Publications by authors named "Jamie Marko"

29 Publications

  • Page 1 of 1

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

AJR Am J Roentgenol 2020 10 5;215(4):903-912. Epub 2020 Aug 5.

Department of Radiology and Imaging Sciences, Clinical Center, National Institutes of Health, Bethesda, MD.

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. 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. 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 ( = 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%, = 0.073). However, for transition zone lesions, sensitivity was higher for AI than for MRI (61.8% vs 50.8%, = 0.001). Reading time was longer for AI than for MRI (4.66 vs 4.03 minutes, < 0.001). There was moderate interreader agreement for AI and MRI with no significant difference (58.7% vs 58.5%, = 0.966). 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.
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http://dx.doi.org/10.2214/AJR.19.22573DOI Listing
October 2020

APECED-Associated Hepatitis: Clinical, Biochemical, Histological and Treatment Data From a Large, Predominantly American Cohort.

Hepatology 2021 Mar;73(3):1088-1104

Translational, Hepatology Section, Liver Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD.

Background And Aims: Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED), caused by autoimmune regulator (AIRE) mutations, manifests with chronic mucocutaneous candidiasis (CMC) and multisystem autoimmunity, most often hypoparathyroidism (HP) and adrenal insufficiency (AI). European cohorts previously reported a ~10% prevalence of APECED-associated hepatitis (APAH) with presentations ranging from asymptomatic laboratory derangements to fatal fulminant hepatic failure. Herein, we characterized APAH in a large APECED cohort from the Americas.

Approach And Results: Forty-five consecutive patients with APECED were evaluated (2013-2015) at the National Institutes of Health (NIH; NCT01386437). Hepatology consultation assessed hepatic and autoimmune biomarkers and liver ultrasound in all patients. Liver biopsies evaluated autoimmune features and fibrosis. The 16S ribosomal RNA (rRNA) sequencing was performed in 35 patients' stools (12 with and 23 without APAH). Among 43 evaluable patients, 18 (42%) had APAH; in 33.3% of those with APAH, APAH occurred before developing classic APECED diagnostic criteria. At APAH diagnosis, the median age was 7.8 years, and patients manifested with aminotransferase elevation and/or hyperbilirubinemia. All patients with APAH were in clinical remission during their NIH evaluation while receiving immunomodulatory treatment. We found no difference in age, sex, or prevalence of CMC, AI, or HP between patients with or without APAH. Autoantibody positivity against aromatic L-amino acid decarboxylase, cytochrome P450 family 1 subfamily A member 2, histidine decarboxylase (HDC), bactericidal/permeability-increasing fold-containing B1, tryptophan hydroxlase, and 21-hydroxylase (21-OH), and the homozygous c.967_979del13 AIRE mutation were associated with APAH development. Classical serological biomarkers of autoimmune hepatitis (AIH) were only sporadically positive. AIH-like lymphoplasmacytic inflammation with mild fibrosis was the predominant histological feature. Stool microbiome analysis found Slackia and Acidaminococcus in greater abundance in patients with APAH.

Conclusions: APAH is more common than previously described, may present early before classic APECED manifestations, and most often manifests with milder, treatment-responsive disease. Several APECED-associated autoantibodies, but not standard AIH-associated biomarkers, correlate with APAH.
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http://dx.doi.org/10.1002/hep.31421DOI Listing
March 2021

ACR Appropriateness Criteria® Hematuria.

J Am Coll Radiol 2020 May;17(5S):S138-S147

Specialty Chair, University of Alabama at Birmingham, Birmingham, Alabama.

Hematuria is a common reason for patients to be referred for imaging of the urinary tract. All patients diagnosed with hematuria should undergo a thorough history and physical examination, urinalysis, and serologic testing prior to any initial imaging. Ultrasound, CT, and MRI are the most common imaging modalities used to evaluate hematuria. This document discusses the following clinical scenarios for hematuria: initial imaging of microhematuria without risk factors or history of recent vigorous exercise, or presence of infection, or viral illness, or present or recent menstruation; initial imaging of microhematuria in patients with known risk factors and no history of recent vigorous exercise, or presence of infection, or viral illness, or present or recent menstruation or renal parenchymal disease; initial imaging of microhematuria in the pregnant patient and initial imaging of gross hematuria. Follow-up of normal or abnormal findings is beyond the scope of this review. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.
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http://dx.doi.org/10.1016/j.jacr.2020.01.028DOI Listing
May 2020

Neck masses due to internal jugular vein phlebectasia: Frequency in Menkes disease and literature review of 85 pediatric subjects.

Am J Med Genet A 2020 06 15;182(6):1364-1377. Epub 2020 Apr 15.

Section on Translational Neuroscience, Molecular Medicine Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, Bethesda, Maryland, USA.

Classic Menkes disease is a rare X-linked recessive disorder of copper metabolism caused by pathogenic variants in the copper transporter gene, ATP7A. Untreated affected individuals suffer failure to thrive and neurodevelopmental delays that begin at 6-8 weeks of age and progress inexorably to death, often within 3 years. Subcutaneous injections of Copper Histidinate (US Food and Drug Administration IND #34,166, Orphan product designation #12-3663) are associated with improved survival and neurological outcomes, especially when commenced within a month of birth. We previously identified internal jugular vein phlebectasia (IJP) in four Menkes disease subjects. This feature and other connective tissue abnormalities appear to be consequences of deficient activity of lysyl oxidase, a copper-dependent enzyme. Here, we report results from a prospective study of IJP based on 178 neck ultrasounds in 66 Menkes subjects obtained between November 2007 and March 2018. Nine patients met the criterion for IJP (one or more cross-sectional area measurements exceeding 2.2 cm ) and five subjects had clinically apparent neck masses that enlarged over time. Our prospective results suggest that IJP occurs in approximately 14% (9/66) of Menkes disease patients and appears to be clinically benign with no specific medical or surgical actionability. We surveyed the medical literature for prior reports of IJP in pediatric subjects and identified 85 individuals and reviewed the distribution of this abnormality by gender, sidedness, and underlying etiology. Taken together, Menkes disease accounts for 16% (15/94) of all reported IJP individuals. Neck masses from IJP represent underappreciated abnormalities in Menkes disease.
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http://dx.doi.org/10.1002/ajmg.a.61572DOI Listing
June 2020

Quality of Prostate MRI: Is the PI-RADS Standard Sufficient?

Acad Radiol 2021 02 4;28(2):199-207. Epub 2020 Mar 4.

Molecular Imaging Program, National Cancer Institute, NIH, Bethesda, MD, USA. Electronic address:

Rationale And Objective: The Prostate Imaging Reporting and Data System version 2 (PI-RADSv2) published a set of minimum technical standards (MTS) to improve image quality and reduce variability in multiparametric prostate MRI. The effect of PIRADSv2 MTS on image quality has not been validated. We aimed to determine whether adherence to PI-RADSv2 MTS improves study adequacy and perceived quality.

