Enhancement patterns of adrenal nodules on magnetic resonance imaging.

OBJECTIVE: To compare enhancement patterns of typical adrenal adenomas, lipid-poor adenomas, and non-adenomas on magnetic resonance imaging (MRI). MATERIALS AND METHODS: Evaluation of adrenal nodules larger than 1.0 cm, with at least 2-year follow-up, evaluated on MRI in January 2007 and December 2016. Two different protocols were included - upper abdomen MRI (delayed phase after 3 minutes) and abdomen and pelvis MRI (delayed phase after 7 minutes) - and nodules were divided in typical adenomas (characterized on out-of-phase MRI sequence), lipid-poor adenomas (based on follow-up imaging stability) and non-adenomas (based on pathological finding or follow-up imaging). T2-weighted and enhancement features were analyzed (absolute and relative washout and enhancement curve pattern), similarly to classic computed tomography equations. RESULTS: Final cohort was composed of 123 nodules in 116 patients (mean diameter of 1.8 cm and mean follow up time of 4 years and 3 months). Of them, 98 (79%) nodules had features of typical adenomas by quantitative chemical shift imaging, and demonstrated type 3 curve pattern in 77%, mean absolute and relative washout of 29% and 16%, respectively. Size, oncologic history and T2-weighted features showed statistically significant differences among groups. Also, a threshold greater than 11.75% for absolute washout on MRI achieved sensitivity of 71.4% and specificity of 70.0%, in differentiating typical adenomas from non-adenomas. CONCLUSION: Calculating absolute washout of adrenal nodules on MRI may help identifying proportion of non-adenomas.

Role of MRI in Staging of Penile Cancer.

Penile cancer is one of the male-specific cancers. Accurate pretreatment staging is crucial due to a plethora of treatment options currently available. The 8th edition American Joint Committee on Cancer-Tumor Node and Metastasis (AJCC-TNM) revised the staging for penile cancers, with invasion of corpora cavernosa upstaged from T2 to T3 and invasion of urethra downstaged from T3 to being not separately relevant. With this revision, MRI is more relevant in local staging because MRI is accurate in identifying invasion of corpora cavernosa, while the accuracy is lower for detection of urethral involvement. The recent European Urology Association (EAU) guidelines recommend MRI to exclude invasion of the corpora cavernosa, especially if penis preservation is planned. Identification of satellite lesions and measurement of residual-penile-length help in surgical planning. When nonsurgical treatment modalities of the primary tumor are being considered, accurate local staging helps in decision-making regarding upfront inguinal lymph node dissection as against surveillance. MRI helps in detection and extent of inguinal and pelvic lymphadenopathy and is superior to clinical palpation, which continues to be the current approach recommended by National Comprehensive Cancer Network (NCCN) treatment guidelines. MRI helps the detection of "bulky" lymph nodes that warrant neoadjuvant chemotherapy and potentially identify extranodal extension. However, tumor involvement in small lymph nodes and differentiation of reactive vs. malignant lymphadenopathy in large lymph nodes continue to be challenging and the utilization of alternative contrast agents (superparamagnetic iron oxide), positron emission tomography (PET)-MRI along with texture analysis is promising. In locally recurrent tumors, MRI is invaluable in identification of deep invasion, which forms the basis of treatment. Multiparametric MRI, especially diffusion-weighted-imaging, may allow for quantitative noninvasive assessment of tumor grade and histologic subtyping to avoid biopsy undersampling. Further research is required for incorporation of MRI with deep learning and artificial intelligence algorithms for effective staging in penile cancer. Level of Evidence: 5 Technical Efficacy: Stage 3 J. Magn. Reson. Imaging 2020;51:1612-1629.

Multicenter Multireader Evaluation of an Artificial Intelligence-Based Attention Mapping System for the Detection of Prostate Cancer With Multiparametric MRI.

