Radiologic Response Assessment in Pediatric Soft Tissue Sarcoma: Computed-Assisted Volume Evaluation.

OBJECTIVES: To compare 3 methods of dimensional assessment, with particular attention to a new software assisted method of volume calculation, in soft tissue sarcoma, and to investigate the interobserver agreement and the intermethod agreement in chemotherapy response classification and resultant clinical repercussions. STUDY DESIGN: We studied 34 pediatric patients with nonmetastatic soft tissue sarcoma who had undergone only diagnostic biopsy. Tumor size was measured both at diagnosis and after induction chemotherapy by 3 observers and using 3 measurement methods: maximum axis (1 diameter), estimated volume (3 diameters), and computed volume (software-assisted volume calculation). We used overall concordance correlation coefficient and Bland-Altman statistical methods to assess interobserver agreement and overall concordance correlation coefficient and the κ Cohen coefficient to assess intermethod agreement. RESULTS: According to overall concordance correlation coefficient, the interobserver agreement was very high for each method, with a slight superiority of the software assisted method; this agreement was not confirmed in Bland-Altman plots for maximum axis and estimated volume methods. According to kappa coefficients, the intermethod agreement in chemotherapy response evaluation was poor. CONCLUSIONS: Computed volume was the most accurate method in soft tissue sarcoma tumor size assessment. One- and 3-dimensional methods are not concordant in chemotherapy response classification. In particular, the maximum axis method underestimates chemotherapy response and can lead to switching the chemotherapy regimen erroneously.

Vascular perfusion kinetics by contrast-enhanced ultrasound are related to synovial microvascularity in the joints of psoriatic arthritis.

The purpose of the study was to assess the relationship of the continuous mode contrast-enhanced harmonic ultrasound (CEUS) imaging with the histopathological and immunohistochemical (IHC) quantitative estimation of microvascular proliferation on synovial samples of patients affected by sustained psoriatic arthritis (PsA). A dedicated linear transducer was used in conjunction with a specific continuous mode contrast enhanced harmonic imaging technology with a second-generation sulfur hexafluoride-filled microbubbles C-agent. The examination was carried out within 1 week before arthroscopic biopsies in 32 active joints. Perfusional parameters were analyzed including regional blood flow (RBF); peak (PEAK) of the C-signal intensity, proportional to the regional blood volume (RBV); beta (ß) perfusion frequency; slope (S), representing the inclination of the tangent in the origin; and the refilling time (RT), the reverse of beta. Arthroscopic synovial biopsies were targeted in the hypervascularity areas, as in the same knee recesses assessed by CEUS; the synovial cell infiltrate and vascularity (vessel density) was evaluated by IHC staining of CD45 (mononuclear cell) and CD31, CD105 (endothelial cell) markers, measured by computer-assisted morphometric analysis. In the CEUS area examined, the corresponding time-intensity curves demonstrated a slow rise time. Synovial histology showed slight increased layer lining thickness, perivascular lymphomonocyte cell infiltration, and microvascular remodeling, with marked vessel wall thickening with reduction of the vascular lumen. A significant correlation was found between RT and CD31+ as PEAK and CD105+ vessel density; RT was inversely correlated to RBF, PEAK, S, and ß. The study demonstrated the association of the CEUS perfusion kinetics with the histopathological quantitative and morphologic estimation of synovial microvascular proliferation, suggesting that a CEUS imaging represents a reliable tool for the estimate of the synovial hypervascularity in PsA.

Effects of mud-bath therapy in psoriatic arthritis patients treated with TNF inhibitors. Clinical evaluation and assessment of synovial inflammation by contrast-enhanced ultrasound (CEUS).

OBJECTIVES: Despite the efficacy of TNF inhibitors, most patients with psoriatic arthritis maintain a residual synovial inflammation. The main aim of the study was to evaluate the effects of mud-bath therapy on clinical picture of PsA patients treated with TNF inhibitors. The secondary outcome was to assess synovial inflammation in hand joints detected by contrast-enhanced ultrasound. Other aims were to verify the risk of arthritis flare and to evaluate the effects of spa treatment on functional ability and on quality of life. METHODS: Thirty-six patients with psoriatic arthritis, treated in the last 6 months with TNF inhibitors, were enrolled. After 1:1 randomisation, 18 patients (group A) underwent mud-bath therapy (12 mudpacks and 12 thermal baths), maintaining treatment with TNF inhibitors; 18 patients (group B) continued pharmacological therapy alone. CRP, PASI, DAS28, swollen and tender joint count, VAS pain, HAQ and SF-36 were evaluated at baseline (T0) and after 45 days (T1). Synovial inflammation detected by contrast-enhanced ultrasound, analysed by a software system, was also assessed. RESULTS: A significant improvement in PASI (P<0.005), DAS28 (P<0.05), swollen joint count and tender joint count (P<0.001), and HAQ (P<0.001) between T0 and T1 was observed in group A. No patient underwent a flare-up of arthritis. Ultrasound videos demonstrated a significant appearance delay (P<0.05) and faster washout (P<0.02) of contrast dye in group A patients with respect to group B. CONCLUSIONS: These data suggest a decrease of residual synovial inflammation and a beneficial clinical effect of spa therapy in psoriatic arthritis patients treated with TNF inhibitors.