Materials And Methods: Sixty-two prostate MRI examinations including T2 weighted (T2W) and diffusion weighted image (DWI) consecutively referred to our center from 62 different institutions within a 12-month period (September 2017 to September 2018) were included. Six readers assessed images as adequate or inadequate for use in PCa detection and a numerical image quality ranking was given using a 1-5 scale. The PI-RADSv2 MTS were synthesized into sets of seven and 10 rules for T2W and DWI, respectively. Image adherence was assessed using Digital Imaging and Communications in Medicine (DICOM) metadata. Statistical analysis of survey results and image adherence was performed based on reader quality scoring (Kendall Rank tau-b) and reader adequate scoring (Wilcoxon test for association) for T2 and DWI quality assessment.

Results: Out of 62 images, 52 (83%) T2W and 38 (61%) DWIs were rated to be adequate by a majority of readers. Reader adequacy scores showed no significant association with adherence to PI-RADSv2. There was a weak (tau-b = 0.22) but significant (p value = 0.01) correlation between adherence to PIRADSv2 MTS and image quality for T2W. Studies following all PI-RADSv2 T2W rules achieved a higher median average quality score (3.58 for 7/7 vs. 3.0 for <7/7, p = 0.012). No statistical relationship with PI-RADSv2 MTS adherence and DWI quality was found.

Conclusion: Among 62 sites performing prostate MRI, few were considered of high quality, but the majority were considered adequate. DWI showed considerably lower rates of adequate studies in the sample. Adherence to PI-RADSv2 MTS did not increase the likelihood of having a qualitatively adequate T2W or DWI.
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http://dx.doi.org/10.1016/j.acra.2020.01.031DOI Listing
February 2021

Eligibility and Radiologic Assessment for Adjuvant Clinical Trials in Kidney Cancer.

JAMA Oncol 2020 Jan;6(1):133-141

Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland.

Purpose: To harmonize the eligibility criteria and radiologic disease assessment definitions in clinical trials of adjuvant therapy for renal cell carcinoma (RCC).

Method: On November 28, 2017, US-based experts in RCC clinical trials, including medical oncologists, urologic oncologists, regulators, biostatisticians, radiologists, and patient advocates, convened at a public workshop to discuss eligibility for trial entry and radiologic criteria for assessing disease recurrence in adjuvant trials in RCC. Multiple virtual meetings were conducted to address the issues identified at the workshop.

Results: The key workshop conclusions for adjuvant RCC therapy clinical trials were as follows. First, patients with non-clear cell RCC could be routinely included, preferably in an independent cohort. Second, patients with T3-4, N+M0, and microscopic R1 RCC tumors may gain the greatest advantages from adjuvant therapy. Third, trials of agents not excreted by the kidney should not exclude patients with severe renal insufficiency. Fourth, therapy can begin 4 to 16 weeks after the surgical procedure. Fifth, patients undergoing radical or partial nephrectomy should be equally eligible. Sixth, patients with microscopically positive soft tissue or vascular margins without gross residual or radiologic disease may be included in trials. Seventh, all suspicious regional lymph nodes should be fully resected. Eighth, computed tomography should be performed within 4 weeks before trial enrollment; for patients with renal insufficiency who cannot undergo computed tomography with contrast, noncontrast chest computed tomography and magnetic resonance imaging of the abdomen and pelvis with gadolinium should be performed. Ninth, when feasible, biopsy should be undertaken to identify any malignant disease. Tenth, when biopsy is not feasible, a uniform approach should be used to evaluate indeterminate radiologic findings to identify what constitutes no evidence of disease at trial entry and what constitutes radiologic evidence of disease. Eleventh, a uniform approach for establishing the date of recurrence should be included in any trial design. Twelfth, patient perspectives on the use of placebo, conditions for unblinding, and research biopsies should be considered carefully during the conduct of an adjuvant trial.

Conclusions And Relevance: The discussions suggested that a uniform approach to eligibility criteria and radiologic disease assessment will lead to more consistently interpretable trial results in the adjuvant RCC therapy setting.
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http://dx.doi.org/10.1001/jamaoncol.2019.4117DOI Listing
January 2020

Individualizing Management of Infertility in Classic Congenital Adrenal Hyperplasia and Testicular Adrenal Rest Tumors.

J Endocr Soc 2019 Dec 17;3(12):2290-2294. Epub 2019 Oct 17.

Section on Congenital Disorders, National Institutes of Health Clinical Center, Bethesda, Maryland.

Testicular adrenal rest tumors (TARTs) are a common cause of male infertility in patients with classic congenital adrenal hyperplasia (CAH). These tumors are located in the rete testis and can lead to impaired blood flow and functional impairment of seminiferous tubules. We describe restoration of fertility in a man with CAH and bilateral TARTs with use of lower-dose glucocorticoid therapy than previously described. A 28-year-old man with classic salt-wasting CAH presented with impaired fertility. Biochemical evaluation showed poor CAH control despite reported compliance with prednisone 5 mg every morning and fludrocortisone 50 μg twice daily. Semen analysis showed azoospermia. Testicular ultrasonography showed TARTs occupying 16% of total testicular volume. After 5 months of dexamethasone 250 μg at bedtime, total TART volume decreased 90%, biochemical control improved, and semen analysis showed a sperm count of 132 × 10 million per milliliter. The patient's wife was confirmed to be pregnant 9 months after the initial visit and delivered a healthy full-term baby girl. The patient's glucocorticoid therapy was changed to prednisone 3 mg twice daily, and 2 years later he continues to show adequate CAH control, stable TART volume, and normal semen analysis, and his wife is pregnant again. Management of CAH in men with TARTs needs to be individualized, and high-dose dexamethasone may not be indicated. The use of a long-acting glucocorticoid at typical recommended dosages can decrease TART size and reverse male infertility. Prednisone given once daily does not adequately control the ACTH-driven complications of CAH.
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http://dx.doi.org/10.1210/js.2019-00227DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6853670PMC
December 2019

Eligibility and Radiologic Assessment in Adjuvant Clinical Trials in Bladder Cancer.

JAMA Oncol 2019 Dec;5(12):1790-1798

Center for Drug Evaluation and Research, US Food and Drug Administration, Silver Spring, Maryland.

Objective: To harmonize eligibility criteria and radiographic disease assessments in clinical trials of adjuvant therapy for muscle-invasive bladder cancer (MIBC).

Methods: National experts in bladder cancer clinical trial research, including medical and urologic oncologists, radiologists, biostatisticians, and patient advocates, convened at a public workshop on November 28, 2017, to discuss eligibility, radiographic entry criteria, and assessment of disease recurrence in adjuvant clinical trials in patients with MIBC.

