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OBJECTIVE: The aim of this study was to characterize the distribution of skeletal involvement in Erdheim-Chester disease (ECD) by using radiography, computed tomography (CT), 18F-FDG positron emission tomography/computed tomography (PET/CT), and bone scans, as well as looking for associations with the BRAFV600E mutation. MATERIAL AND METHODS: Prospective study of 50 consecutive patients with biopsy-confirmed ECD who had radiographs, CT, 18F-FDG PET/CT, and Tc-99m MDP bone scans. At least two experienced radiologists with expertise in the relevant imaging studies analyzed the images. Summary statistics were expressed as the frequency with percentages for categorical data. Fisher's exact test, as well as odds ratios (OR) with 95 % confidence intervals (CI), were used to link imaging findings to BRAFV600E mutation. The probability for co-occurrence of bone involvement at different locations was calculated and graphed as a heat map. RESULTS: All 50 cases revealed skeletal involvement at different regions of the skeleton. The BRAFV600E mutation, which was found in 24 patients, was correlated with femoral and tibial involvement on 18F-FDG PET/CT and bone scan. The appearance of changes on the femoral, tibial, fibular, and humeral involvement showed correlation with each other based on heat maps of skeletal involvement on CT. CONCLUSION: This study reports the distribution of skeletal involvement in a cohort of patients with ECD. CT is able to detect the majority of ECD skeletal involvement. Considering the complementary nature of information from different modalities, imaging of ECD skeletal involvement is optimized by using a multi-modality strategy.
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Doença de Erdheim-Chester , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Humanos , Doença de Erdheim-Chester/diagnóstico por imagem , Doença de Erdheim-Chester/genética , Fluordesoxiglucose F18 , Imagem Multimodal , Mutação , Estudos Prospectivos , Proteínas Proto-Oncogênicas B-raf/genéticaRESUMO
Pulmonary hypertension may be idiopathic or related to a large variety of diseases. Various imaging examinations may be helpful in diagnosing and determining the etiology of pulmonary hypertension. Imaging examinations discussed in this document include chest radiography, ultrasound echocardiography, ventilation/perfusion scintigraphy, CT, MRI, right heart catheterization, and pulmonary angiography. The ACR Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision process support the systematic analysis of the medical literature from peer-reviewed journals. Established methodology principles such as Grading of Recommendations Assessment, Development, and Evaluation or GRADE are adapted to evaluate the evidence. The RAND/UCLA Appropriateness Method User Manual provides the methodology to determine the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances in which peer-reviewed literature is lacking or equivocal, experts may be the primary evidentiary source available to formulate a recommendation.
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Hipertensão Pulmonar , Humanos , Hipertensão Pulmonar/diagnóstico por imagem , Sociedades Médicas , Medicina Baseada em Evidências , Ecocardiografia , Imageamento por Ressonância MagnéticaRESUMO
Supplemental material is available for this article.
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Ischemic heart disease is a leading cause of death worldwide and comprises a large proportion of annual health care expenditure. Management of ischemic heart disease is now best guided by the physiologic significance of coronary artery stenosis. Invasive coronary angiography is the standard for diagnosing coronary artery stenosis. However, it is expensive and has risks including vascular access site complications and contrast material-induced nephropathy. Invasive coronary angiography requires fractional flow reserve (FFR) measurement to determine the physiologic significance of a coronary artery stenosis. Multiple noninvasive cardiac imaging modalities can also anatomically delineate or functionally assess for significant coronary artery stenosis, as well as detect the presence of myocardial infarction (MI). While coronary CT angiography can help assess the degree of anatomic stenosis, its inability to assess the physiologic significance of lesions limits its specificity. Physiologic significance of coronary artery stenosis can be determined by cardiac MR vasodilator or dobutamine stress imaging, CT stress perfusion imaging, FFR CT, PET myocardial perfusion imaging (MPI), SPECT MPI, and stress echocardiography. Clinically unrecognized MI, another clear indicator of physiologically significant coronary artery disease, is relatively common and is best evaluated with cardiac MRI. The authors illustrate the spectrum of imaging findings of ischemic heart disease (coronary artery disease, myocardial ischemia, and MI); highlight the advantages and disadvantages of the various noninvasive imaging methods used to assess ischemic heart disease, as illustrated by recent clinical trials; and summarize current indications and contraindications for noninvasive imaging techniques for detection of ischemic heart disease. Online supplemental material is available for this article. Published under a CC BY 4.0 license.