OBJECTIVE. The purpose of this study was to evaluate in a multicenter dataset the performance of an artificial intelligence (AI) detection system with attention mapping compared with multiparametric MRI (mpMRI) interpretation in the detection of prostate cancer. MATERIALS AND METHODS. MRI examinations from five institutions were included in this study and were evaluated by nine readers. In the first round, readers evaluated mpMRI studies using the Prostate Imaging Reporting and Data System version 2. After 4 weeks, images were again presented to readers along with the AI-based detection system output. Readers accepted or rejected lesions within four AI-generated attention map boxes. Additional lesions outside of boxes were excluded from detection and categorization. The performances of readers using the mpMRI-only and AI-assisted approaches were compared. RESULTS. The study population included 152 case patients and 84 control patients with 274 pathologically proven cancer lesions. The lesion-based AUC was 74.9% for MRI and 77.5% for AI with no significant difference (p = 0.095). The sensitivity for overall detection of cancer lesions was higher for AI than for mpMRI but did not reach statistical significance (57.4% vs 53.6%, p = 0.073). However, for transition zone lesions, sensitivity was higher for AI than for MRI (61.8% vs 50.8%, p = 0.001). Reading time was longer for AI than for MRI (4.66 vs 4.03 minutes, p < 0.001). There was moderate interreader agreement for AI and MRI with no significant difference (58.7% vs 58.5%, p = 0.966). CONCLUSION. Overall sensitivity was only minimally improved by use of the AI system. Significant improvement was achieved, however, in the detection of transition zone lesions with use of the AI system at the cost of a mean of 40 seconds of additional reading time.

Magnetic Resonance Imaging Evaluation of Left Common Iliac Vein Compression in Patients With and Without Symptoms of Venous Disease.

BACKGROUND: The goal of this study was to assess the degree of the left common iliac vein (LCIV) compression by the right common iliac artery (RCIA) on magnetic resonance imaging (MRI), and verify differences in compression measurements in end-inspiration, end-expiration, supine, and prone decubitus between patients with and without symptoms in the lower limbs.Methods and Results:A total of 82 consecutive participants provided informed consent and underwent pelvic MRI for different clinical indications other than vascular-related disorders. The participants answered a questionnaire about venous disease in the lower limbs and history of previous deep vein thrombosis. This study measured the area and diameter of the LCIV at the site of the crossing with the RCIA and in the uncompressed caudal LCIV segment. Statistical analysis was performed to compare the degree of LCIV compression by the RCIA and verify differences in compression measurements. A total of 71 participants were included; 46.6% were in group A and did not experience signs and symptoms in lower limbs and 53.6% were in group B and answered at least one question with a positive answer. It was observed that there was a statistically significant difference between groups for end-expiration. No statistically significant differences were observed for all other measurements. CONCLUSIONS: A substantial percentage of the asymptomatic and symptomatic population showed compression of the LCIV, suggesting there are no clear relationships between stenosis and non-specific symptomatology in the general population.

Comparison of Radiation Dose and Image Quality of Abdominopelvic CT Using Iterative (AIDR 3D) and Conventional Reconstructions.

OBJECTIVE: The purpose of this study is to compare radiation dose and image quality of abdominopelvic CT studies reconstructed with iterative and conventional techniques. MATERIALS AND METHODS: This retrospective study enrolled 99 patients who underwent abdominopelvic CT examinations with the portal venous phase images reconstructed with both filtered back projection and Adaptive Iterative Dose Reduction 3D (AIDR 3D) at different time points. Subjective assessment of image quality was performed by two radiologists who scored axial images for overall quality, sharpness, noise, and acceptability in a blinded fashion. The SD of the mean attenuation of the liver, aorta, and paraspinal muscle (as a measurement of image noise) and contrast-to-noise and signal-to-noise ratios for liver and aorta were used as objective parameters of image quality. Radiation dose parameters included CT dose index volume (CTDIvol), dose-length product, effective dose (ED), and size-specific dose estimate (SSDE). Results were compared for different body mass index (BMI; weight in kilograms divided by the square of height in meters) categories. Paired t test and McNemar paired tests for noninferiority were used, with p < 0.05 considered statistically significant. RESULTS: We obtained a 62.5% mean reduction in CTDIvol, a 58% mean reduction in ED, and a 63% mean reduction in SSDE when AIDR 3D was used (p < 0.001). Subjective parameters of image quality were considered noninferior for AIDR 3D studies compared with filtered back projection (p < 0.001), except for the sharpness of images of patients with BMI 20-24.9. Variable results were found regarding objective assessment of image quality. CONCLUSION: AIDR 3D allowed a significant reduction in radiation dose of abdominopelvic CT examinations without a loss of image quality in general.