Results: The key workshop conclusions for adjuvant MIBC clinical trials included the following points: (1) patients with urothelial carcinoma with divergent histologic differentiation should be allowed to enroll; (2) neoadjuvant chemotherapy is defined as at least 3 cycles of neoadjuvant cisplatin-based combination chemotherapy; (3) patients with muscle-invasive, upper-tract urothelial carcinoma should be included in adjuvant trials of MIBC; (4) patients with severe renal insufficiency can enroll into trials using agents that are not renally excreted; (5) patients with microscopic surgical margins can be included; (6) patients should undergo a standard bilateral lymph node dissection prior to enrollment; (7) computed tomographic (CT) imaging should be performed within 4 weeks prior to enrollment. For patients with renal insufficiency who cannot undergo CT imaging with contrast, noncontrast chest CT and magnetic resonance imaging of the abdomen and pelvis with gadolinium should be done; (8) biopsy of indeterminate lesions to evaluate for malignant disease should be done when feasible; (9) a uniform approach to evaluate indeterminate radiographic lesions when biopsy is not feasible should be included in any trial design; (10) a uniform approach to determining the date of recurrence is important in interpreting adjuvant trial results; and (11) new high-grade, upper-tract primary tumors and new MIBC tumors should be considered recurrence events.

Conclusions And Relevance: A uniform approach to eligibility criteria, definitions of no evidence of disease, and definitions of disease recurrence may lead to more consistent interpretations of adjuvant trial results in MIBC.
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http://dx.doi.org/10.1001/jamaoncol.2019.4114DOI Listing
December 2019

Clinical symptoms and associated vascular imaging findings in Takayasu's arteritis compared to giant cell arteritis.

Ann Rheum Dis 2020 02 24;79(2):262-267. Epub 2019 Oct 24.

Systemic Autoimmunity Branch, National Institutes of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD, USA

Objective: To compare the presence of head, neck and upper extremity symptoms in patients with Takayasu's (TAK) and giant cell arteritis (GCA) and their association with vascular inflammation assessed by F-fluorodeoxyglucose positron emission tomography (FDG-PET) or arterial damage assessed by magnetic resonance angiography (MRA).

Methods: Patients with TAK and GCA underwent clinical and imaging assessments within 24 hours, blinded to each other. Vascular inflammation was defined as arterial FDG-PET uptake greater than liver by visual assessment. Arterial damage was defined as stenosis, occlusion, or aneurysm by MRA. Clinically reported symptoms were compared with corresponding imaging findings using generalised mixed model regression. Cranial symptoms were studied in association with burden of arterial disease in the neck using ordinal regression.

Results: Participants with TAK (n=56) and GCA (n=54) contributed data from 270 visits. Carotidynia was reported only in patients with TAK (21%) and was associated with vascular inflammation (p<0.01) but not damage (p=0.33) in the corresponding carotid artery. Posterior headache was reported in TAK (16%) and GCA (20%) but was only associated with corresponding vertebral artery inflammation and damage in GCA (p<0.01). Arm claudication was associated with subclavian artery damage (p<0.01) and inflammation (p=0.04) in TAK and with damage in GCA (p<0.01). Patients with an increased burden of damaged neck arteries were more likely to experience positional lightheadedness (p<0.01) or a major central nervous system event (p=0.01).

Conclusion: The distribution of symptoms and association with imaging abnormalities differs in patients with TAK and GCA. These findings may help clinicians predict associated FDG-PET and MRA findings based on a specific clinical symptom.

Clinical Trial Registration Number: NCT02257866.
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http://dx.doi.org/10.1136/annrheumdis-2019-216145DOI Listing
February 2020

Mucinous Neoplasms of the Ovary: Radiologic-Pathologic Correlation.

Radiographics 2019 Jul-Aug;39(4):982-997

From the Department of Radiology and Imaging Sciences, National Institutes of Health Clinical Center, Bethesda, Md (J.M.); Department of Obstetrics and Gynecology, George Washington University School of Medicine and Health Sciences, Washington, DC (K.I.M.); University of Illinois College of Medicine, Peoria, Ill (S.L.P.); Department of Pathology, the Joint Pathology Center, Washington, DC (B.A.C., R.M.); American Institute for Radiologic Pathology, 1100 Wayne Ave, Suite 1020, Silver Spring, MD 20910 (J.M., D.J.W.); and Community Radiology Division, Department of Radiology, Johns Hopkins Hospital and Health System, Washington, DC (D.J.W.).

Mucinous neoplasms of the ovary account for 10%-15% of ovarian neoplasms. They may be benign, borderline, or malignant. The large majority are benign or borderline, accounting for 80% and 16%-17%, respectively. Mucinous neoplasms of the ovary most commonly affect women in their 20s to 40s. The clinical manifestation is nonspecific, but most mucinous ovarian neoplasms manifest as large unilateral pelvic masses. At gross pathologic analysis, mucinous ovarian neoplasms appear as large multiloculated cystic masses. The contents of the cyst loculi vary on the basis of differences in internal mucin content. At histologic analysis, mucinous ovarian neoplasms are composed of multiple cysts lined by mucinous epithelium, often resembling gastrointestinal-type epithelium. Imaging evaluation most commonly includes US and/or MRI. The imaging findings parallel the gross pathologic features and include a large, unilateral, multiloculated cystic mass. The cyst loculi vary in echogenicity, attenuation, and signal intensity depending on the mucin content. Mucinous neoplasms of the ovary are staged surgically using the FIGO (International Federation of Gynecology and Obstetrics) staging system. Primary treatment is surgical, with adjuvant chemotherapy considered in the uncommon case of mucinous carcinoma with extraovarian disease. Since most mucinous ovarian neoplasms are benign or borderline, the overall prognosis is excellent.
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http://dx.doi.org/10.1148/rg.2019180221DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6677283PMC
May 2020

Interreader Variability of Prostate Imaging Reporting and Data System Version 2 in Detecting and Assessing Prostate Cancer Lesions at Prostate MRI.

AJR Am J Roentgenol 2019 Mar 27:1-8. Epub 2019 Mar 27.

14 National Institutes of Health Clinical Center, Imaging Biomarkers and Computer-Aided Diagnosis Laboratory, Radiology and Imaging Sciences, Bethesda, MD.

Objective: The purpose of this study was to evaluate agreement among radiologists in detecting and assessing prostate cancer at multiparametric MRI using Prostate Imaging Reporting and Data System version 2 (PI-RADSv2).

Materials And Methods: Treatment-naïve patients underwent 3-T multipara-metric MRI between April 2012 and June 2015. Among the 163 patients evaluated, 110 underwent prostatectomy after MRI and 53 had normal MRI findings and transrectal ultrasound-guided biopsy results. Nine radiologists participated (three each with high, intermediate, and low levels of experience). Readers interpreted images of 58 patients on average (range, 56-60) using PI-RADSv2. Prostatectomy specimens registered to MRI were ground truth. Interob-server agreement was evaluated with the index of specific agreement for lesion detection and kappa and proportion of agreement for PI-RADS category assignment.