Role of Radiologic Imaging in Genetic and Acquired Neuromuscular Disorders.

UNLABELLED: Great technologic and clinical progress have been made in the last two decades in identifying genetic defects of several neuromuscular diseases, as Spinal Muscular Atrophy, genetic muscular dystrophies and other genetic myopathies. The diagnosis is usually challenging, due to great variability in genetic abnormalities and clinical phenotypes and the poor specificity of complementary analyses, i.e., serum creatine kinase (CK) and electrophysiology. Muscle biopsy represents the gold standard for the diagnosis of genetic neuromuscular diseases, but clinical imaging of muscle tissue is an important diagnostic tool to identify and quantifyies muscle damage. Radiologic imaging is, indeed, increasingly used as a diagnostic tool to describe patterns and the extent of muscle involvement, thanks to modern techniques that enable to definethe definition of degrees of muscle atrophy and changes in connective tissue. They usually grade the severity of the disease process with greater accuracy than clinical scores. Clinical imaging is more than complementary to perform muscle biopsy, especially as ultrasound scans are often mandatory to identify the muscle to be biopsied. We will here detail and provideWe will herein provide detailed examples of the radiologic methods that can be used in genetic and acquired neuromuscular disorders, stressing pros and cons. KEY WORDS: Muscle Imaging, MRI, CT, genetic muscle disorders, myopathies, dystrophies.

Muscle MR imaging in tubular aggregate myopathy.

PURPOSE: To evaluate with Magnetic Resonance (MR) the degree of fatty replacement and edematous involvement in skeletal muscles in patients with Tubular Aggregate Myopathy (TAM). To asses the inter-observer agreement in evaluating muscle involvement and the symmetry index of fatty replacement. MATERIALS AND METHODS: 13 patients were evaluated by MR to ascertain the degree of fatty replacement (T1W sequences) according to Mercuri's scale, and edema score (STIR sequences) according to extent and site. RESULTS: Fatty replacement mainly affects the posterior superficial compartment of the leg; the anterior compartment is generally spared. Edema was generally poor and almost only in the superficial compartment of the leg. The inter-observer agreement is very good with a Krippendorff's coefficient >0.9. Data show a total symmetry in the muscular replacement (McNemar-Bowker test with p = 1). CONCLUSIONS: MR reveals characteristic muscular involvement, and is a reproducible technique for evaluation of TAM. There may also be a characteristic involvement of the long and short heads of the biceps femoris. It is useful for aimed biopsies, diagnostic hypotheses and evaluation of disease progression.

MR and CEUS monitoring of patients with severe rheumatoid arthritis treated with biological agents: a preliminary study.

PURPOSE: This study was done to propose a study protocol for patients with rheumatoid arthritis (RA) treated with biological agents, by evaluating the contribution of contrast-enhanced magnetic resonance (CE-MR) imaging, a software programme that calculates the volume of synovitis on CE-MR images, and contrast-enhanced ultrasound (CEUS). MATERIALS AND METHODS: Sixteen patients with RA receiving treatment with biologics were analysed. The patients underwent clinical examination, CE-MR imaging and CEUS on the same day. Images were postprocessed with the software and evaluated independently by three physicians in terms of RAMRIS (Rheumatoid Arthritis Magnetic Resonance Imaging Score), SAMIS (Simplified Rheumatoid Arthritis Magnetic Resonance Imaging Score) and CEUS grade. The techniques were correlated statistically. RESULTS: The RAMRIS and SAMIS scores were found to correlate statistically. CE-MR imaging correlated with the clinical data (p < 0.05), whereas CEUS did not. The data provided by the software did not correlate statistically with the other techniques. The most painful joint was consistently found to be the joint with most synovitis. CONCLUSIONS: CE-MR imaging may be used prior to treatment and for long-term follow-up. CEUS might be useful in the short-term follow-up, as it seems to provide an indication of the presence or absence of disease, though not of its severity. The software is a very useful tool that can supplement, but not replace, the other techniques.