Objective value on Apparent diffusion coefficient (ADC) map to categorize the intensity of diffusion-weighted imaging (DWI) restriction for prostate cancer detection on multiparametric prostate MRI.

PURPOSE: To identify objective and subjective criteria on multiparametric prostate MRI that can be helpful for prostate cancer detection. MATERIALS AND METHODS: Retrospective study, IRB approved, including 122 patients who had suspicious lesion on MRI and who underwent prostate biopsy with ultrasonography (US)/MRI imaging fusion. There were 60 patients with positive biopsies and 62 with negative biopsies. MRI of these patients were randomized and evaluated independently by two blinded radiologists. The following variables were analyzed in each lesion: morphology, contours, T2 signal, diffusion restriction (subjective impression and objective values), hyper-enhancement, contact with transition zone or prostatic contour, prostatic contour retraction, Likert and PIRADS classification. RESULTS: Apparent diffusion coefficient (ADC) value was the best predictor of positivity for prostate cancer, with mean value of 1.08 (SD 0.20) and 1.09 mm2/sec (SD 0.24) on negative biopsies and 0.81 (SD 0.22) and 0.84 mm2/sec (SD 0.22) on positive biopsies for readers 1 and 2, respectively (p < 0.001 in both analysis). For the others categorical variables evaluated the best AUC for reader 1 was subjective intensity of diffusion restriction (AUC of 0.74) and for reader 2 was hyper-enhancement (AUC of 0.65), all inferior comparing to the value of ADC map. Interobserver agreement ranged from 0.13 to 0.75, poor in most measurements, and good or excellent (kappa > 0.6) only in lesion size and ADC values. CONCLUSIONS: Diffusion restriction with lower ADC-values is the best parameter to predict cancer on MRI prior to biopsy. Efforts to establish an ADC cutoff value would improve cancer detection, especially for less experience reader.

Comparison between multiparametric MRI with and without post - contrast sequences for clinically significant prostate cancer detection.

BACKGROUND: Dynamic-contrast enhanced (DCE) sequence is used to increase detection of small lesions, based on increased vascularization. However, literature is controversy about the real incremental value of DCE in detection of clinically significant (CS) prostate cancer (PCa), since absence of enhancement does not exclude cancer, and enhancement alone is not definitive for tumor. PURPOSE: To test the hypothesis that DCE images do not increase CS PCa detection on MRI prior to biopsy, comparing exams without and with contrast sequences. Material and Materials and Methods: All men who come to our institution to perform MRI on a 3T scanner without a prior diagnosis of CS PCa were invited to participate in this study. Reference standard was transrectal prostate US with systematic biopsy and MRI/US fusion biopsy of suspicious areas. Radiologists read the MRI images prospectively and independently (first only sequences without contrast, and subsequently the entire exam) and graded them on 5-points scale of cancer suspicion. RESULTS: 102 patients were included. Overall detection on biopsy showed CS cancer in 43 patients (42.2%), clinically non-significant cancer in 11 (10.8%) and negative results in 48 patients (47%). Positivities for CS PCa ranged from 8.9% to 9.8% for low suspicion and 75.0% to 88.9% for very high suspicion. There was no statistical difference regarding detection of CS PCa (no statistical difference was found when compared accuracies, sensitivities, specificities, PPV and NPV in both types of exams). Inter-reader agreement was 0.59. CONCLUSION: Exams with and without contrast-enhanced sequences were similar for detection of CS PCa on MRI.

Comparison of Gleason upgrading rates in transrectal ultrasound systematic random biopsies versus US-MRI fusion biopsies for prostate cancer.