Results: The radiologists detected 336 lesions. Sensitivity for index lesions was 80.9% (95% CI, 75.1-85.9%), comparable across reader experience (p = 0.392). Patient-level specificity was experience dependent; highly experienced readers had 84.0% specificity versus 55.2% for all others (p < 0.001). Interobserver agreement was excellent for detecting index lesions (index of specific agreement, 0.871; 95% CI, 0.798-0.923). Agreement on PI-RADSv2 category assignment of index lesions was moderate (κ = 0.419; 95% CI, 0.238-0.595). For individual category assignments, proportion of agreement was slight for PI-RADS category 3 (0.208; 95% CI, 0.086-0.284) but substantial for PI-RADS category 4 (0.674; 95% CI, 0.540-0.776). However, proportion of agreement for T2-weighted PI-RADS 4 in the transition zone was 0.250 (95% CI, 0.108-0.372). Proportion of agreement for category assignment of index lesions on dynamic contrast-enhanced MR images was 0.822 (95% CI, 0.728-0.903), on T2-weighted MR images was 0.515 (95% CI, 0.430-0623), and on DW images was 0.586 (95% CI, 0.495-0.682). Proportion of agreement for dominant lesion was excellent (0.828; 95% CI, 0.742-0.913).

Conclusion: Radiologists across experience levels had excellent agreement for detecting index lesions and moderate agreement for category assignment of lesions using PI-RADS. Future iterations of PI-RADS should clarify PI-RADS 3 and PI-RADS 4 in the transition zone.
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http://dx.doi.org/10.2214/AJR.18.20536DOI Listing
March 2019

Retroperitoneal Leiomyosarcoma From the Radiologic Pathology Archives.

Radiographics 2018 Sep-Oct;38(5):1403-1420

From the Department of Radiology and Imaging Sciences, National Institutes of Health, Bethesda, Md (J.M.); American Institute for Radiologic Pathology, 1100 Wayne Ave, Suite 1020, Silver Spring, MD 20910 (J.M., D.J.W.); and Community Radiology Division, Johns Hopkins School of Medicine, Washington, DC (D.J.W.).

Leiomyosarcoma is a malignant neoplasm that shows smooth muscle differentiation. It is the second most common sarcoma to affect the retroperitoneum. Retroperitoneal leiomyosarcomas may grow to large sizes before detection and may be an incidental finding at imaging. When symptomatic, retroperitoneal leiomyosarcoma may cause compressive symptoms, including pain. Retroperitoneal leiomyosarcoma most commonly manifests as a large soft-tissue mass, with areas of necrosis. The most frequent pattern of growth is an entirely extravascular mass. Less commonly, leiomyosarcoma may demonstrate both extravascular and intravascular components. Rarely, retroperitoneal leiomyosarcomas are completely intravascular, typically arising from the inferior vena cava. Given its variable imaging features, a large variety of neoplastic and nonneoplastic conditions are included in the differential diagnosis of retroperitoneal leiomyosarcoma. In this review, the authors discuss retroperitoneal leiomyosarcoma, with emphasis on the pathologic basis of disease, and illustrate the multimodality imaging appearances of retroperitoneal leiomyosarcoma using cases from the Radiologic Pathology Archives of the American Institute for Radiologic Pathology. The authors review important differential considerations of retroperitoneal leiomyosarcoma, focusing on the extravascular pattern of growth, and emphasize clinical and imaging features that help radiologists differentiate leiomyosarcoma from the most frequent mimics. The information presented in this review will aid radiologists in fulfilling their key roles in the diagnosis, operative planning, and follow-up of patients with retroperitoneal leiomyosarcoma.
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http://dx.doi.org/10.1148/rg.2018180006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6166742PMC
December 2018

Comparison of magnetic resonance angiography and F-fluorodeoxyglucose positron emission tomography in large-vessel vasculitis.

Ann Rheum Dis 2018 08 17;77(8):1165-1171. Epub 2018 Apr 17.

Systemic Autoimmunity Branch, National Institutes of Health, NIAMS, Bethesda, Maryland, USA.

Objectives: To assess agreement between interpretation of magnetic resonance angiography (MRA) and F-fluorodeoxyglucose positron emission tomography (PET) for disease extent and disease activity in large-vessel vasculitis (LVV) and determine associations between imaging and clinical assessments.

Methods: Patients with giant cell arteritis (GCA), Takayasu's arteritis (TAK) and comparators were recruited into a prospective, observational cohort. Imaging and clinical assessments were performed concurrently, blinded to each other. Agreement was assessed by per cent agreement, Cohen's kappa and McNemar's test. Multivariable logistic regression identified MRA features associated with PET scan activity.

Results: Eighty-four patients (GCA=35; TAK=30; comparator=19) contributed 133 paired studies. Agreement for disease extent between MRA and PET was 580 out of 966 (60%) arterial territories with Cohen's kappa=0.22. Of 386 territories with disagreement, MRA demonstrated disease in more territories than PET (304vs82, p<0.01). Agreement for disease activity between MRA and PET was 90 studies (68%) with Cohen's kappa=0.30. In studies with disagreement, MRA demonstrated activity in 23 studies and PET in 20 studies (p=0.76). Oedema and wall thickness on MRA were independently associated with PET scan activity. Clinical status was associated with disease activity by PET (p<0.01) but not MRA (p=0.70), yet 35/69 (51%) patients with LVV in clinical remission had active disease by both MRA and PET.

Conclusions: In assessment of LVV, MRA and PET contribute unique and complementary information. MRA better captures disease extent, and PET scan is better suited to assess vascular activity. Clinical and imaging-based assessments often do not correlate over the disease course in LVV.

Trial Registration Number: NCT02257866.
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http://dx.doi.org/10.1136/annrheumdis-2018-213102DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6045453PMC
August 2018

Computer-aided diagnosis prior to conventional interpretation of prostate mpMRI: an international multi-reader study.

Eur Radiol 2018 Oct 12;28(10):4407-4417. Epub 2018 Apr 12.

Molecular Imaging Program, NCI, NIH, 10 Center Drive, Room B3B85, Bethesda, MD, 20892, USA.

Objectives: To evaluate if computer-aided diagnosis (CAD) prior to prostate multi-parametric MRI (mpMRI) can improve sensitivity and agreement between radiologists.

Methods: Nine radiologists (three each high, intermediate, low experience) from eight institutions participated. A total of 163 patients with 3-T mpMRI from 4/2012 to 6/2015 were included: 110 cancer patients with prostatectomy after mpMRI, 53 patients with no lesions on mpMRI and negative TRUS-guided biopsy. Readers were blinded to all outcomes and detected lesions per PI-RADSv2 on mpMRI. After 5 weeks, readers re-evaluated patients using CAD to detect lesions. Prostatectomy specimens registered to MRI were ground truth with index lesions defined on pathology. Sensitivity, specificity and agreement were calculated per patient, lesion level and zone-peripheral (PZ) and transition (TZ).

