Imaging of pediatric extragonadal pelvic soft tissue tumors: A COG Diagnostic Imaging Committee/SPR Oncology Committee White Paper.

The most common pediatric extragonadal pelvic cancers include germ cell tumors, sacrococcygeal teratomas, and rhabdomyosarcomas (arising from the urinary bladder, prostate, paratesticular tissues, vagina, uterus, and perineum). This paper describes the radiological and nuclear medicine features of these entities and provides consensus-based recommendations for the assessment at diagnosis, during, and after treatment.

Communication - a lost art?

The field of radiology has benefited greatly from the technological boom that has brought greater precision, efficiency and utilization amid an exponential growth in medical science. The downside is that the same technology that has allowed the field to grow is contributing to an erosion of interpersonal communication and connection with patients and referring physicians. Remote reading has displaced us from the communal reading room, where much interaction and teaching used to take place. The "invisible" radiologist must transcend these barriers in order to preserve and strengthen the role of radiology in medical care. With modest adaptation, radiologists can regain their identity as consultants, where they have the greatest chance to show their value and thwart the drive toward commoditization.

MRI for Response Assessment of Extensive Lymphatic Malformations in Children Treated With Sirolimus.

BACKGROUND. Extensive lymphatic malformations (LMs) may cause substantial morbidity. The mammalian target of rapamycin (mTOR) inhibitor sirolimus shows promise for treating vascular anomalies, although response assessment is not standardized. OBJECTIVE. The purpose of this study was to retrospectively characterize changes seen on MRI of children with extensive LMs treated with sirolimus. METHODS. Twenty-five children treated with sirolimus for extensive LMs were included. Baseline MRI was defined as the MRI examination performed closest to therapy initiation; follow-up MRI was defined as the most recent MRI examination performed while the patient was receiving therapy. Two pediatric radiologists independently determined MRI lesion volume by tracing lesion contours on all slices (normalized to patient body surface area expressed in square meters) and determined signal by placing an ROI on the dominant portion of the lesions (normalized to CSF signal) on baseline and follow-up T2-weighted MRI sequences. Interreader agreement was determined, and values were averaged for further analysis. Volume and signal changes were compared with patient, lesion, and treatment characteristics. RESULTS. The mean (± SD) interval between initiation of sirolimus treatment and follow-up MRI was 22.1 ± 13.8 months. The mean lesion volume index on baseline and follow-up MRI was 728 ± 970 and 345 ± 501 mL/m2, respectively (p < .001). Ninety-two percent of children showed a decrease in lesion volume index that was greater than 10% (mean volume change, -46.4% ± 28.2%). Volume change was inversely correlated with age (r = -0.466; p = .02). The mean volume change was -64.7% ± 25.4% in children younger than 2 years old versus -32.0% ± 21.6% in children 2 years old or older (p = .008). The mean volume change was -58.1% ± 24.0% for craniocervical lesions versus -35.5% ± 28.2% for lesions involving the trunk and/or extremities (p = .03). Mean lesion signal ratio on baseline and follow-up MRI was 0.81 ± 0.29 and 0.59 ± 0.26, respectively (p < .001). Mean signal ratio change was -23.8% ± 22.7%. Volume and signal changes were moderately correlated (r = 0.469; p = .02). Volume and signal changes were not associated with sex, lesion subtype, serum concentration of sirolimus, or the interval between sirolimus initiation and follow-up MRI (p > .05). Interreader agreement for volume index change was excellent (intraclass correlation coefficient, 0.983), and that for signal ratio change was moderate to good (intraclass correlation coefficient, 0.764). CONCLUSION. Sirolimus treatment of extensive LMs in children is associated with significant reductions in volume and signal on T2-weighted MRI. The decrease in volume is greater in younger children and craniocervical lesions. CLINICAL IMPACT. The results may facilitate development of standardized MRI-based criteria for assessing the response of vascular malformations to pharmacotherapy.