Hydroxyurea treatment can avoid the need for aggressive surgery in pediatric fibromatosis.

Aggressive fibromatosis is a rare tumor of borderline malignancy with a marked local aggressiveness. Surgery is the mainstay of treatment but complete tumor resection is often not easy to achieve without functional and cosmetic sequelae. We report a case of a pretreated child with aggressive fibromatosis who responded to hydroxyurea, avoiding the need for demolitive surgery.

Improved detection of synovial boundaries in ultrasound examination by using a cascade of active-contours.

Rheumatoid arthritis (RA) is a chronic multisystemic autoimmune disease, with an unclear etiopathogenesis. Its early diagnosis and activity assessment are essential to adjust the proper therapy. Among the different imaging techniques, ultrasonography (US) allows direct visualization of early inflammatory joint changes as synovitis, being also rapidly performed and easily accepted by patients. We propose an algorithm to semi-automatically detect synovial boundaries on US images, requiring minimal user interaction. In order to identify the synovia-bone and the synovia-soft tissues interfaces, and to tackle the morphological variability of diseased joints, a cascade of two different active contours is developed, whose composition corresponds to the whole synovial boundary. The algorithm was tested on US images acquired from proximal interphalangeal (PIP) and metacarpophalangeal (MCP) finger joints of 34 subjects. The results have been compared with a consensus manual segmentation. We obtained an overall mean sensitivity of 85±13%, and a mean Dice's similarity index of 80±8%, with a mean Hausdorff distance from the manual segmentation of 28±10 pixels (approximately 1.4±0.5mm), that are a better performance than those obtained by the raters with respect to the consensus.

Imaging of soft-tissue tumors.

The objective of this review is to highlight the major imaging characteristics of the main soft-tissue sarcoma histotypes observed in the group "Sarcomi" of the Istituto Oncologico Veneto in the last 5 years. A literature review was performed using PubMed and textbooks. Radiological imaging can guide the diagnosis for the subset of lesions that have typical clinical and imaging features. Soft-tissue tumors are common in clinical practice and a systematic clinical and imaging approach may guide the diagnosis.

Conservative treatment for cystic duct stenosis in a child.

Introduction. Few cases of common bile duct stenosis have been reported in the literature, and observations of strictures in the cystic duct are even more rare. Surgical cholecystectomy is the treatment needed in most cases of gallbladder hydrops. This paper describes the diagnosis and successful medical treatment of a rare pediatric case of cystic duct stenosis and gallbladder hydrops. Case Report. A formerly healthy one-year-old girl was admitted with colicky abdominal pain. Blood tests were normal, except for an increase in transaminases. Abdominal ultrasound excluded intestinal intussusception and identified a distended gallbladder with biliary sludge. MR cholangiography revealed a dilated gallbladder containing bile sediment and no detectable cystic duct, while the rest of the intra- and extrahepatic biliary tree and hepatic parenchyma were normal. This evidence was consistent with gallbladder hydrops associated with cystic duct stenosis. The baby was treated with i.v. hydration, corticosteroids, antibiotics, and ursodeoxycholic acid. Her general condition rapidly improved, with no further episodes of abdominal pain and normalization of liver enzymes. This allowed to avoid cholecystectomy, and the child is well 1.5 years after diagnosis. Conclusions. Although cholecystectomy is usually necessary in case of gallbladder hydrops, our experience suggests that surgical procedures can be avoided when the distension is caused by a cystic duct stenosis.

Sonographic evaluation of bone fractures: a reliable alternative in clinical practice?

OBJECTIVE: The objective was to compare the diagnostic accuracy of conventional radiography and ultrasonography (US) for the diagnosis of suspected bone fractures. METHOD: Eighty-six patients were assessed using conventional radiography and US on the affected bone district. RESULTS: Radiographic and sonographic findings were concordant in 93% of cases. In one case, US suggested a fracture not seen on radiographic assessment. Ultrasonography showed a sensitivity of 0.94 and a specificity of 0.92. CONCLUSION: In clinical practice, US could become the first diagnostic approach.

Evaluation of finger joint synovial vascularity in patients with rheumatoid arthritis using contrast-enhanced ultrasound with water immersion and a stabilized probe.