PURPOSE: Ultrasound-magnetic resonance imaging (US-MRI) fusion biopsy (FB) improves the detection of clinically significant prostate cancer (PCa). We aimed to compare the Gleason upgrading (GU) rates and the concordance of the Gleason scores in the biopsy versus final pathology after surgery in patients who underwent transrectal ultrasound (TRUS) systematic random biopsies (SRB) versus US-MRI FB for PCa. MATERIALS AND METHODS: A retrospective analysis of data that were collected prospectively from January 2011 to June 2016 from patients who underwent prostate biopsy and subsequent radical prostatectomy. The study cohort was divided into two groups: US-MRI FB (Group A) and TRUS SRB (Group B). US-MRI FB was performed in patients with a previous MRI with a focal lesion with a Likert score ≥3; otherwise, a TRUS SRB was performed. RESULTS: In total, 73 men underwent US-MRI FB, and 89 underwent TRUS SRB. The GU rate was higher in Group B (31.5% vs. 16.4%; p=0.027). According to the Gleason grade pattern, GU was higher in Group B than in Group A (40.4% vs. 23.3%; p=0.020). Analyses of the Gleason grading patterns showed that Gleason scores 3+4 presented less GU in Group A (24.1% vs. 52.6%; p=0.043). The Bland-Altman plot analysis showed a higher bias in Group B than in Group A (-0.27 [-1.40 to 0.86] vs. -0.01 [-1.42 to 1.39]). In the multivariable logistic regression analysis, the only independent predictor of GU was the use of TRUS SRB (2.64 [1.11 - 6.28]; p=0.024). CONCLUSIONS: US-MRI FB appears to be related to a decrease in GU rate and an increase in concordance between biopsy and final pathology compared to TRUS SRB, suggesting that performing US-MRI FB leads to greater accuracy of diagnosis and better treatment decisions.

Anastomosing hemangioma simulating renal cell carcinoma.

The anastomosing hemangioma is a recent described rare variant, which histologically simulates an angiosarcoma and occurs primarily in the genitourinary tract. We present a case of renal anastomosing hemangioma from a radiologic perspective, describing its imaging features and reviewing its presentation and management.

Prostate cancer detection using multiparametric 3 - tesla MRI and fusion biopsy: preliminary results.

OBJECTIVE: To evaluate the diagnostic efficacy of transrectal ultrasonography (US) bi¬opsy with imaging fusion using multiparametric (mp) magnetic resonance imaging (MRI) in patients with suspicion of prostate cancer (PCa), with an emphasis on clini¬cally significant tumors according to histological criteria. MATERIALS AND METHODS: A total of 189 consecutive US/MRI fusion biopsies were per¬formed obtaining systematic and guided samples of suspicious areas on mpMRI using a 3 Tesla magnet without endorectal coil. Clinical significance for prostate cancer was established based on Epstein criteria. RESULTS: In our casuistic, the average Gleason score was 7 and the average PSA was 5.0ng/mL. Of the 189 patients that received US/MRI biopsies, 110 (58.2%) were positive for PCa. Of those cases, 88 (80%) were clinically significant, accounting for 46.6% of all patients. We divided the MRI findings into 5 Likert scales of probability of having clinically significant PCa. The positivity of US/MRI biopsy for clinically significant PCa was 0%, 17.6% 23.5%, 53.4% and 84.4% for Likert scores 1, 2, 3, 4 and 5, respectively. There was a statistically significant difference in terms of biopsy results between dif¬ferent levels of suspicion on mpMRI and also when biopsy results were divided into groups of clinically non-significant versus clinically significant between different lev¬els of suspicion on mpMRI (p-value < 0.05 in both analyzes). CONCLUSION: We found that there is a significant difference in cancer detection using US/MRI fusion biopsy between low-probability and intermediate/high probability Likert scores using mpMRI.

Spectrum of abdominal imaging findings in histiocytic disorders.

OBJECTIVES: The present article provides an overview of the spectrum of abdominal findings of histiocytic disorders that may be observed in multimodality imaging illustrated by clinical cases from our Imaging Center. METHODS: We will review abdominal findings of Langerhans cell histiocytosis, Rosai-Dorfman disease, Erdheim-Chester disease, and hemophagocytic syndrome illustrated by clinical cases from our imaging department with histologic correlation. RESULTS: Abdominal involvement of histiocytic disorders is rare and may occur in the liver, biliary tract, kidney, retroperitoneum, kidney, gastrointestinal tract, and lymph nodes. CONCLUSION: Histiocytic disorders encompass a group of rare diseases with a wide range of manifestations in which the abdominal involvement is quite infrequent. The role of the radiologist is to report the major imaging findings and the differential diagnosis; however, the imaging features are unspecific and biopsy usually is necessary to establish the definitive diagnosis.

