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OBJECTIVES: To prospectively investigate concordance between whole-body MRI (WB-MRI) and a composite reference standard for initial staging and interim response evaluation in paediatric and adolescent Hodgkin's lymphoma. METHODS: Fifty patients (32 male, age range 6-19 years) underwent WB-MRI and standard investigations, including 18F-FDG-PET-CT at diagnosis and following 2-3 chemotherapy cycles. Two radiologists in consensus interpreted WB-MRI using prespecified definitions of disease positivity. A third radiologist reviewed a subset of staging WB-MRIs (n = 38) separately to test for interobserver agreement. A multidisciplinary team derived a primary reference standard using all available imaging/clinical investigations. Subsequently, a second multidisciplinary panel rereviewed all imaging with long-term follow-up data to derive an enhanced reference standard. Interobserver agreement for WB-MRI reads was tested using kappa statistics. Concordance for correct classification of all disease sites, true positive rate (TPR), false positive rate (FPR) and kappa for staging/response agreement were calculated for WB-MRI. RESULTS: There was discordance for full stage in 74% (95% CI 61.9-83.9%) and 44% (32.0-56.6%) of patients against the primary and enhanced reference standards, respectively. Against the enhanced reference standard, the WB-MRI TPR, FPR and kappa were 91%, 1% and 0.93 (0.90-0.96) for nodal disease and 79%, < 1% and 0.86 (0.77-0.95) for extra-nodal disease. WB-MRI response classification was correct in 25/38 evaluable patients (66%), underestimating response in 26% (kappa 0.30, 95% CI 0.04-0.57). There was a good agreement for nodal (kappa 0.78, 95% CI 0.73-0.84) and extra-nodal staging (kappa 0.60, 95% CI 0.41-0.78) between WB-MRI reads CONCLUSIONS: WB-MRI has reasonable accuracy for nodal and extra-nodal staging but is discordant with standard imaging in a substantial minority of patients, and tends to underestimate disease response. KEY POINTS: ⢠This prospective single-centre study showed discordance for full patient staging of 44% between WB-MRI and a multi-modality reference standard in paediatric and adolescent Hodgkin's lymphoma. ⢠WB-MRI underestimates interim disease response in paediatric and adolescent Hodgkin's lymphoma. ⢠WB-MRI shows promise in paediatric and adolescent Hodgkin's lymphoma but currently cannot replace conventional staging pathways including 18F-FDG-PET-CT.
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Enfermedad de Hodgkin/diagnóstico por imagen , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Niño , Preescolar , Femenino , Fluorodesoxiglucosa F18 , Enfermedad de Hodgkin/tratamiento farmacológico , Enfermedad de Hodgkin/patología , Humanos , Imagen por Resonancia Magnética/métodos , Masculino , Estadificación de Neoplasias , Variaciones Dependientes del Observador , Tomografía Computarizada por Tomografía de Emisión de Positrones/métodos , Tomografía de Emisión de Positrones/métodos , Estudios Prospectivos , Radiofármacos , Estándares de Referencia , Imagen de Cuerpo Entero/métodos , Adulto JovenRESUMEN
Nephropathic cystinosis is an autosomal recessive lysosomal storage disorder in which intracellular cystine accumulates. It is caused by mutations in the CTNS gene. Clinical manifestations include renal tubular Fanconi syndrome in the first year of life, rickets, hypokalaemia, polyuria, dehydration and acidosis, growth retardation, hypothyroidism, photophobia and renal glomerular deterioration. Late complications include myopathy, pancreatic insufficiency and retinal blindness. Skeletal manifestations described in these patients include failure to thrive, osteomalacia, rickets and short stature. This paper describes progressive bony abnormalities in three unrelated patients with nephropathic cystinosis that have not been reported previously.
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The International Association for the Study of Lung Cancer proposed changes to the 7th edition of the Tumor, Node, and Metastasis (TNM) staging manual of non-small cell lung cancer (NSCLC) to improve the prognostic relevance of its descriptors. These changes include the subdivision of T1 and T2 disease according to size cut points; reassignment of the T and M categories of same-lobe, ipsilateral, and contralateral malignant pulmonary nodules; reassignment of pleural disease to metastatic disease; and introduction of intra- and extrathoracic metastatic disease. Because of movement between T and M descriptors and resultant stage migration, new stage groupings that contain TNM subsets different from those of the previous edition were created. The new staging classification was created on the basis of statistical analysis of a large international database of cases of NSCLC. The new classification has many advantages; however, limitations remain. Problems with routine radiologic staging of NSCLC have not been addressed, the varied survival rates for patients with the different histologic subtypes is not reflected, the new classification is not compatible with the previous system, and application of treatment algorithms on the basis of evidence from the previous edition is less clear.