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Doença da Artéria Coronariana , Estenose Coronária , Reserva Fracionada de Fluxo Miocárdico , Isquemia Miocárdica , Imagem de Perfusão do Miocárdio , Angiografia Coronária , Humanos , Isquemia Miocárdica/diagnóstico por imagemRESUMO
PURPOSE: To assess differences in FDG-PET/CT uptake among four subtypes of renal tumors: clear cell RCC (ccRCC), papillary type I and II RCC (pRCC), and oncocytoma. METHODS: This retrospective study investigated 33 patients with 98 hereditary renal tumors. Lesions greater than 1 cm and patients with a timeframe of less than 18 months between preoperative imaging and surgery were considered. FDG-PET/CT images were independently reviewed by two nuclear medicine physicians, blinded to clinical information. Volumetric lesion SUVmean was measured and used to calculate a target-to-background ratio respective to liver (TBR). The Shrout-Fleiss intra-class correlation coefficient was used to assess reliability between readers. A linear mixed effects model, accounting for within-patient correlations, was used to compare TBR values of primary renal lesions with and without distant metastasis. RESULTS: The time interval between imaging and surgery for all tumors had a median of 77 (Mean: 139; Range: 1-512) days. Intra-class reliability of mean TBR resulted in a mean κ score of 0.93, indicating strong agreement between the readers. The mixed model showed a significant difference in mean TBR among the subtypes (p < 0.0001). Pairwise comparison showed significant differences between pRCC type II and ccRCC (p < 0.0001), pRCC type II and pRCC type I (p = 0.0001), and pRCC type II and oncocytoma (p = 0.0016). Furthermore, a significant difference in FDG uptake was present between primary pRCC type II renal lesions with and without distant metastasis (p = 0.023). CONCLUSION: pRCC type II lesions demonstrated significantly higher FDG activity than ccRCC, pRCC type I, or oncocytoma. These findings indicate that FDG may prove useful in studying the metabolic activity of renal neoplasms, identifying lesions of highest clinical concern, and ultimately optimizing active surveillance, and personalizing management plans.
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Carcinoma de Células Renais , Neoplasias Renais , Carcinoma de Células Renais/diagnóstico por imagem , Carcinoma de Células Renais/genética , Diferenciação Celular , Fluordesoxiglucose F18 , Humanos , Neoplasias Renais/diagnóstico por imagem , Neoplasias Renais/genética , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Reprodutibilidade dos Testes , Estudos RetrospectivosRESUMO
In this work, we sought to delineate the prevalence of cardiothoracic imaging findings of Proteus syndrome in a large cohort at our institution. Of 53 individuals with a confirmed diagnosis of Proteus syndrome at our institution from 10/2001 to 10/2019, 38 individuals (men, n = 23; average age = 24 years) underwent cardiothoracic imaging (routine chest CT, CT pulmonary angiography and/or cardiac MRI). All studies were retrospectively and independently reviewed by two fellowship-trained cardiothoracic readers. Disagreements were resolved by consensus. Differences between variables were analyzed via parametric and nonparametric tests based on the normality of the distribution. The cardiothoracic findings of Proteus syndrome were diverse, but several were much more common and included: scoliosis from bony overgrowth (94%), pulmonary venous dilation (62%), band-like areas of lung scarring (56%), and hyperlucent lung parenchyma (50%). In addition, of 20 individuals who underwent cardiac MRI, 9/20 (45%) had intramyocardial fat, mostly involving the endocardial surface of the left ventricular septal wall. There was no statistically significant difference among the functional cardiac parameters between individuals with and without intramyocardial fat. Only one individual with intramyocardial fat had mildly decreased function (LVEF = 53%), while all others had normal ejection fraction.