The skin-to-calyx distance measured by renal ct scan and ultrasound.

PURPOSE: We developed a stereotactic device to guide the puncture for percutaneous nephrolithotripsy, which uses the distance from the target calyx to its perpendicular point on skin (SCD) to calculate the needle´s entry angle. This study seeks to validate the use of measurements obtained by ultrasound (US) and computerized tomography (CT) for needle´s entry angle calculation and to study factors that may interfere in this procedure. MATERIALS AND METHODS: Height, weight, abdominal circumference, CT of the urinary tract in dorsal decubitus (DD) and ventral decubitus (VD), and US of the kidneys in VD were obtained from thirty-five renal calculi patients. SCD obtained were compared and correlated with body-mass index (BMI). RESULTS: BMI was 28.66 ± 4.6 Kg/m2. SCD on CT in DD was 8.40 ± 2.06cm, in VD was 8.32 ± 1.95cm, in US was 6.74 ± 1.68cm. SCD measured by US and CT were statistically different (p < 0.001), whereas between CT in DD and VD were not. SCD of the lower calyx presented moderate correlation with BMI. CONCLUSION: SCD obtained by CT in ventral and dorsal decubitus may be used for calculation of the needle´s entry angle. SCD obtained by US cannot be used. A rule for the correlation between BMI and the SCD could not be determined.

Strategies for improving image quality in prostate MRI.

Prostate magnetic resonance imaging (MRI) stands as the cornerstone in diagnosing prostate cancer (PCa), offering superior detection capabilities while minimizing unnecessary biopsies. Despite its critical role, global disparities in MRI diagnostic performance persist, stemming from variations in image quality and radiologist expertise. This manuscript reviews the challenges and strategies for enhancing image quality in prostate MRI, spanning patient preparation, MRI unit optimization, and radiology team engagement. Quality assurance (QA) and quality control (QC) processes are pivotal, emphasizing standardized protocols, meticulous patient evaluation, MRI unit workflow, and radiology team performance. Additionally, artificial intelligence (AI) advancements offer promising avenues for improving image quality and reducing acquisition times. The Prostate-Imaging Quality (PI-QUAL) scoring system emerges as a valuable tool for assessing MRI image quality. A comprehensive approach addressing technical, procedural, and interpretative aspects is essential to ensure consistent and reliable prostate MRI outcomes.

PRECISE Version 2: Updated Recommendations for Reporting Prostate Magnetic Resonance Imaging in Patients on Active Surveillance for Prostate Cancer.

BACKGROUND AND OBJECTIVE: The Prostate Cancer Radiological Estimation of Change in Sequential Evaluation (PRECISE) recommendations standardise the reporting of prostate magnetic resonance imaging (MRI) in patients on active surveillance (AS) for prostate cancer. An international consensus group recently updated these recommendations and identified the areas of uncertainty. METHODS: A panel of 38 experts used the formal RAND/UCLA Appropriateness Method consensus methodology. Panellists scored 193 statements using a 1-9 agreement scale, where 9 means full agreement. A summary of agreement, uncertainty, or disagreement (derived from the group median score) and consensus (determined using the Interpercentile Range Adjusted for Symmetry method) was calculated for each statement and presented for discussion before individual rescoring. KEY FINDINGS AND LIMITATIONS: Participants agreed that MRI scans must meet a minimum image quality standard (median 9) or be given a score of 'X' for insufficient quality. The current scan should be compared with both baseline and previous scans (median 9), with the PRECISE score being the maximum from any lesion (median 8). PRECISE 3 (stable MRI) was subdivided into 3-V (visible) and 3-NonV (nonvisible) disease (median 9). Prostate Imaging Reporting and Data System/Likert ≥3 lesions should be measured on T2-weighted imaging, using other sequences to aid in the identification (median 8), and whenever possible, reported pictorially (diagrams, screenshots, or contours; median 9). There was no consensus on how to measure tumour size. More research is needed to determine a significant size increase (median 9). PRECISE 5 was clarified as progression to stage ≥T3a (median 9). CONCLUSIONS AND CLINICAL IMPLICATIONS: The updated PRECISE recommendations reflect expert consensus opinion on minimal standards and reporting criteria for prostate MRI in AS. PATIENT SUMMARY: The Prostate Cancer Radiological Estimation of Change in Sequential Evaluation (PRECISE) recommendations are used in clinical practice and research to guide the interpretation and reporting of magnetic resonance imaging for patients on active surveillance for prostate cancer. An international panel has updated these recommendations, clarified the areas of uncertainty, and highlighted the areas for further research.