Results: Index lesion sensitivity was 78.2% for mpMRI alone and 86.3% for CAD-assisted mpMRI (p = 0.013). Sensitivity was comparable for TZ lesions (78.7% vs 78.1%; p = 0.929); CAD improved PZ lesion sensitivity (84% vs 94%; p = 0.003). Improved sensitivity came from lesions scored PI-RADS < 3 as index lesion sensitivity was comparable at PI-RADS ≥ 3 (77.6% vs 78.1%; p = 0.859). Per patient specificity was 57.1% for CAD and 70.4% for mpMRI (p = 0.003). CAD improved agreement between all readers (56.9% vs 71.8%; p < 0.001).

Conclusions: CAD-assisted mpMRI improved sensitivity and agreement, but decreased specificity, between radiologists of varying experience.

Key Points: • Computer-aided diagnosis (CAD) assists clinicians in detecting prostate cancer on MRI. • CAD assistance improves agreement between radiologists in detecting prostate cancer lesions. • However, this CAD system induces more false positives, particularly for less-experienced clinicians and in the transition zone. • CAD assists radiologists in detecting cancer missed on MRI, suggesting a path for improved diagnostic confidence.
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http://dx.doi.org/10.1007/s00330-018-5374-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8023433PMC
October 2018

Liver disturbances in activated phosphoinositide 3-kinase δ syndrome.

J Allergy Clin Immunol Pract 2018 Sep - Oct;6(5):1763-1765. Epub 2018 Feb 16.

Translational Hepatology Section, Liver Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Md.

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http://dx.doi.org/10.1016/j.jaip.2018.01.005DOI Listing
November 2019

Validation of the Dominant Sequence Paradigm and Role of Dynamic Contrast-enhanced Imaging in PI-RADS Version 2.

Radiology 2017 12 19;285(3):859-869. Epub 2017 Jul 19.

From the Molecular Imaging (M.D.G., F.V.M., P.L.C., B.T.) and Biometric Research (J.H.S.) Programs, Laboratory of Pathology (M.J.M.), and Urologic Oncology Branch (P.A.P.), National Cancer Institute, National Institutes of Health, 10 Center Dr, Room B3B85, Bethesda, MD 20892; Imaging Biomarkers and Computer-Aided Diagnosis Laboratory, Department of Radiology and Imaging Sciences, National Institutes of Health Clinical Center, Bethesda, Md (N.L., R.M.S.); Department of Radiology, University of Cambridge School of Medicine, Cambridge, England (T.B.); Department of Radiology, Universidade Federal Fluminense, Rio de Janeiro, Brazil (L.K.B.); Department of Body Imaging, CDPI Clinics/DASA, Rio de Janeiro, Brazil (L.K.B.); Department of Radiology, George Washington University Hospital, Washington, DC (S.B.); Department of Radiology, Hacettepe University, Ankara, Turkey (I.M.K.); Department of Diagnostic Radiology Singapore General Hospital, Singapore (Y.M.L.); Department of Radiology, Walter Reed National Military Medical Center, Bethesda, Md (J.M.); Department of Radiology, Nephrology Center, Mansoura University, Mansoura, Egypt (H.S.); Center for Interventional Oncology, National Cancer Institute and Clinical Center, and Radiology Imaging Sciences, National Institutes of Health, Bethesda, Md (B.J.W.).

Purpose To validate the dominant pulse sequence paradigm and limited role of dynamic contrast material-enhanced magnetic resonance (MR) imaging in the Prostate Imaging Reporting and Data System (PI-RADS) version 2 for prostate multiparametric MR imaging by using data from a multireader study. Materials and Methods This HIPAA-compliant retrospective interpretation of prospectively acquired data was approved by the local ethics committee. Patients were treatment-naïve with endorectal coil 3-T multiparametric MR imaging. A total of 163 patients were evaluated, 110 with prostatectomy after multiparametric MR imaging and 53 with negative multiparametric MR imaging and systematic biopsy findings. Nine radiologists participated in this study and interpreted images in 58 patients, on average (range, 56-60 patients). Lesions were detected with PI-RADS version 2 and were compared with whole-mount prostatectomy findings. Probability of cancer detection for overall, T2-weighted, and diffusion-weighted (DW) imaging PI-RADS scores was calculated in the peripheral zone (PZ) and transition zone (TZ) by using generalized estimating equations. To determine dominant pulse sequence and benefit of dynamic contrast-enhanced (DCE) imaging, odds ratios (ORs) were calculated as the ratio of odds of cancer of two consecutive scores by logistic regression. Results A total of 654 lesions (420 in the PZ) were detected. The probability of cancer detection for PI-RADS category 2, 3, 4, and 5 lesions was 15.7%, 33.1%, 70.5%, and 90.7%, respectively. DW imaging outperformed T2-weighted imaging in the PZ (OR, 3.49 vs 2.45; P = .008). T2-weighted imaging performed better but did not clearly outperform DW imaging in the TZ (OR, 4.79 vs 3.77; P = .494). Lesions classified as PI-RADS category 3 at DW MR imaging and as positive at DCE imaging in the PZ showed a higher probability of cancer detection than did DCE-negative PI-RADS category 3 lesions (67.8% vs 40.0%, P = .02). The addition of DCE imaging to DW imaging in the PZ was beneficial (OR, 2.0; P = .027), with an increase in the probability of cancer detection of 15.7%, 16.0%, and 9.2% for PI-RADS category 2, 3, and 4 lesions, respectively. Conclusion DW imaging outperforms T2-weighted imaging in the PZ; T2-weighted imaging did not show a significant difference when compared with DW imaging in the TZ by PI-RADS version 2 criteria. The addition of DCE imaging to DW imaging scores in the PZ yields meaningful improvements in probability of cancer detection. RSNA, 2017 An earlier incorrect version of this article appeared online. This article was corrected on July 27, 2017. Online supplemental material is available for this article.
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http://dx.doi.org/10.1148/radiol.2017161316DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5708285PMC
December 2017

Testicular Seminoma and Its Mimics: From the Radiologic Pathology Archives.

Radiographics 2017 Jul-Aug;37(4):1085-1098. Epub 2017 Jun 2.

From the Department of Radiology and Imaging Sciences, National Institutes of Health, Bethesda, Md (J.M.); American Institute for Radiologic Pathology, 1100 Wayne Ave, Suite 1020, Silver Spring, MD 20910 (J.M., D.J.W.); Department of Radiology, Johns Hopkins School of Medicine, Washington, DC (D.J.W., A.L.A.); and Joint Pathology Center, Silver Spring, Md (I.A.S.).