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Carcinoma de Pulmón de Células no Pequeñas/patología , Neoplasias Pulmonares/patología , Estadificación de Neoplasias , Anciano , Carcinoma de Pulmón de Células no Pequeñas/clasificación , Carcinoma de Pulmón de Células no Pequeñas/diagnóstico por imagen , Femenino , Humanos , Neoplasias Pulmonares/clasificación , Neoplasias Pulmonares/diagnóstico por imagen , Masculino , RadiografíaRESUMEN
To investigate the diagnostic value of different whole-body magnetic resonance imaging (WB-MRI) protocols for staging Hodgkin and diffuse-large B-cell lymphomas (HL and DLBCL), twenty-two patients (M/F 12/10, median age 32, range 22-87, HL/DLBCL 14/8) underwent baseline WB-MRI and 18F-2-fluoro-2-deoxy-D-glucose (18F-FDG) positron emission tomography (PET) fused with computed tomography (CT) scan 18F-FDG-PET-CT. The 3.0 T WB-MRI was performed using pre-contrast modified Dixon (mDixon), T2-weighted turbo-spin-echo (TSE), diffusion-weighted-imaging (DWI), dynamic-contrast-enhanced (DCE) liver/spleen, contrast-enhanced (CE) lung MRI and CE whole-body mDixon. WB-MRI scans were divided into: (1) "WB-MRI DWI+IP": whole-body DWI + in-phase mDixon (2) "WB-MRI T2-TSE": whole-body T2-TSE (3) "WB-MRI Post-C": whole-body CE mDixon + DCE liver/spleen and CE lung mDixon (4) "WB-MRI All ": the entire protocol. Two radiologists evaluated WB-MRIs at random, independently and then in consensus. Two nuclear-medicine-physicians reviewed 18F-FDG PET-CT in consensus. An enhanced-reference-standard (ERS) was derived using all available baseline and follow-up imaging. The sensitivity and specificity of WB-MRI protocols for nodal and extra-nodal staging was derived against the ERS. Agreement between the WB-MRI protocols and the ERS for overall staging was assessed using kappa statistic. For consensus WB-MRI, the sensitivity and specificity for nodal staging were 75%, 98% for WB-MRI DWI+IP, 76%, 98% for WB-MRI Post-C, 83%, 99% for WB-MRI T2-TSE and 87%, 100% for WB-MRI All. The sensitivity and specificity for extra-nodal staging were 67% 100% for WB-MRI DWI+IP, 89%, 100% for WB-MRI Post-C, 89%, 100% for WB-MRI T2-TSE and 100%, 100% for the WB-MRI All. The consensus WB-MRI All read had perfect agreement with the ERS for overall staging [kappa = 1.00 (95% CI: 1.00-1.00)]. The best diagnostic performance is achieved combining all available WB-MRI sequences.
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Segmentation of the placenta from fetal MRI is challenging due to sparse acquisition, inter-slice motion, and the widely varying position and shape of the placenta between pregnant women. We propose a minimally interactive framework that combines multiple volumes acquired in different views to obtain accurate segmentation of the placenta. In the first phase, a minimally interactive slice-by-slice propagation method called Slic-Seg is used to obtain an initial segmentation from a single motion-corrupted sparse volume image. It combines high-level features, online Random Forests and Conditional Random Fields, and only needs user interactions in a single slice. In the second phase, to take advantage of the complementary resolution in multiple volumes acquired in different views, we further propose a probability-based 4D Graph Cuts method to refine the initial segmentations using inter-slice and inter-image consistency. We used our minimally interactive framework to examine the placentas of 16 mid-gestation patients from MRI acquired in axial and sagittal views respectively. The results show the proposed method has 1) a good performance even in cases where sparse scribbles provided by the user lead to poor results with the competitive propagation approaches; 2) a good interactivity with low intra- and inter-operator variability; 3) higher accuracy than state-of-the-art interactive segmentation methods; and 4) an improved accuracy due to the co-segmentation based refinement, which outperforms single volume or intensity-based Graph Cuts.