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Diagnóstico por Imagem , Síndrome de Proteu/diagnóstico , Tórax/anormalidades , Tórax/diagnóstico por imagem , Adolescente , Adulto , Criança , Diagnóstico por Imagem/métodos , Feminino , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/genética , Humanos , Pulmão/anormalidades , Pulmão/diagnóstico por imagem , Imageamento por Ressonância Magnética , Masculino , Mediastino/anormalidades , Mediastino/diagnóstico por imagem , Pessoa de Meia-Idade , Parede Torácica/anormalidades , Parede Torácica/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Adulto JovemRESUMO
RATIONALE AND OBJECTIVES: Our primary aim was to improve radiology reports by increasing concordance of target lesion measurements with oncology records using radiology preprocessors (RP). Faster notification of incidental actionable findings to referring clinicians and clinical radiologist exam interpretation time savings with RPs quantifying tumor burden were also assessed. MATERIALS AND METHODS: In this prospective quality improvement initiative, RPs annotated lesions before radiologist interpretation of CT exams. Clinical radiologists then hyperlinked approved measurements into interactive reports during interpretations. RPs evaluated concordance with our tumor measurement radiologist, the determinant of tumor burden. Actionable finding detection and notification times were also deduced. Clinical radiologist interpretation times were calculated from established average CT chest, abdomen, and pelvis interpretation times. RESULTS: RPs assessed 1287 body CT exams with 812 follow-up CT chest, abdomen, and pelvis studies; 95 (11.7%) of which had 241 verified target lesions. There was improved concordance (67.8% vs. 22.5%) of target lesion measurements. RPs detected 93.1% incidental actionable findings with faster clinician notification by a median time of 1 hour (range: 15 minutes-16 hours). Radiologist exam interpretation times decreased by 37%. CONCLUSIONS: This workflow resulted in three-fold improved target lesion measurement concordance with oncology records, earlier detection and faster notification of incidental actionable findings to referring clinicians, and decreased exam interpretation times for clinical radiologists. These findings demonstrate potential roles for automation (such as AI) to improve report value, worklist prioritization, and patient care.
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Inteligência Artificial , Radiologia , Fluxo de Trabalho , Humanos , Estudos Prospectivos , RadiologistasRESUMO
Bladder cancer is the ninth most common cancer, expected to lead to an estimated 17,670 deaths in the United States in 2019. Clinical management and prognosis of bladder cancer mainly depend on the extent of locoregional disease, particularly whether bladder muscle is involved. Therefore, bladder cancer is often divided into superficial, non-muscle-invasive bladder cancer and muscle-invasive bladder cancer; the latter often prompts consideration for cystectomy. While precise staging prior to cystectomy is crucial, the optimal preoperative imaging modality used to stage the disease remains controversial. Transurethral resection of bladder tumor (TURBT) followed by computed tomography (CT) urography is the current recommended approach for staging bladder cancer but suffers from a high rate of understaging. We review the recent literature and compare different imaging modalities for assessing the presence of muscle invasion and lymph node involvement prior to cystectomy and highlight the advantages of each modality.
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Período Pré-Operatório , Neoplasias da Bexiga Urinária/diagnóstico por imagem , Neoplasias da Bexiga Urinária/patologia , Cistectomia , Cistoscopia , Humanos , Metástase Linfática/diagnóstico por imagem , Metástase Linfática/patologia , Invasividade Neoplásica/diagnóstico por imagem , Invasividade Neoplásica/patologia , Estadiamento de Neoplasias , Sensibilidade e Especificidade , Neoplasias da Bexiga Urinária/cirurgiaRESUMO
BACKGROUND: Peritoneal free fluid can indicate an underlying disease process; however detection of minimal peritoneal free fluid in healthy children is not uncommon. OBJECTIVE: To assess the significance of incidental peritoneal free fluid within healthy children by MRI and its relation to physiological changes during puberty. MATERIALS AND METHODS: This prospective study was performed on 32 healthy volunteers (20 boys) between the ages of 8 years and 13 years, with consecutive follow-ups every 8-10 months for an average of 3 years. Body mass index (BMI) z-score, pubertal status, C-reactive protein and sex hormone concentrations were assessed prior to MRI studies. We reviewed a total of 120 pelvic MRI studies (61 boys) and measured the quantity of peritoneal free fluid. For statistical analysis we used linear mixed-model accounting for within-patient correlations. RESULTS: The mean ± standard deviation volume of peritoneal free fluid was 4.7±5.7 mL in girls and 1.9±3.1 mL in boys, with a maximum volume of 25 mL and 17 mL, respectively. The prevalence of peritoneal free fluid was significantly higher in girls (91%) compared to boys (67%; P=0.0035). In 15% of the girls and 3% of the boys the fluid was greater than 10 mL. The mean volume of peritoneal free fluid in the fourth stage of puberty was higher and significantly different from the mean volume in the first stage of puberty (P=0.01). CONCLUSION: Among healthy pubescent children, the prevalence of peritoneal free fluid is significantly higher in girls. The volume of peritoneal free fluid can reach volumes greater than 10 mL during normal puberty, especially in the fourth stage, and can be assumed normal in the absence of active disease.