Preserving the value of legacy film-based teaching files in pediatric radiology.

Senior pediatric radiologists who have spent a major portion of their careers interpreting conventional film-screen radiographic studies have collected a wealth of hard-copy teaching material that is at risk of becoming obsolete. The teaching value and usefulness of analog film teaching files can be preserved using available hardware and standard software. The final product can be made available in a high-quality digital format to students, trainees and faculty without complicated search-and-retrieval methodology. This paper describes a relatively simple and low-cost procedure to preserve and use this source of wisdom and experience. It also emphasizes the role that such a resource can play as part of a comprehensive educational program.

Pediatric magnetic resonance imaging training versus job-readiness: using education research tools to re-align.

BACKGROUND: Fellows begin MRI training with variable experience and expertise. To better serve patients, pediatric radiology fellows should gain competence in MRI that enables seamless transition to independent practice. OBJECTIVE: We implemented a needs assessment survey and conducted a focus group discussion to identify knowledge gaps and inform creation of a curriculum for pediatric body MRI. MATERIALS AND METHODS: We electronically distributed a comprehensive anonymous needs assessment survey in October 2016 to current fellows and recent (<5 years) graduates from Accreditation Council for Graduate Medical Education (ACGME)-accredited pediatric radiology fellowships, with follow-up in January 2017. We conducted a focus group discussion among current fellows at our institution in October 2017 to inform a better understanding of the results. RESULTS: Eighty-one pediatric radiologists (8 fellows/73 attendings) completed the survey (24%); 5 current fellows participated in the focus group. The technical issues most commonly identified with limited or no instruction during training included setting up an MR service, accessory equipment (coil) selection and MRI field inhomogeneity correction. Areas needing increased attention and inclusion within the curriculum included coil choice/patient positioning (n=42, 52%), contrast agents (n=40, 49%), field strength (n=33, 41%) and strategies for motion correction (n=33, 41%). Most fellows were uncomfortable with setting up an MR service (n=57, 70%), correcting field inhomogeneity (n=56, 69%) and improving image quality (n=50, 62%). The focus group showed consensus that there was insufficient MR training in residency to prepare them for fellowship. The group also preferred shorter lectures and streaming via video education/tutorials. CONCLUSION: While traditional instruction emphasizes image interpretation, trainees in pediatric radiology need a curriculum that also emphasizes technical and non-interpretive aspects of MRI.

RADIAL: leveraging a learning management system to support radiology education.

Increasing volume and complexity of cases in academic radiology and the drive toward pediatric sub-specialization have threatened knowledge assimilation for radiologists. There is a clear need for a system that retrieves vetted information from the excess available on the internet. Partnered with an interdisciplinary team from the Graduate School of Education, the authors created the first comprehensive learning management system (LMS) for radiology, implemented in the reading room to augment image interpretation and point-of-care education. The LMS supports quantitative analysis using a robust analytics platform to evaluate user statistics, facilitating improved quality of patient care by revolutionizing the way radiologists assimilate knowledge. This integration promises to enhance workflow and point-of-care teaching and to support the highest quality of care.

Whole-Body PET/MRI Applications in Pediatric Oncology.

OBJECTIVE. Fluorine-18-labeled FDG PET/CT and MRI are current imaging standards for staging and assessing tumor response and recurrence of pediatric extracranial solid tumors. PET/MRI combines anatomic and physiologic imaging in a single session with reduced radiation compared with CT. Pediatric protocols are primarily whole-body protocols because of the behavior and type of cancers unique to children. This article will focus on the practice and utility of whole-body PET/MRI for pediatric oncologic imaging. CONCLUSION. The strengths of PET/MRI over PET/CT are compelling and include decreased radiation exposure, decreased number of sedation and general anesthesia events, single-day one-stop visits, and simultaneous imaging with two stand-alone advanced imaging techniques essential to staging and assessing treatment response in pediatric oncology.