PURPOSE.: To assess synovial microvascularity in finger joints with rheumatoid arthritis (RA) by contrast-enhanced ultrasound (CEUS), distinguishing between cases of active disease and those in remission; to standardize the technique for software analysis. METHODS.: Fifty-two finger joints of RA patients (26 with active disease and 26 in remission) were immersed in water and examined by CEUS using a fixed probe. Signal intensity curves were calculated with the software. RESULTS.: Contrast enhancement was detectable in all 26 patients with active RA (100%), but not in 25 of 26 patients in remission (96%); one of the latter patients (4%) showed minimal enhancement. The method's sensitivity and specificity in distinguishing active disease from remission were 100% and 96%. The grades of synovial enhancement correlated with clinical disease activity and software flow parameters. The peak contrast levels correlated with clinical activity, a peak of 9% representing the cutoff between remission and active disease. CONCLUSIONS.: CEUS with a fixed probe on finger joints immersed in water detected synovial vascularization in RA, producing results suitable for standardized software analysis and avoiding artifacts.

Structured reporting using a shared indexed multilingual radiology lexicon.

PURPOSE: A system for creating structured reports (SRs) using a standardized radiology lexicon was developed and tested to facilitate automated translation of content and multidisciplinary international communications. METHODS: A database of radiology terms, RadLex developed by the Radiological Society of North America, was used to create a shared indexed multilingual radiology lexicon. A diagnostic workstation for generating structured reports (OpenEye) was implemented with a "RadLex manager" function for adding new words to the lexicon in both English and Italian. Sample reports of examinations included in the Medical Imaging Resource Center (MIRC) radiology imaging database of clinical cases were prepared using this system. The system was evaluated for teaching purposes and scientific dissemination. RESULTS: The OpenEye system was able to manage the glossary to create new SRs and manually translate existing reports containing freely worded descriptions. The OpenEye system provides instant translation from Italian into English and enables clinical cases to be published in the MIRC, while making them accessible for consultation on an international scale. CONCLUSION: The SR is advantageous compared with a freely worded report in terms of clarity and completeness of the content. Creating SRs for each clinical case enables rapid and focused analysis at multidisciplinary meetings. All our cases have been included in the MIRC database as part of a broader-based European Project for research on soft tissues sarcomas (CONTICANET).

Contrast-enhanced ultrasound for characterizing lymph nodes with focal cortical thickening in patients with cutaneous melanoma.

OBJECTIVE: The aim of this study was to ascertain the utility of contrast-enhanced ultrasound in assessing the significance of focal cortical thickening in the lymph nodes of patients followed up after surgery for cutaneous melanoma. MATERIALS AND METHODS: Ultrasound was used to examine 460 consecutive patients to identify nodes with focal hypoechoic cortical thickening. Patients whose nodes revealed these features underwent contrast-enhanced ultrasound and ultrasound-guided fine-needle aspiration cytology (FNAC) focusing on the area of cortical thickening. Enhancement in the arterial and parenchymal phases was evaluated: A generalized homogeneous or intense enhancement was considered benign and the presence of a perfusion defect was considered metastatic. RESULTS: After exclusion of 24 patients with frank signs of malignancy at gray-scale ultrasound, the study included 436 patients. Focal hypoechoic cortical thickening was seen in 44 of 436 nodes in as many patients. In 29 nodes, the area of focal thickening showed contrast enhancement similar to that of the remaining cortex on contrast-enhanced ultrasound. In 15 nodes, the area of cortical thickening was less well vascularized than the adjacent parenchyma in the arterial phase and there were areas with perfusion defects that were more evident in the parenchymal phase. FNAC focusing on the areas of focal cortical thickening identified 13 metastatic nodes and 31 nodes with benign features. Contrast-enhanced ultrasound compared with FNAC correctly classified 42 of 44 nodes, showing a sensitivity of 100% and a specificity of 99.5%. CONCLUSION: Although our findings need to be confirmed in larger series, they indicate that contrast-enhanced ultrasound can be useful in clinical practice for characterizing focal cortical thickening in lymph nodes. The exclusion or identification of regional lymph node metastases is of fundamental importance in oncologic staging because this issue directly influences both the prognosis and the choice of therapeutic strategy.

The role of contrast-enhanced gray-scale ultrasonography in the differential diagnosis of superficial lymph nodes.

Lymph node micrometastases are common, but too often in clinical practice lack the tools for their accurate prebiopsy detection. The gray-scale contrast-enhanced ultrasonography technique permits high-resolution imaging of both the arterial and parenchymal phase and allows visualization of diffuse and partial alterations of nodal perfusion even in lymph nodes with a maximum diameter smaller than 1 cm. The gray-scale contrast-enhanced ultrasonography can supply further useful information in case where doubt has arisen with conventional techniques. The results obtained show that it affords highly accurate differentiation between benign and metastatic lymph nodes.