CD43-positive, EWSR1::FLI1 -rearranged Soft Tissue Sarcoma in a Pediatric Patient With History of B-Cell Acute Lymphoblastic Leukemia.

Ewing sarcoma is a small round blue cell tumor typically characterized by an EWSR1 rearrangement and expression of CD99 and NKX2.2, without expression of hematopoietic markers such as CD45. CD43 is an alternative hematopoietic immunohistochemical marker often utilized in the workup of these tumors and its expression typically argues against Ewing sarcoma. We report a 10-year-old with history of B-cell acute lymphoblastic leukemia presenting with an unusual malignant shoulder mass with variable CD43 positivity, but with an EWSR1::FLI1 fusion detected by RNA sequencing. Her challenging workup highlights the utility of next-generation DNA-based and RNA-based sequencing methods in cases with unclear or conflicting immunohistochemical results.

Safety considerations related to intravenous contrast agents in pediatric imaging.

Intravenous contrast media are used in MRI, CT and US studies for anatomical evaluation and lesion characterization. Safety is always of paramount importance when administering any contrast media to children, and it is important for radiologists and ordering providers to be knowledgeable of the safety profiles and potential adverse events that can occur. This manuscript reviews the frequency and types of adverse events associated with intravenous contrast agents reported in the pediatric literature. Overall, intravenous contrast agents are very safe to use in children. However, familiarity with how to treat and prevent these uncommon events is crucial in preventing poor outcomes. In addition, an understanding of gadolinium deposition in tissues can help facilitate conversations with concerned physicians and parents. This review provides a concise yet comprehensive reference for radiologists and ordering providers on intravenous contrast safety considerations in the pediatric patient.

Frequency of technical success of ultrasound-guided arthrogram injections in children.

BACKGROUND: MR arthrography is an essential diagnostic tool to assess and guide management of labral, ligamentous, fibrocartilaginous, and capsular abnormalities in children. While fluoroscopy is traditionally used for intra-articular contrast administration, ultrasound offers advantages of portability and lack of ionizing radiation exposure for both the patient and proceduralist. OBJECTIVE: The purpose of this retrospective study is to quantify technical success and frequency of complications of ultrasound-guided arthrogram injections at our institution. MATERIALS AND METHODS: This retrospective analysis investigates the results of 217 ultrasound-guided arthrograms of the shoulder, elbow, and hip in patients aged 5-18 years. Successful injection of contrast into the target joint, clinical indication for MR arthrography, and complications were reviewed. RESULTS: Accurate ultrasound-guided intra-articular administration of contrast into the target joint was successful for 100% of shoulder cases (90/90), 97% of elbow cases (77/79), and 98% of hip cases (47/48). Leak of contrast outside the target joint occurred in 1.4% (3/217) of cases. No major side effects including excessive bleeding, paresthesia, allergic reactions, or infection occurred during or after the procedure. Additionally, no major vessel, nerve, or tendon complications were observed on MR images. CONCLUSION: Ultrasound guidance is a reliable, effective, and safe approach to arthrography in children.

Update of pediatric bone tumors: osteogenic tumors and osteoclastic giant cell-rich tumors.

There are numerous bone tumors in the pediatric population, with imaging playing an essential role in diagnosis and management. Our understanding of certain bone tumors has rapidly evolved over the past decade with advancements in next-generation genetic sequencing techniques. This increased level of understanding has altered the nomenclature, management approach, and prognosis of certain lesions. We provide a detailed update of bone tumors that occur in the pediatric population with emphasis on the recently released nomenclature provided in the 5th edition of the World Health Organization Classification of Soft Tissue and Bone Tumours.

Update of pediatric bone tumors-other mesenchymal tumors of bone, hematopoietic neoplasms of bone, and WHO classification of undifferentiated small round cell sarcomas of bone.

There are numerous bone tumors in the pediatric population, with imaging playing an essential role in diagnosis and management. Our understanding of certain bone tumors has rapidly evolved over the past decade with advancements in next-generation genetic sequencing techniques. This increased level of understanding has altered the nomenclature, management approach, and prognosis of certain lesions. We provide a detailed update of bone tumors that occur in the pediatric population with emphasis on the recently released nomenclature provided in the 5th edition of the World Health Organization Classification of Soft Tissue and Bone Tumours. We discuss other mesenchymal tumors of bone, hematopoietic neoplasms of bone, and WHO classification of undifferentiated small round cell sarcomas of bone. We have detailed osteogenic tumors and osteoclastic giant cell-rich tumors, as well as notochordal tumors, chondrogenic tumors, and vascular tumors of the bone in separate manuscripts.