The continuous lure of pediatric radiology.

Pediatric radiology is an immensely rewarding career choice. Eight pediatric radiologists, enthusiastic for their profession, were asked six questions about their career choice. Their responses illustrate the common virtues of pediatric radiology and also demonstrate the diverse paths and activities that pediatric radiologists take and pursue.

Diagnostic performance and role of the contrast enema for low intestinal obstruction in neonates.

PURPOSE: We aim to evaluate the diagnostic performance and relationship between clinical characteristics, imaging findings, and final diagnosis for the neonatal contrast enema (CE). METHODS: Retrospective 10-year review of all neonatal CEs including imaging findings, clinical information, indication, and final diagnosis from discharge summaries, surgical reports, and pathology (reference standard). Two blinded pediatric radiologists reinterpreted 366 CEs for obstruction, microcolon, rectosigmoid index (RSI), serrations, meconium, ileal cut-off, transition zone, diagnosis, and level of confidence. CE diagnostic performance was calculated versus reference standard. RESULTS: Diagnoses included Hirschsprung disease (HD) (15.8%), small left colon syndrome (14.8%), small intestinal atresia/colonic atresia (SIA/CA) (12.6%), meconium ileus (MI) (4.4%), and normal (48.9%). CE had a moderate specificity (87.7%) and low sensitivity (65.5%) for HD; abnormal RSI and serrations showed high specificities (90.3%, 97.4%) but low sensitivities (46.6%, 17.2%). CE showed high specificity (97.4%) and low sensitivity (56.3%) for MI blinded to cystic fibrosis status. Microcolon was specific (96.6%) but not sensitive (68.8%) for MI. CE showed highest PPV (73.1%) (specificity 95.6%, sensitivity 82.6%) for SIA/CA. Microcolon with an abrupt cut-off was specific (99.1%) but not sensitive (41.3%) for atresias. CONCLUSION: Neonatal CE demonstrates high specificities and low to moderate sensitivities across all diagnoses, with lowest performance in HD. CLINICAL TRIAL REGISTRATION: None.

Imaging surveillance for children with predisposition to renal tumors.

Effective surveillance is necessary for early detection of tumors in children with cancer predisposition syndromes. Instituting a surveillance regimen in children comes with practical challenges that include determining imaging modality and timing, and considering cost efficiency, accessibility, and the significant consequences of false-positive and false-negative results. To address these challenges, the American Association for Cancer Research has recently published consensus recommendations that focus on surveillance of cancer predisposition syndromes in children. This review condenses the imaging surveillance recommendations for syndromes that carry a predisposition to renal tumors in childhood, and includes summaries of the predisposition syndromes and discussion of considerations of available imaging modalities.

Whole-body magnetic resonance imaging of pediatric cancer predisposition syndromes: special considerations, challenges and perspective.

Cancer predisposition syndromes increase the incidence of tumors during childhood and are associated with significant morbidity and mortality. Imaging is paramount for ensuring early detection of neoplasms, impacting therapeutic interventions and potentially improving outcome. While conventional imaging techniques involve considerable exposure to ionizing radiation, whole-body MRI is a radiation-free modality that allows continuous imaging of the entire body and has increasingly gained relevance in the surveillance, diagnosis, staging and monitoring of pediatric patients with cancer predisposition syndromes. Nevertheless, widespread implementation of whole-body MRI faces several challenges as a screening tool. Some of these challenges include developing clinical indications, variability in protocol specifications, image interpretation as well as coding and billing practices. These factors impact disease management, patient and family experience and research collaborations. In this discussion we review the aforementioned special considerations and the potential direction that might help overcome these challenges and promote more widespread use of whole-body MRI in children with cancer predisposition syndromes.

Progressive pseudorheumatoid dysplasia: a report of three cases and a review of radiographic and magnetic resonance imaging findings.