Quality assessment of prostate MRI by PI-QUAL score: Inter-reader agreement and impact on prostate cancer local staging at 3 Tesla.

PURPOSE: To evaluate whether the Prostate Imaging Quality (PI-QUAL) score impacts prostate cancer (PCa) staging on MRI. The secondary goal was to test inter-reader agreement among radiologists experienced in prostate imaging. METHOD: A retrospective, single-center study with patients who underwent 3 Tesla prostate MRI scans and were submitted to radical prostatectomy (RP) between January 2018 and November 2021 and were eligible for our study. Extraprostatic extension (EPE) data were collected from original MR reports (EPEm) and pathological reports of RP specimens (EPEp). Three expert prostate radiologists (ESUR/ESUI criteria R1, R2, R3) independently evaluated all MRI exams according to PI-QUAL score for image quality (1 to 5; 1: poor, 5: excellent), blinded to original imaging reports and clinical data. We studied the diagnostic performance of MRI using pooled data from PI-QUAL scores (≤3 vs. ≥4). We also performed univariate and multivariate analyses to assess the PI-QUAL score impact on local PCa staging. Cohen's K and Tau-b Kendall tests were used to assess the inter-reader agreement for PI-QUAL score, T2WI, DWI, and DCE. RESULTS: Our final cohort included 146 patients, of which 27.4% presented EPE on pathology. We observed no impact of imaging quality on accuracy for EPE prediction: AUC of 0.750 (95% CI 0.26-1) for PI-QUAL ≤ 3 and 0.705 (95% CI 0.618-0.793) for PI-QUAL ≥ 4. The multivariate analysis demonstrated a correlation of EPEm (OR 3.25, p 0.001) and ISUP grade group (OR 1.89, p 0.012) to predict EPEp. The inter-reader agreement was moderate to substantial (0.539 for R1-R2, 0.522 for R2-R3, and 0.694 for R1-R3). CONCLUSION: Our clinical impact evaluation showed no direct correlation between MRI quality by PI-QUAL score and accuracy in detecting EPE in patients undergoing RP. Additionally, we had moderate to a substantial inter-reader agreement for the PI-QUAL score.

Musculoskeletal pitfalls in 68Ga-PSMA PET/CT.

Prostate-specific membrane antigen (PSMA) is a transmembrane protein expressed in normal prostate cells and overexpressed in prostate cancer. Consequently, it is an important tool in the evaluation of prostate cancer, including the staging of high-risk patients and the assessment of biochemical recurrence. Despite the "specific" designation, benign musculoskeletal conditions, such as fractures, osteodegenerative changes, and fibrous dysplasia, can also show PSMA uptake, which can lead to misinterpretation of the imaging findings. Therefore, radiologists must be aware of these potential pitfalls, understand their causes, and fully analyze their morphologic features on unfused computed tomography (CT) and magnetic resonance imaging scans to correctly interpret the examination. In this pictorial essay, we review the basic characteristics of the 68Ga-PSMA positron-emission tomography/CT (PET/CT) radiotracer, discuss potential causes of false-positive findings on 68Ga-PSMA PET/CT in the musculoskeletal system, and illustrate the corresponding imaging findings.