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Líquido Ascítico , Imageamento por Ressonância Magnética/métodos , Peritônio/diagnóstico por imagem , Puberdade , Adolescente , Criança , Feminino , Humanos , Achados Incidentais , Masculino , Estudos ProspectivosRESUMO
Limited information is available regarding interstitial lung disease (ILD) in Erdheimâ»Chester disease (ECD), a rare multisystemic non-Langerhans cell histiocytosis. Sixty-two biopsy-confirmed ECD patients were divided into those with no ILD (19.5%), minimal ILD (32%), mild ILD (29%), and moderate/severe ILD (19.5%), based on computed tomography (CT) findings. Dyspnea affected at least half of the patients with mild or moderate/severe ILD. Diffusion capacity was significantly reduced in ECD patients with minimal ILD. Disease severity was inversely correlated with pulmonary function measurements; no correlation with BRAF V600E mutation status was seen. Reticulations and ground-glass opacities were the predominant findings on CT images. Automated CT scores were significantly higher in patients with moderate/severe ILD, compared to those in other groups. Immunostaining of lung biopsies was consistent with ECD. Histopathology findings included subpleural and septal fibrosis, with areas of interspersed normal lung, diffuse interstitial fibrosis, histiocytes with foamy cytoplasm embedded in fibrosis, lymphoid aggregates, and focal type II alveolar cell hyperplasia. In conclusion, ILD of varying severity may affect a high proportion of ECD patients. Histopathology features of ILD in ECD can mimic interstitial fibrosis patterns observed in idiopathic ILD.
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OBJECTIVES: To investigate the computed tomography (CT) thoracic findings in Erdheim-Chester disease (ECD) and evaluate the association of these findings with the BRAFV600E mutation. METHODS: This was a prospective study of patients with ECD (n=61, men=46) who underwent thoracic CT imaging. CT examinations were independently interpreted by two experienced radiologists. Association of imaging findings with BRAFV600E was achieved via the Chi-square or Fisher's exact test and odds ratios (OR) with 95% confidence intervals (CI), as appropriate. RESULTS: Fifty-five ECD patients (90%) showed pulmonary findings, which included interlobular septal thickening (69%), pulmonary nodules (62%), airway thickening (13%) and ground glass opacities (36%). Pulmonary nodules were classified by the pattern of distribution: subpleural regions (36%), lung parenchyma (13%) and both regions (13%). Pleural and mediastinal involvement were present in 15% and 62% of cases, respectively. The most common mediastinal finding was sheathing of the right coronary artery (34%), followed by sheathing of the thoracic aorta (30%). The BRAFV600E mutation, positive in 31 patients, was associated with the frequency of sheathing of the coronary arteries (p = 0.01). CONCLUSIONS: Of the thoracic findings reported in this study, we found a statistically significant positive association between the BRAFV600E mutation and presence of coronary artery sheathing. KEY POINTS: ⢠To assess the degree of thoracic involvement in ECD with CT. ⢠BRAF V600E mutation has a high association with right coronary artery sheathing. ⢠BRAF V600E genetic testing detects patients at high risk of developing RCA sheathing.