Update of pediatric bone tumors-notochordal tumors, chondrogenic tumors, and vascular tumors of the bone.

There are numerous bone tumors in the pediatric population, with imaging playing an essential role in diagnosis and management. Our understanding of certain bone tumors has rapidly evolved over the past decade with advancements in next-generation genetic sequencing techniques. This increased level of understanding has altered the nomenclature, management approach, and prognosis of certain lesions. We provide a detailed update of bone tumors that occur in the pediatric population with emphasis on the recently released nomenclature provided in the 5th edition of the World Health Organization Classification of Soft Tissue and Bone Tumours. In the current manuscript, we address notochordal tumors, chondrogenic tumors, and vascular tumors of the bone.

Whole-body MRI at initial presentation of pediatric chronic recurrent multifocal osteomyelitis and correlation with clinical assessment.

BACKGROUND: Chronic recurrent multifocal osteomyelitis (CRMO) is a diagnosis of exclusion, relying heavily on whole-body magnetic resonance imaging (WB-MRI) for diagnosing and evaluating response to therapy. Information with respect to disease distribution and imaging correlation with clinical disease severity at initial presentation is lacking. OBJECTIVE: To retrospectively characterize distribution of disease on WB-MRI and to correlate imaging findings with disease severity at initial rheumatology presentation. MATERIALS AND METHODS: Using a modified version of a recently devised imaging-based scoring system, we evaluated disease distribution and correlation between findings on WB-MRI and clinical disease severity in 54 patients presenting for initial evaluation of CRMO. Symptomatic lesion sites were extracted from chart review and physician global assessment was determined by the consensus of two rheumatologists. RESULTS: Sites of CRMO involvement evident on imaging at initial presentation had a strong predilection for the pelvis and lower extremities. There was significant correlation between the number of lesions detected on WB-MRI and total clinical severity score at initial rheumatology presentation (P<0.01). However, no other imaging parameter correlated with disease severity. CONCLUSION: While the overall number of lesions identified on MRI correlates with clinical severity scores at initial imaging, other MR parameters of CRMO lesions may not be reliable indicators of disease severity at initial presentation. Further research is needed to assess whether these parameters are implicated in longitudinal disease severity or overall response to therapy.

Palpable pediatric chest wall masses.

Pediatric chest wall lesions are varied in etiology ranging from normal and benign to aggressive and malignant. When palpable, these lesions can alarm parents and clinicians alike. However, most palpable pediatric chest lesions are benign. Familiarity with the various entities, their incidences, and how to evaluate them with imaging is important for clinicians and radiologists. Here we review the most relevant palpable pediatric chest entities, their expected appearance and the specific clinical issues to aid in diagnosis and appropriate treatment.

Gadolinium retention: should pediatric radiologists be concerned, and how to frame conversations with families.

Gadolinium retention in the brain and other organs has recently been identified by imaging and confirmed histologically. No direct clinical effects of gadolinium retention, which occurs after gadolinium-based contrast agent (GBCA) administration for MRI, have been scientifically accepted at this time. However, there is understandable concern among medical professionals and the public about the potential effects of gadolinium retention, particularly in the brain. Part of this concern might stem from the identification of nephrogenic systemic fibrosis caused by GBCAs in people with severe renal failure in 2006. This article briefly describes the characteristics of GBCAs; reviews and differentiates gadolinium retention, nephrogenic systemic fibrosis, and "gadolinium deposition disease" or "gadolinium toxicity"; and discusses societal guidelines and current usage in children. With the belief that GBCAs should not be withheld for appropriate indications in the absence of evidence of its potential risks, we offer a framework for determining when GBCA use is appropriate and suggestions for discussing its risks and benefits with children and their families.

Non-inferiority of a non-gadolinium-enhanced magnetic resonance imaging follow-up protocol for isolated optic pathway gliomas.