O antígeno de membrana próstata específico (PSMA) é uma proteína transmembrana que apresenta expressão em células prostáticas normais e superexpressão em neoplasia da próstata. Dessa forma, é uma importante ferramenta na avaliação da neoplasia prostática, de utilidade no estadiamento de pacientes de alto risco e na análise de recorrência bioquímica. Apesar do termo "específico", condições musculoesqueléticas benignas podem demonstrar captação de PSMA, como fraturas, alterações osteodegenerativas e displasia fibrosa, podendo levar a uma avaliação equivocada dos achados de imagem. Assim, o radiologista deve conhecer esses potenciais pitfalls, compreender suas causas e analisar as características morfológicas nas imagens não fundidas de TC e RM para interpretar corretamente o exame. Neste ensaio iconográfico, revisaremos as características básicas do radiofármaco 68Ga-PSMA PET/CT, discutiremos possíveis causas de resultados falso-positivos na 68Ga-PSMA PET/CT no sistema musculoesquelético e ilustraremos os achados de imagem correspondentes.

Extramedullary haematopoiesis in patients with thalassemia: a cross-sectional description of its prevalence, clinical features and survival.

INTRODUCTION: Despite knowledge advances on extramedullary haematopoiesis (EMH) in thalassemic patients, the real picture remains an open issue. OBJECTIVES: To assess EMH prevalence in patients with thalassemia major (TM) and intermedia (TI), to describe magnetic resonance imaging (MRI) findings and to explore clinical risk factors. METHODS: In this cross-sectional study, images and clinical records of 184 consecutive patients with thalassemia who underwent T2* MRI between 2004 and 2011 were reviewed. Association of EMH with survival was investigated for patients with available follow-up charts. RESULTS: EMH was detected in 16/168 (9.5%) patients with TM (aged 19-49 years) and in 3/16 (18.8%) with TI (aged 36-41 years). Most (88%) had paravertebral thoracic and/or abdominal masses. Age was significantly associated with EMH risk (hazard ratio, [HR] 1.10/year; confidence interval [CI]: 1.03-1.18; p-value < 0.001), while lower pancreatic iron content by T2*MRI (HR: 0.94/ms; CI: 0.89-0.99; p-value = 0.049) was a protective factor. Estimated survival rate was superior for EMH-positive (n = 19) when compared to EMH-negative patients (n = 75) (p-value = 0.013). CONCLUSIONS: The prevalence of EMH was 10.3% (19/184), presented mainly as tumoral masses of 3 to 10 cm. Age was a risk factor for EMH development, while lower pancreatic iron might be a protective factor in this cohort.

Cardiac iron overload evaluation in thalassaemic patients using T2* magnetic resonance imaging following chelation therapy: a multicentre cross-sectional study.

INTRODUCTION: Magnetic resonance imaging (MRI) T2* technique is used to assess iron overload in the heart, liver and pancreas of thalassaemic patients. Optimal iron chelation and expected tissue iron response rates remain under investigation. The objective of this study was to analyse serum ferritin and the iron concentration in the heart, liver and pancreas measured by MRI T2*/R2* during regular chelation therapy in a real-world cohort of patients with thalassemia. METHODS: We evaluated thalassaemic patients ≥ 7 years old undergoing chelation/transfusion therapy by MRI and assessed serum ferritin at baseline and follow-up from 2004-2011. RESULTS: We evaluated 136 patients, 92% major thalassaemic, with a median age of 18 years, and median baseline ferritin 2.033ng/ml (range: 59-14,123). Iron overload distribution was: liver (99%), pancreas (74%) and heart (36%). After a median of 1.2 years of follow-up, the iron overload in the myocardium reduced from 2,63 Fe mg/g to 2,05 (p 0.003). The optimal R2* pancreas cut-off was 148 Hertz, achieving 78% sensitivity and 73% specificity. However, when combining the R2* pancreas cut off ≤ 50 Hertz and a ferritin ≤ 1222 ng/ml, we could reach a negative predictive value (NPV) of 98% for cardiac siderosis. Only 28% were undergoing combined chelation at baseline assessment, which increased up to 50% on follow up evaluation. CONCLUSIONS: Chelation therapy significantly reduced cardiac siderosis in thalassaemic patients. In patients with moderate/severe liver iron concentration undergoing chelation therapy, ferritin levels and myocardium iron improved earlier than the liver siderosis.