Progressive pseudorheumatoid dysplasia (PPD) is a rare disorder of postnatal skeletal and cartilage development that often presents with similar clinical findings to juvenile idiopathic arthritis. Patients with PPD display findings of progressive cartilage loss and secondary osteoarthritis over serial imaging studies and have an absence of elevation of inflammatory markers. Awareness of the imaging features of PPD on radiographs and magnetic resonance imaging (MRI) may be important for early diagnosis and surveillance of the disease.

The Asian cyclopoid copepod Mesocyclops pehpeiensis reported from the western basin of Lake Erie.

The Asian cyclopoid copepod Mesocyclops pehpeiensis Hu, 1943 has been reported as an introduced species at several locations in the western hemisphere. In the United States, reports of this exotic species are restricted to localities in Louisiana, Mississippi, and Washington D.C. This report documents a new record of occurrence for M. pehpeiensis from the western basin of Lake Erie. The detection of M. pehpeiensis in Lake Erie constitutes the first record of this species from the Laurentian Great Lakes, and the northernmost record in the western hemisphere. The species was found in 2016, 2017 and 2018, including females with egg sacks, and can therefore be considered established in the area. The occurrence of M. pehpeiensis in Lake Erie suggests that this Asian copepod may be more widely distributed in North America than is currently understood.

Evidence that cell surface localization of serine protease activity facilitates cleavage of the protease activated receptor CDCP1.

The cellular receptor CUB domain containing protein 1 (CDCP1) is commonly elevated and functionally important in a range of cancers. CDCP1 is cleaved by serine proteases at adjacent sites, arginine 368 (R368) and lysine 369 (K369), which induces cell migration in vitro and metastasis in vivo. We demonstrate that membrane localization of serine protease activity increases efficacy of cleavage of CDCP1, and that both secreted and membrane anchored serine proteases can have distinct preferences for cleaving at CDCP1-R368 and CDCP1-K369. Approaches that disrupt membrane localization of CDCP1 cleaving serine proteases may interfere with the cancer promoting effects of CDCP1 proteolysis.

Synthesis of deuterium-labelled analogues of NLRP3 inflammasome inhibitor MCC950.

This study describes the syntheses of di, tetra and hexa deuterated analogues of the NOD-like receptor pyrin domain-containing protein 3 (NLRP3) inflammasome inhibitor MCC950. In di and tetra deuterated analogues, deuteriums were incorporated into the 1,2,3,5,6,7-hexahydro-s-indacene moiety, whereas in the hexa deuterated MCC950 deuteriums were incorporated into the 2-(furan-3-yl)propan-2-ol moiety. The di deuterated MCC950 analogue was synthesised from 4-amino-3,5,6,7-tetrahydro-s-indacen-1(2H)-one 5. Tetra deuterated analogues were synthesised in 10 chemical steps starting with 5-bromo-2,3-dihydro-1H-inden-1-one 9, whereas the hexa deuterated analogue was synthesised in four chemical steps starting with ethyl-3-furoate 24. All of the compounds exhibited similar activity to MCC950 (IC50 = 8 nM). These deuterated analogues are useful as internal standards in LC-MS analyses of biological samples from in vivo studies.

Designing and testing an educational innovation.

Technology provides an opportunity to develop innovations to improve the way we teach. Developing educational innovations must be approached in a methodical and thorough manner to identify the educational gap and validate the innovation's success in filling the gap. A successful educational innovation requires a stepwise approach that includes needs assessment, design of intervention, testing and analysis, evaluation of intervention, and determination of learning retention.

Teaching and learning in the millennial age.

Medical education has changed and evolved over the years and has been greatly influenced by advances in technology. While the learners have also changed and the information and skills to be learned and acquired have exponentially increased, the ultimate purpose of medical education has not changed. Our focus is and has always been to improve patient care. This minisymposium highlights selected specific topics that have the potential to enhance our ability to teach and pass along essential ideas and concepts to modern learners, thus improving the health and wellbeing of patients now and in the future.