Testicular seminoma is the most common malignant tumor of the testis. It classically manifests as a painless mass. Radiologic evaluation with high-frequency ultrasonography (US) is critical for diagnosis. Seminomas are usually homogeneously hypoechoic masses at US. In challenging cases, magnetic resonance (MR) imaging may help confirm that a mass is intratesticular and provide data for local staging. Computed tomography (CT) provides valuable information for staging, including the presence and size of retroperitoneal lymph nodes. Testicular seminoma is treated with radical inguinal orchiectomy and is highly curable even at advanced stages of disease. Several neoplastic and nonneoplastic conditions may mimic testicular seminoma at imaging. Benign mimics include segmental infarction, hematoma, infection, epidermoid cyst, adrenal rests, sarcoidosis, splenogonadal fusion, and sex cord-stromal tumors. Malignant mimics include nonseminomatous germ cell tumors, lymphoma, and metastases. These conditions are individually reviewed with emphasis on features that allow differentiation from seminoma. Spermatocytic tumor, formerly known as spermatocytic seminoma, accounts for only 1% of testicular tumors. It is distinct from classic seminoma, with unique histologic, molecular, and genetic features. It affects an older patient population than classic seminoma and demonstrates indolent clinical behavior. Radiologists serve a key role in diagnosis, staging, and surveillance of patients with seminoma. A thorough knowledge of related clinical, radiologic, and pathologic findings will help the radiologist contribute to high-quality interdisciplinary care of affected patients.
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http://dx.doi.org/10.1148/rg.2017160164DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5548453PMC
November 2017

11-Oxygenated Androgens Are Biomarkers of Adrenal Volume and Testicular Adrenal Rest Tumors in 21-Hydroxylase Deficiency.

J Clin Endocrinol Metab 2017 08;102(8):2701-2710

National Institutes of Health Clinical Center, Bethesda, Maryland 20892.

Context: Patients with 21-hydroxylase deficiency (21OHD) have long-term complications, resulting from poor disease control and/or glucocorticoid overtreatment. Lack of optimal biomarkers has made it challenging to tailor therapy and predict long-term outcomes.

Objective: To identify biomarkers of disease control and long-term complications in 21OHD.

Setting And Participants: Cross-sectional study of 114 patients (70 males), ages 2 to 67 years (median, 15 years), seen in a tertiary referral center.

Methods: We correlated a mass-spectrometry panel of 23 steroids, obtained before first morning medication, with bone age advancement (children), adrenal volume (adults), testicular adrenal rest tumors (TART), hirsutism, menstrual disorders, and pituitary hormones.

Results: Total adrenal volume correlated positively with 18 steroids, most prominently 21-deoxycortisol and four 11-oxygenated-C19 (11oxC19) steroids: 11β-hydroxyandrostenedione (11OHA4), 11-ketoandrostenedione (11ketoA4), 11β-hydroxytestosterone (11OHT), and 11-ketotestosterone (11ketoT) (r ≈ 0.7, P < 0.0001). Nine steroids were significantly higher (P ≤ 0.01) in males with TART compared with those without TART, including 11OHA4 (6.8-fold), 11OHT (4.9-fold), 11ketoT (3.6-fold), 11ketoA4 (3.3-fold), and pregnenolone sulfate (PregS; 4.8-fold). PregS (28.5-fold) and 17-hydroxypregnenolone sulfate (19-fold) levels were higher (P < 0.01) in postpubertal females with menstrual disorders. In males, testosterone levels correlated positively with all 11oxC19 steroids in Tanner stages 1 and 2 (r ≈ 0.7; P < 0.001) but negatively in Tanner stage 5 (r = -0.3 and P < 0.05 for 11ketoA4 and 11ketoT). In females, testosterone level correlated positively with all four 11oxC19 steroids across all Tanner stages (r ≈ 0.8; P < 0.0001).

Conclusion: 11oxC19 steroids and PregS might serve as clinically useful biomarkers of disease control and long-term complications in 21OHD.
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http://dx.doi.org/10.1210/jc.2016-3989DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5546849PMC
August 2017

Accuracy and agreement of PIRADSv2 for prostate cancer mpMRI: A multireader study.

J Magn Reson Imaging 2017 02 8;45(2):579-585. Epub 2016 Jul 8.

Molecular Imaging Program, NCI, NIH, Bethesda, Maryland, USA.

Purpose: Multiparametric MRI (mpMRI) improves the detection of clinically significant prostate cancer, but is limited by interobserver variation. The second version of theProstate Imaging Reporting and Data System (PIRADSv2) was recently proposed as a standard for interpreting mpMRI. To assess the performance and interobserver agreement of PIRADSv2 we performed a multi-reader study with five radiologists of varying experience.

Materials And Methods: Five radiologists (n = 2 prostate dedicated, n = 3 general body) blinded to clinicopathologic results detected and scored lesions on prostate mpMRI using PIRADSv2. The endorectal coil 3 Tesla MRI included T2W, diffusion-weighted imaging (apparent diffusion coefficient, b2000), and dynamic contrast enhancement. Thirty-four consecutive patients were included. Results were correlated with radical prostatectomy whole-mount histopathology produced with patient-specific three-dimensional molds. An index lesion was defined on pathology as the lesion with highest Gleason score or largest volume if equivalent grades. Average sensitivity and positive predictive values (PPVs) for all lesions and index lesions were determined using generalized estimating equations. Interobserver agreement was evaluated using index of specific agreement.

Results: Average sensitivity was 91% for detecting index lesions and 63% for all lesions across all readers. PPV was 85% for PIRADS ≥ 3 and 90% for PIRADS ≥ 4. Specialists performed better only for PIRADS ≥ 4 with sensitivity 90% versus 79% (P = 0.01) for index lesions. Index of specific agreement among readers was 93% for the detection of index lesions, 74% for the detection of all lesions, and 85% for scoring index lesions, and 58% for scoring all lesions.

Conclusion: By using PIRADSv2, general body radiologists and prostate specialists can detect high-grade index prostate cancer lesions with high sensitivity and agreement.

Level Of Evidence: 1 J. Magn. Reson. Imaging 2017;45:579-585.
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http://dx.doi.org/10.1002/jmri.25372DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7900895PMC
February 2017

Mesenchymal Extratesticular Tumors and Tumorlike Conditions: From the Radiologic Pathology Archives.

Radiographics 2015 Nov-Dec;35(7):1943-54. Epub 2015 Oct 30.

From the American Institute for Radiologic Pathology, 1010 Wayne Ave, Suite 320, Silver Spring, MD 20910 (D.J.W., G.E.L.); Department of Radiology, Walter Reed National Military Medical Center, Bethesda, Md (D.J.W., J.M., C.F.G.); Department of Radiology and Radiological Sciences, Uniformed Services University of the Health Sciences, Bethesda, Md (D.J.W., J.M., G.E.L.); and Joint Pathology Center, Silver Spring, Md (I.A.S.).