BACKGROUND: Pediatric patients with optic pathway gliomas (OPGs) typically undergo a large number of follow-up MRI brain exams with gadolinium-based contrast media (GBCM), which have been associated with gadolinium tissue retention. Therefore, careful consideration of GBCM use in these children is warranted. OBJECTIVE: To investigate whether GBCM is necessary for OPG MR imaging response assessment using a blinded, non-inferiority, multi-reader study. MATERIALS AND METHODS: We identified children with OPG and either stable disease or change in tumor size on MRI using a regional cancer registry serving the U.S. Pacific Northwest. For each child, the two relevant, consecutive MRI studies were anonymized and standardized into two imaging sets excluding or including GBCM-enhanced images. Exam pairs were compiled from 42 children with isolated OPG (19 with neurofibromatosis type 1), from a population of 106 children with OPG. We included 28 exam pairs in which there was a change in size between exams. Seven pediatric radiologists measured tumor sizes during three blinded sessions, spaced by at least 1 week. The first measuring session excluded GBCM-enhanced sequences; the others did not. The primary endpoint was intra-reader agreement for ≥ 25% change in axial cross-product measurement, using a 12% non-inferiority threshold. RESULTS: Analysis demonstrated an overall 1.2% difference (95% confidence interval, -3.2% to 5.5%) for intra-reader agreement using a non-GBCM-enhanced protocol and background variability. CONCLUSION: A non-GBCM-enhanced protocol was non-inferior to a GBCM-enhanced protocol for assessing change in size of isolated OPGs on follow-up MRI exams.

Update of pediatric soft tissue tumors with review of conventional MRI appearance-part 1: tumor-like lesions, adipocytic tumors, fibroblastic and myofibroblastic tumors, and perivascular tumors.

There are numerous soft tissue tumors and tumor-like conditions in the pediatric population. Magnetic resonance imaging is the most useful modality for imaging these lesions. Although certain soft tissue lesions exhibit magnetic resonance features characteristic of a specific diagnosis, most lesions are indeterminate, and a biopsy is necessary for diagnosis. We provide a detailed update of soft tissue tumors and tumor-like conditions that occur in the pediatric population, emphasizing each lesion's conventional magnetic resonance imaging appearance, using the recently released 5th edition of the World Health Organization Classification of Soft Tissue and Bone Tumors as a guide. In part one of this review, pediatric tumor-like lesions, adipocytic tumors, fibroblastic and myofibroblastic tumors, and perivascular tumors are discussed. In part two, vascular lesions, fibrohistiocytic tumors, muscle tumors, peripheral nerve sheath tumors, tumors of uncertain differentiation, and undifferentiated small round cell sarcomas are reviewed. Per the convention of the WHO, these lesions involve the connective, subcutaneous, and other non-parenchymatous-organ soft tissues, as well as the peripheral and autonomic nervous system.

Update of pediatric soft tissue tumors with review of conventional MRI appearance-part 2: vascular lesions, fibrohistiocytic tumors, muscle tumors, peripheral nerve sheath tumors, tumors of uncertain differentiation, and undifferentiated small round cell sarcomas.

There are numerous soft tissue tumors and tumor-like conditions in the pediatric population. Magnetic resonance imaging is the most useful modality for imaging these lesions. Although certain soft tissue lesions exhibit magnetic resonance features characteristic of a specific diagnosis, most lesions are indeterminate, and a biopsy is necessary for diagnosis. We provide a detailed update of soft tissue tumors and tumor-like conditions that occur in the pediatric population, emphasizing each lesion's conventional magnetic resonance imaging appearance, using the recently released 5th edition of the World Health Organization Classification of Soft Tissue and Bone Tumors as a guide. In part one of this review, pediatric tumor-like lesions, adipocytic tumors, fibroblastic and myofibroblastic tumors, and perivascular tumors are discussed. In part two, vascular lesions, fibrohistiocytic tumors, muscle tumors, peripheral nerve sheath tumors, tumors of uncertain differentiation, and undifferentiated small round cell sarcomas are reviewed. Per the convention of the WHO, these lesions involve the connective, subcutaneous, and other non-parenchymatous organ soft tissues, as well as the peripheral and autonomic nervous system.

Contemporary imaging of the pediatric shoulder: pearls and pitfalls.