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Doença de Erdheim-Chester/genética , Doença de Erdheim-Chester/patologia , Proteínas Proto-Oncogênicas B-raf/genética , Tórax/patologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Aorta Torácica/patologia , Criança , Pré-Escolar , Vasos Coronários/patologia , Doença de Erdheim-Chester/diagnóstico por imagem , Feminino , Humanos , Pulmão/patologia , Masculino , Mediastino/patologia , Pessoa de Meia-Idade , Nódulos Pulmonares Múltiplos/patologia , Mutação , Pleura/patologia , Estudos Prospectivos , Proto-Oncogene Mas , Tórax/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Adulto JovemRESUMO
OBJECTIVES: To use magnetic resonance imaging (MRI) and computed tomography (CT) to define abdominal involvement in Erdheim-Chester disease (ECD), and to investigate the association between these findings and the BRAFV600E mutation. METHODS: This prospective study was performed on 61 ECD patients (46 men). The MRI and CT imaging studies were reviewed independently by two experienced radiologists. The association between BRAFV600E mutation and imaging findings was analysed using Fisher's exact test, and odds ratios with 95% confidence intervals. RESULTS: Perinephric infiltration was the most common finding (67%), followed by involvement of proximal ureters (61%). In 56% of cases, infiltration extended to the renal sinuses, and in 38% caused hydronephrosis. Adrenal gland infiltration was present in 48% of patients. Infiltration of renal artery (49%) and aorta (43%) were the most common vascular findings, followed by sheathing of celiac, superior mesenteric artery (SMA) or inferior mesenteric artery (IMA) (23%). The BRAFV600E mutation was positive in 53% of patients with interpretable BRAF sequencing. There was a statistically significant association between this mutation and perinephric infiltration (p = 0.003), renal sinus involvement (p < 0.001), infiltration of proximal ureters (p < 0.001), hydronephrosis (p < 0.001), adrenal gland involvement (p < 0.001), periaortic infiltration (p = 0.03), sheathing or stenosis of renal artery (p < 0.001) and sheathing of other aortic branches (p = 0.04). CONCLUSIONS: Renal and vascular structures are the most commonly affected abdominal organs in ECD patients. Some of these findings have significant positive association with the BRAFV600E mutation. KEY POINTS: ⢠Abdominal imaging plays a crucial role in management of Erdheim-Chester disease. ⢠Significant associations exist between BRAF V600E mutation and several abdominal imaging findings. ⢠Considering several associations, evaluating BRAFV600E mutation status is recommended in ECD patients.
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Abdome/patologia , Doença de Erdheim-Chester/diagnóstico por imagem , Doença de Erdheim-Chester/genética , Imageamento por Ressonância Magnética/métodos , Proteínas Proto-Oncogênicas B-raf/genética , Tomografia Computadorizada por Raios X/métodos , Abdome/diagnóstico por imagem , Adulto , Idoso , Doença de Erdheim-Chester/patologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Mutação/genética , Estudos Prospectivos , Proto-Oncogene Mas , Adulto JovemRESUMO
PURPOSE: To determine whether objective volumetric whole-lesion apparent diffusion coefficient (ADC) distribution analysis improves upon the capabilities of conventional subjective small region-of-interest (ROI) ADC measurements for prediction of renal cell carcinoma (RCC) subtype. METHODS: This IRB-approved study retrospectively enrolled 55 patients (152 tumors). Diffusion-weighted imaging DWI was acquired at b values of 0, 250, and 800 s/mm2 on a 1.5T system (Aera, Siemens Healthcare). Whole-lesion measurements were performed by a research fellow and reviewed by a fellowship-trained radiologist. Mean, median, skewness, kurtosis, and every 5th percentile ADCs were determined from the whole-lesion histogram. Linear mixed models that accounted for within-subject correlation of lesions were used to compare ADCs among RCC subtypes. Receiver-operating characteristic (ROC) curve analysis with optimal cutoff points from the Youden index was used to test the ability of ADCs to differentiate clear cell RCC (ccRCC), papillary RCC (pRCC), and oncocytoma subtypes. RESULTS: Whole-lesion ADC values were significantly different between pRCC and ccRCC, and between pRCC and oncocytoma, demonstrating strong ability to differentiate subtypes across the quantiles (both P < 0.001). Best percentile ROC analysis demonstrated AUC values of 95.2 for ccRCC vs. pRCC; 67.6 for oncocytoma vs. ccRCC; and 95.8 for oncocytoma vs. pRCC. Best percentile ROC analysis further indicated model sensitivities/specificities of 84.5%/93.1% for ccRCC vs. pRCC; 100.0%/10.3% for oncocytoma vs. ccRCC; and 88.5%/93.1% for oncocytoma vs. pRCC. CONCLUSION: The objective methodology of whole-lesion volumetric ADC measurements maintains the sensitivity/specificity of conventional expert-based ROI analysis, provides information on lesion heterogeneity, and reduces observer bias.