Both benign and malignant tumors and tumorlike conditions can arise from the nonepididymal extratesticular tissues. Benign tumors are far more common than malignant tumors, with lipoma being the most common lesion found at this site. Several imaging features can help narrow the differential diagnosis of these lesions, including the presence of fat and imaging features suggestive of fibrous tissue. Lesions that contain fat represent lipoma, liposarcoma, or angiomyofibroblastoma (AMF)-like tumor. If enhancing soft tissue is present in conjunction with fat, the differential diagnosis narrows further to just liposarcoma and AMF-like tumor. Lesions that display magnetic resonance imaging characteristics compatible with fibrous tissue are likely to be fibrous pseudotumor. However, many of the lesions in this location have overlapping imaging findings, and surgical excision is most often necessary for accurate diagnosis. The ability to narrow the differential diagnosis with imaging, however, is helpful for the clinician for both treatment planning and patient counseling.
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http://dx.doi.org/10.1148/rg.2015150179DOI Listing
September 2016

Conventional Medical Education and the History of Simulation in Radiology.

Acad Radiol 2015 Oct 12;22(10):1252-67. Epub 2015 Aug 12.

Department of Radiology, Penn State Milton S. Hershey Medical Center, 30 Hope Drive, Suite 1800, EC 008, Hershey, PA 17033-0859.

Simulation is a promising method for improving clinician performance, enhancing team training, increasing patient safety, and preventing errors. Training scenarios to enrich medical student and resident education, and apply toward competency assessment, recertification, and credentialing are important applications of simulation in radiology. This review will describe simulation training for procedural skills, interpretive and noninterpretive skills, team-based training and crisis management, professionalism and communication skills, as well as hybrid and in situ applications of simulation training. A brief overview of current simulation equipment and software and the barriers and strategies for implementation are described. Finally, methods of measuring competency and assessment are described, so that the interested reader can successfully implement simulation training into their practice.
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http://dx.doi.org/10.1016/j.acra.2015.07.003DOI Listing
October 2015

Prostate Cancer: Interobserver Agreement and Accuracy with the Revised Prostate Imaging Reporting and Data System at Multiparametric MR Imaging.

Radiology 2015 Dec 18;277(3):741-50. Epub 2015 Jun 18.

From the Molecular Imaging Program (B.G.M., S.S., P.L.C., B.T.), Biometric Research Branch, Division of Cancer Treatment and Diagnosis (J.H.S.), Urologic Oncology Branch (S.R.B., A.G., P.P.), Laboratory of Pathology (M.J.M.), and Center for Interventional Oncology (B.J.W.), National Cancer Institute, National Institutes of Health, 10 Center Dr, MSC 1182, Bldg 10, Room B3B85, Bethesda, MD 20892-1088; Department of Urology, AMC University Hospital, Amsterdam, the Netherlands (B.G.M., J.J.M.C.H.d.l.R.); and Department of Radiology and Radiological Sciences, Edward Hébert School of Medicine, Uniformed Services University of The Health Sciences, Bethesda, Md (J.M.).

Purpose: To evaluate accuracy and interobserver variability with the use of the Prostate Imaging Reporting and Data System (PI-RADS) version 2.0 for detection of prostate cancer at multiparametric magnetic resonance (MR) imaging in a biopsy-naïve patient population.

Materials And Methods: This retrospective HIPAA-compliant study was approved by the local ethics committee, and written informed consent was obtained from all patients for use of their imaging and histopathologic data in future research studies. In 101 biopsy-naïve patients with elevated prostate-specific antigen levels who underwent multiparametric MR imaging of the prostate and subsequent transrectal ultrasonography (US)-MR imaging fusion-guided biopsy, suspicious lesions detected at multiparametric MR imaging were scored by five readers who were blinded to pathologic results by using to the newly revised PI-RADS and the scoring system developed in-house. Interobserver agreement was evaluated by using κ statistics, and the correlation of pathologic results with each of the two scoring systems was evaluated by using the Kendall τ correlation coefficient.

Results: Specimens of 162 lesions in 94 patients were sampled by means of transrectal US-MR imaging fusion biopsy. Results for 87 (54%) lesions were positive for prostate cancer. Kendall τ values with the PI-RADS and the in-house-developed scoring system, respectively, at T2-weighted MR imaging in the peripheral zone were 0.51 and 0.17 and in the transitional zone, 0.45 and -0.11; at diffusion-weighted MR imaging, 0.42 and 0.28; at dynamic contrast material-enhanced MR imaging, 0.23 and 0.24, and overall suspicion scores were 0.42 and 0.49. Median κ scores among all possible pairs of readers for PI-RADS and the in-house-developed scoring system, respectively, for T2-weighted MR images in the peripheral zone were 0.47 and 0.15; transitional zone, 0.37 and 0.07; diffusion-weighted MR imaging, 0.41 and 0.57; dynamic contrast-enhanced MR imaging, 0.48 and 0.41; and overall suspicion scores, 0.46 and 0.55.

Conclusion: Use of the revised PI-RADS provides moderately reproducible MR imaging scores for detection of clinically relevant disease.
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http://dx.doi.org/10.1148/radiol.2015142818DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4666087PMC
December 2015

State-of-the-art imaging of prostate cancer.

Urol Oncol 2016 Mar 16;34(3):134-46. Epub 2015 Jun 16.

Department of Radiology, Walter Reed National Military Medical Center, Bethesda, MD; Uniformed Services University of the Health Sciences, Bethesda, MD; American Institute for Radiologic Pathology, Silver Spring, MD.

Prostate cancer is the most common cancer in men. Modern medical imaging is intimately involved in the diagnosis and management of prostate cancer. Ultrasound is primarily used to guide prostate biopsy to establish the diagnosis of prostate carcinoma. Prostate magnetic resonance imaging uses a multiparametric approach, including anatomic and functional imaging sequences. Multiparametric magnetic resonance imaging can be used for detection and localization of prostate cancer and to evaluate for disease recurrence. Computed tomography and scintigraphic imaging are primarily used to detect regional lymph node spread and distant metastases. Recent advancements in ultrasound, multiparametric magnetic resonance imaging, and scintigraphic imaging have the potential to change the way prostate cancer is diagnosed and managed. This article addresses the major imaging modalities involved in the evaluation of prostate cancer and updates the reader on the state of the art for each modality.
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http://dx.doi.org/10.1016/j.urolonc.2015.05.015DOI Listing
March 2016

From the radiologic pathology archives: Adrenal tumors and tumor-like conditions in the adult: radiologic-pathologic correlation.