In skeletally immature patients, the presence of growth plates and articular cartilage of the shoulder can create a predisposition for unique injuries not observed in adults. Furthermore, increasing participation in sports by children and adolescents appears to be leading to a corresponding increase in the number of sports-related injuries. The importance of radiologists being familiar with pediatric shoulder imaging and its associated injuries is therefore growing. In this article, we review the normal development and maturation pattern of ossification centers of the shoulder from the early gestational period through adolescence. Brachial plexus birth palsy, physeal injuries, shoulder dislocation, and internal impingement are discussed within the context of the child's age and the mechanism of injury to guide radiologists to a correct diagnosis.

Foot and Ankle Musculoskeletal Imaging of Pediatric Patients With Cerebral Palsy.

OBJECTIVE. Any combination of abnormal positioning of the ankle, hindfoot, midfoot, and forefoot is possible in the context of cerebral palsy, but some patterns are more common than others. The purpose of this article is to discuss the radiographic manifestations and surgical management of the following common conditions: equinus, equinoplanovalgus, equinocavovarus, vertical talus, oblique talus, hallux valgus, and ankle valgus. CONCLUSION. CP is defined by abnormalities of the developing fetal or infant brain that result in permanent central motor dysfunction. Foot and ankle deformities are very common in the patients with CP, occurring in up to 93% of such patients as a result of underlying abnormal muscle tone, impaired motor control, and dynamic muscle imbalance. Radiologists must develop knowledge of the most common changes in alignment and basic techniques for correction to better recognize abnormalities and improve communication with orthopedic colleagues.

Clarifying radiology's role in safety events: a 5-year retrospective common cause analysis of safety events at a pediatric hospital.

BACKGROUND: Common cause analysis of hospital safety events that involve radiology can identify opportunities to improve quality of care and patient safety. OBJECTIVE: To study the most frequent system failures as well as key activities and processes identified in safety events in an academic children's hospital that underwent root cause analysis and in which radiology was determined to play a contributing role. MATERIALS AND METHODS: All safety events involving diagnostic or interventional radiology from April 2013 to November 2018, for which the hospital patient safety department conducted root cause analysis, were retrospectively analyzed. Pareto charts were constructed to identify the most frequent modalities, system failure modes, key processes and key activities. RESULTS: In 19 safety events, 64 sequential interactions were attributed to the radiology department by the patient safety department. Five of these safety events were secondary to diagnostic errors. Interventional radiology, radiography and diagnostic fluoroscopy accounted for 89.5% of the modalities in these safety events. Culture and process accounted for 55% of the system failure modes. The three most common key processes involved in these sequential interactions were diagnostic (39.1%) and procedural services (25%), followed by coordinating care and services (18.8%). The two most common key activities were interpreting/analyzing (21.9%) and coordinating activities (15.6%). CONCLUSION: Proposing and implementing solutions based on the analysis of a single safety event may not be a robust strategy for process improvement. Common cause analyses of safety events allow for a more robust understanding of system failures and have the potential to generate more specific process improvement strategies to prevent the reoccurrence of similar errors. Our analysis demonstrated that the most common system failure modes in safety events attributed to radiology were culture and process. However, the generalizability of these findings is limited given our small sample size. Aligning with other children's hospitals to use standard safety event terminology and shared databases will likely lead to greater clarity on radiology's direct and indirect contributions to patient harm.

Brain tissue gadolinium retention in pediatric patients after contrast-enhanced magnetic resonance exams: pathological confirmation.

