system. Terminology in science and clinical practice is divergent. Herein, we use the term pheochromocytoma for location in the adrenal glands, extraadrenal abdominal, and thoracic locations (eg, where nearly all tumors are endocrinologically active). In contrast, the term paraganglioma is only used for tumors in the head and neck area where most tumors are nonfunctioning. All these tumors have been described as sporadic and as hereditary entities. [1][2][3] Estimated yearly incidence of

To develop evidence-based recommendations for the use of imaging modalities in primary large vessel vasculitis (LVV) including giant cell arteritis (GCA) and Takayasu arteritis (TAK).European League Against Rheumatism (EULAR) standardised operating procedures were followed. A systematic literature review was conducted to retrieve data on the role of imaging modalities including ultrasound, MRI, CT and [ 18

Purpose:To evaluate an improved image acquisition and data-processing strategy for assessing aortic vascular geometry and 3D blood flow at 3T. Materials and Methods:In a study with five normal volunteers and seven patients with known aortic pathology, prospectively ECG-gated cine three-dimensional (3D) MR velocity mapping with improved navigator gating, real-time adaptive k-space ordering and dynamic adjustment of the navigator acceptance criteria was performed. In addition to morphological information and three-directional blood flow velocities, phase-contrast (PC)-MRA images were derived from the same data set, which permitted 3D isosurface rendering of vascular boundaries in combination with visualization of blood-flow patterns.Results: Analysis of navigator performance and image quality revealed improved scan efficiencies of 63.6% Ϯ 10.5% and temporal resolution (Ͻ50 msec) compared to previous implementations. Semiquantitative evaluation of image quality by three independent observers demonstrated excellent general image appearance with moderate blurring and minor ghosting artifacts. Results from volunteer and patient examinations illustrate the potential of the improved image acquisition and data-processing strategy for identifying normal and pathological blood-flow characteristics. Conclusion:Navigator-gated time-resolved 3D MR velocity mapping at 3T in combination with advanced data processing is a powerful tool for performing detailed assessments of global and local blood-flow characteristics in the aorta to describe or exclude vascular alterations.

A wide variety of fat suppression and water-fat separation methods are used to suppress fat signal and improve visualization of abnormalities. This article reviews the most commonly used techniques for fat suppression and fatwater imaging including 1) chemically selective fat suppression pulses "FAT-SAT"; 2) spatial-spectral pulses (water excitation); 3) short inversion time (TI) inversion recovery (STIR) imaging; 4) chemical shift based water-fat separation methods; and finally 5) fat suppression and balanced steady-state free precession (SSFP) sequences. The basic physical background of these techniques including their specific advantages and disadvantages is given and related to clinical applications. This enables the reader to understand the reasons why some fat suppression methods work better than others in specific clinical settings.

Dystrophic epidermolysis bullosa (DEB) is a severe skin fragility disorder associated with trauma-induced blistering, progressive soft tissue scarring, and increased risk of skin cancer. DEB is caused by mutations in type VII collagen. In this study, we describe the generation of a collagen VII hypomorphic mouse that serves as an immunocompetent animal model for DEB. These mice expressed collagen VII at about 10% of normal levels, and their phenotype closely resembled characteristics of severe human DEB, including mucocutaneous blistering, nail dystrophy, and mitten deformities of the extremities. The oral blistering experienced by these mice resulted in growth retardation, and repeated blistering led to excessive induction of tissue repair, causing TGF-β1-mediated contractile fibrosis generated by myofibroblasts and pseudosyndactyly in the extremities. Intradermal injection of WT fibroblasts resulted in neodeposition of collagen VII and functional restoration of the dermal-epidermal junction. Treated areas were also resistant to induced frictional stress. In contrast, untreated areas of the same mouse showed dermal-epidermal separation following induced stress. These data demonstrate that fibroblast-based treatment can be used to treat DEB in a mouse model and suggest that this approach may be effective in the development of clinical therapeutic regimens for patients with DEB. IntroductionSkin integrity and resistance to mechanical stress rely on the function of the dermal-epidermal junction zone (DEJZ), which anchors the epidermis to the underlying dermal matrix. The supramolecular cell adhesion complexes at the DEJZ mediate interactions of the cytoskeleton in basal keratinocytes with the basement membrane and the extracellular anchoring fibrils, which emanate from the basement membrane into the dermis and entrap dermal collagen bundles, thus establishing stable dermal-epidermal cohesion (1).The main component of the anchoring fibrils is collagen VII, a homotrimeric collagen synthesized by keratinocytes and fibroblasts (2). Fibril formation and deposition at the DEJZ requires proteolytic processing of procollagen VII to mature collagen (3). Loss of collagen VII functions in dystrophic epidermolysis bullosa (DEB) leads to absence or anomalies of the anchoring fibrils and to dermal-epidermal tissue separation. DEB refers to a clinically heterogeneous group of disorders including recessively and dominantly inherited subtypes (4, 5). All forms of DEB are allelic and caused by mutations in the collagen VII gene, COL7A1. Early investigations demonstrated reduced amounts of anchoring fibrils and collagen VII in the skin of patients with mild and moderate, reces-