Radiographics 2014 May-Jun;34(3):805-29

From the Department of Radiology and Radiological Sciences, F. Edward Hébert School of Medicine, Uniformed Services University of the Health Sciences, 4301 Jones Bridge Rd, Bethesda, MD 20814 (G.E.L., C.A.T., J.M., W.D.C.); American Institute for Radiologic Pathology, Silver Spring, Md (G.E.L., E.D.S., W.D.C.); Department of Radiology, Naval Medical Center Portsmouth, Portsmouth, Va (E.D.S.); Department of Radiology, David Grant USAF Medical Center, Travis AFB, Calif (C.A.T.); Department of Radiology, Walter Reed National Military Medical Center, Bethesda, Md (J.M.); School of Medicine, Georgetown University, Washington, DC (K.W.S.); Department of Radiology, Suburban Hospital, Bethesda, Md (W.D.C.); and Department of Endocrine Pathology, The Joint Pathology Center, Silver Spring, Md (E.E.L.).

Advanced imaging often reveals adrenal tumors and tumor-like conditions in both symptomatic and asymptomatic patients. When adrenal disease is clinically suspected, cross-sectional imaging can be helpful in evaluating the etiology of the patient's symptoms. When adrenal disease is incidentally identified, what the clinician and patient really want to know is whether the findings are benign or malignant, as this ultimately will affect their next step in management. Using radiologic-pathologic correlation, we broadly classify common, uncommon, and rare tumors and tumor-like conditions that can occur in the adrenal as benign or malignant. This classification follows predominant trends in observed biologic behavior while acknowledging those tumors that may behave in the minority in an unpredictable manner. We review the clinical background and presentation of functional adrenal tumors including Conn syndrome, Cushing syndrome, and catecholamine-secreting tumors, as well as their relationship with adrenal anatomy. We discuss a variety of benign tumors, including adrenal cortical adenoma (including oncocytoma) and pheochromocytoma, as well as uncommonly and rarely encountered tumors such as myelolipoma, hemangioma, lymphangioma, schwannoma, ganglioneuroma, and adenomatoid tumor. A variety of tumefactive but nonneoplastic lesions are addressed, including adrenal cortical hyperplasia, adrenal hemorrhage, adrenal cysts, and infections. Malignant tumors discussed include adrenal cortical carcinoma, the rare malignant pheochromocytoma, lymphoma, metastases, and sarcomas. For each tumor and tumor-like lesion, the clinical presentation, epidemiology, key imaging findings, diagnostic differential considerations, and management options are briefly addressed. Finally, an approach to the workup of suspected or incidentally discovered tumors is presented based on a selected literature survey and our clinical experience. Radiologists play an important role in identification and diagnosis of adrenal tumors and tumor-like conditions in both symptomatic and asymptomatic patients.
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http://dx.doi.org/10.1148/rg.343130127DOI Listing
February 2015

OctreoScan positive Crohn's disease mimicking an ileal carcinoid tumor.

J Clin Gastroenterol 2008 Jan;42(1):66-8

Department of Radiology, Feinberg School of Medicine, Northwestern University, 676 North St. Clair Street, Suite 800, Chicago, IL 60611, USA.

Somatostatin receptors have been identified in many tissues throughout the human body. Alterations in the expression of somatostatin receptors have been reported in many disease states including both tumorous and nontumorous conditions. Somatostatin receptor scintigraphy utilizing OctreoScan (Mallinckrodt Medical, Inc., St. Louis, MO), a radiolabled form of octreotide, has been reported to be a highly sensitive imaging technique for identifying pathology, such as neuroendocrine tumors, that are somatostatin receptor dense. Unfortunately, many conditions cause an increase in the quantity of somatostatin receptors and therefore may cause false positive Octreoscans. In this report, we discuss the alterations in somatostatin receptors that occur with Crohn's disease and describe a case of an OctreoScan-positive inflammatory mass mimicking a carcinoid tumor.
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http://dx.doi.org/10.1097/01.mcg.0000212640.52359.caDOI Listing
January 2008

Cystadenoma of the rete testis: sonographic appearance.

AJR Am J Roentgenol 2007 Aug;189(2):W67-9

Department of Radiology, Northwestern University, Feinberg School of Medicine, 676 N St. Clair St., Ste. 800, Chicago, IL 60611, USA.

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http://dx.doi.org/10.2214/AJR.05.0949DOI Listing
August 2007

Compound versus fundamental imaging in the detection of subdermal contraceptive implants.

J Ultrasound Med 2007 Mar;26(3):355-9

Department of Radiology, Division of Ultrasound, Northwestern University, Feinberg School of Medicine, 676 N St Clair St, Suite 800, Chicago, IL 60611, USA.

Objective: The purpose of this study was to compare the use of fundamental versus compound sonographic imaging in the localization of nonpalpable subdermal contraceptive implants.

Methods: We describe 3 cases of subdermal implants for which detection was requested via sonography. The implants were evaluated with both fundamental and compound imaging with various transducers ranging from 5-2 to 15-7 MHz. The fundamental and compound images were compared with respect to echogenicity and, therefore, visibility, of the contraceptive implant and the appearance of posterior acoustic features.

Results: Both fundamental and compound imaging visualized the focus representing the implant equally well. However, fundamental imaging proved superior to compound imaging for visualizing the posterior acoustic shadowing created by the implant. The posterior acoustic shadowing was the most helpful feature in the identification of these implant rods.

Conclusions: When attempting to image a subdermal contraceptive implant with sonography, fundamental imaging should be used. Compound imaging may mask the posterior acoustic features that can aid in implant identification.
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http://dx.doi.org/10.7863/jum.2007.26.3.355DOI Listing
March 2007

Incidence of visualization of the normal appendix on different MRI sequences.

Emerg Radiol 2006 Jul 24;12(5):223-6. Epub 2006 May 24.

Department of Radiology, Northwestern University Medical School, 676 North St. Clair, Chicago, IL 60611, USA.

The purpose of this paper is to assess the incidence of visualization of the normal appendix on magnetic resonance imaging (MRI) examinations of the pelvis in a large adult population and to compare the yield of commonly used sequences. Pelvic MRI scans of 111 randomly selected patients were retrospectively reviewed by two fellowship-trained body imagers. Thirty-six cases, where the entire cecum and terminal ileum were not included in the field of view, were excluded. A normal appendix was definitively visualized in 55 of 71 patients on T1 spin echo (SE) sequences (78%). The appendix was seen on 25 of 42 (60%) half-Fourier single-shot turbo spin echo T2. Visualization rates were 42% on pre-gadolinium T1 FS GRE, 54% on post-gadolinium T1 fat-suppressed gradient echo, and 17% of short tau inversion recovery sequences. MRI is an effective modality for visualization of the normal appendix. This may have important implications in patients with abdominal or pelvic pain, as visualization of a normal appendix should exclude the possibility of acute appendicitis from the differential diagnosis.
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http://dx.doi.org/10.1007/s10140-006-0489-5DOI Listing
July 2006