Purpose Investigating the value of 68Ga-PSMA-PET/CT in biochemically recurring prostate cancer patients with negative 18F-choline-PET/CT. Patients and Methods One hundred thirty-nine consecutive patients with biochemical recurrence after curative (surgery and/or radiotherapy) therapy were offered participation in this sequential clinical imaging approach. Patients first underwent an 18F-choline-PET/CT. If negative, an additional 68Ga-PSMA-PET/CTwas offered. One hundred twenty-five of 139 eligible patients were included in the study; 32 patients underwent additional 68Ga-PSMA-PET/CT. Patients with equivocal findings (n = 5) on 18F-choline-PET/CT and those who declined the additional 68Ga-PSMA-PET/CT (n = 9) were excluded. Images were analyzed visually for the presence of suspicious lesions. Findings on PET/CT were correlated with PSA level, PSA doubling time (dt), and PSA velocity (vel). Results The overall detection rates were 85.6% (107/125) for the sequential imaging approach and 74.4% (93/125) for 18F-choline-PET/CT alone. 68Ga-PSMA-PET/CT detected sites of recurrence in 43.8% (14/32) of the choline-negative patients. Detection rates of the sequential imaging approach and 18F-choline-PET/CT alone increased with higher serum PSA levels and PSA vel. Subgroup analysis of 68Ga-PSMA-PET/CT in 18F-choline negative patients revealed detection rates of 28.6%, 45.5%, and 71.4% for PSA levels of 0.2 or greater to less than 1 ng/mL, 1 to 2 ng/mL, and greater than 2 ng/mL, respectively. Conclusions The sequential imaging approach designed to limit 68Ga-PSMA imaging to patients with negative choline scans resulted in high detection rates. 68Ga-PSMA-PET/CT identified sites of recurrent disease in 43.8% of the patients with negative 18F-choline PET/CT scans.

Purpose: To determine the sources of variability of MRE hepatic stiffness measurements using healthy volunteers and patients and to calculate the minimum change required for statistical significance. Hepatic stiffness measured with magnetic resonance elastography (MRE) has demonstrated tremendous potential as a noninvasive surrogate of hepatic fibrosis, although the underlying repeatability of MRE for longitudinal tracking of liver disease has not been documented. Materials and Methods:MRE stiffness measurements from 20 healthy volunteers and 10 patients were obtained twice on the same day, and repeated 2-4 weeks later for volunteers in this institutional review board-approved study. A linear mixed effects model was used to estimate the component sources of variability in the data. Results:The standard deviation of MRE measurements of the same individual on different days is 11.9% (percent of the measured stiffness) using the same reader and 12.0% using different readers. The standard deviation of the difference between two measurements (i.e., longitudinal change in an individual) is 17.4%; the corresponding 95% confidence interval for zero change is (À27.0%, 37.0%).Conclusion: MRE is a repeatable method for quantifying liver stiffness. Using the described MRE technique, changes greater than 37.0% of the smaller measured stiffness value represent meaningful changes in longitudinal liver stiffness measurements.

Time-resolved phase contrast (PC) MRI with velocity encoding in three directions (flow-sensitive four-dimensional MRI) can be employed to assess three-dimensional blood flow in the entire aortic lumen within a single measurement. These data can be used not only for the visualization of blood flow but also to derive additional information on vascular geometry with three-dimensional PC MR angiography (MRA). As PC-MRA is sensitive to available signal-to-noise ratio, standard and novel blood pool contrast agents may help to enhance PC-MRA image quality. In a group of 30 healthy volunteers, the influence of different contrast agents on vascular signalto-noise ratio, PC-MRA quality, and subsequent three-dimensional stream-line visualization in the thoracic aorta was determined. Flow-sensitive four-dimensional MRI data acquired with contrast agent provided significantly improved signal-to-noise ratio in magnitude data and noise reduction in velocity data compared to measurements without contrast media. The agreement of three-dimensional PC-MRA with reference standard contrast-enhanced MRA was good for both contrast agents, with improved PC-MRA performance for blood pool contrast agent, particularly for the smaller supraaortic branches. While most clinical applications of MRA rely on the application of Gadolinium (Gd) contrast agent (CA), three-dimensional (3D) phase contrast (PC)-MRA based on velocity-encoded 3D MRI with encoding in three directions has proven to be a useful alternative (8-11). PC-MRA can provide detailed information on vascular geometry and may offer additional information on flow direction. However, most PC-MRA implementations used nongated data acquisition, which can result in artifacts for pulsatile blood flow. Further drawbacks of the PC-MRA method are long scan times and lack of respiration control, which limited most applications of 3D PC-MRA to static regions with low pulsatile flow such as the cranial vessels (12,13).Recently, improved time-resolved (CINE) 3D PC MRI techniques using electrocardiography (ECG) gating and advanced navigator respiration control (flow-sensitive four-dimensional [4D] MRI) have been successfully applied for the analysis of pulsatile 3D blood flow in the aorta (14-24). Such techniques offer the opportunity for the detailed analysis of pulsatile 3D blood flow but require scan times up to 20 min. We previously reported an approach to derive 3D angiographic information (3D PC-MRA) from flow-sensitive 4D MRI (24,25). We showed that it was possible to exploit the information in the acquired flow-sensitive 4D data to derive angiographic information without performing additional MRA measurements. Although the derived 3D PC-MRA does not provide the same detailed depiction of anatomy and morphology compared to CE-MRA, it can improve considerably the presentation of the results by combining 3D visualization of anatomy and flow for large vascular geometries such as the thoracic aorta.Based on this strategy, an improved data processing workflow including noise masking was impleme...