BACKGROUND: Retained gadolinium from gadolinium-based contrast agents (GBCAs) used in MR exams has been inferred based on signal changes on serial brain MRI and subsequently demonstrated pathologically in adults. Retention has been similarly inferred in children but pathological demonstration in pediatric patients is limited. The long-term effects of retained gadolinium are unknown but are potentially of greater concern in children given their increased vulnerability from continuing development and their expected longer period of exposure. Several factors can influence gadolinium retention. In adults as well as in children, greater accumulation has been demonstrated based on MR signal changes with linear compared with macrocyclic gadolinium chelates, attributed to lower chelate affinity with linear agents. Effects of age at exposure on retention are unknown, while differences in GBCA washout rates are still under investigation and might affect gadolinium retention relative to time of GBCA administration. OBJECTIVE: The purpose of this study was to confirm whether gadolinium brain deposits are present in pediatric patients who received GBCAs and to quantify the amounts present. MATERIALS AND METHODS: Brain autopsy specimens from 10 pediatric patients between 1 year and 13 years of age who underwent at least one contrast-enhanced MR exam were analyzed for elemental gadolinium using inductively coupled plasma mass spectrometry. Brain samples included white matter, basal ganglia (putamen, globus pallidus), thalamus, dentate nucleus and tumor tissue as available. Type and dose of contrast agent, number and timing of contrast-enhanced MR exams and renal function (estimated glomerular filtration rate [eGFR]) were documented for each child. RESULTS: Patient exposures ranged from 1 dose to 20 doses of GBCAs including both macrocyclic and linear ionic agents. Gadolinium was found to be present in brain tissue in all children and was generally highest in the globus pallidus. Those who received only macrocyclic agents showed lower levels of gadolinium retention. CONCLUSION: This study demonstrates pathological confirmation of gadolinium retention in brain tissue of a series of pediatric patients exposed to GBCAs including not only linear ionic agents but also macrocyclic agents with both nonionic and ionic compounds. The distribution and deposition levels in this small pediatric population are comparable with the findings in adults. While the clinical significance of these deposits remains unknown, at this point it would be prudent to exert caution and avoid unnecessary use of GBCAs in pediatric patients.

Practical administration of intravenous contrast media in children: screening, prophylaxis, administration and treatment of adverse reactions.

Administration of intravenous contrast media to children is a routine practice at many clinical imaging centers, that can involve special considerations. In this paper, we provide practical information to facilitate optimal performance and oversight of this task. We provide targeted screening questions that can help to identify high-risk pediatric patients for both iodine-based and gadolinium-based intravenous contrast media administration. These include children at risk for allergic-like reactions, thyroid dysfunction, contrast-induced nephropathy, and nephrogenic systemic fibrosis. We make recommendations for addressing "yes" responses to screening questions using risk stratification schema that are specific to children. We also present criteria for selecting children for premedication prior to intravenous contrast administration, and suggest pediatric regimens. Additionally, we discuss practical nuances of intravenous contrast media administration to children and provide a quick-reference table of appropriate treatments with pediatric dosages for adverse contrast reactions.

The Pediatric Patella: Normal Development, Anatomical Variants and Malformations, Stability, Imaging, and Injury Patterns.

We discuss the pediatric patella, with an emphasis on diagnostic imaging. Topics include normal patellar development, anatomical variants and their physiologic significance, genetic syndromes that alter the appearance of the patella, physiology of patellar tracking and stability, patellofemoral instability, and injury patterns and classification. Recognition of appropriate development on imaging prevents diagnostic error and unnecessary evaluation. Knowledge of the pertinent features of syndromes associated with morphological patellar abnormalities can aid in generating a succinct and relevant differential diagnosis. In patellofemoral instability, the patient's baseline anatomy, factors that predispose to instability, and the specific injuries that occur as a result are critical considerations for determining the course of treatment. Patellar sleeve fractures are unique to pediatric patients, and timely identification is critical to achieving an optimal treatment outcome.

Anatomy and injuries of the pediatric wrist: beyond the basics.

Ligamentous injuries of the pediatric wrist, once thought to be relatively uncommon, are increasingly recognized in the context of acute high-energy mechanism trauma and chronic axial loading, including those encountered in both recreational and high-performance competitive sports. Recent advances in MR-based techniques for imaging the pediatric wrist allow for sensitive identification of these often radiographically occult injuries. Detailed knowledge of the intrinsic and supportive extrinsic ligamentous complexes, as well as normal developmental anatomy and congenital variation, are essential to accurately diagnose injuries to these structures. Early identification of ligamentous injury of the pediatric wrist is essential within the conservative treatment culture of modern pediatric orthopedics because treatment of these lesions often necessitates surgery, and outcomes often depend on early and sometimes aggressive intervention. In this article, we review MR arthrogram technique and pediatric wrist anatomy, and correlate appearances on MR and selected ligamentous pathologies of the pediatric wrist.

The central nervous system manifestations of localized craniofacial scleroderma: a study of 10 cases and literature review.

BACKGROUND: Localized craniofacial scleroderma is a rare pediatric disease that involves a spectrum of discoloration, fibrosis and hemiatrophy of the face and scalp. Children with localized craniofacial scleroderma may have neurological symptoms, and in this context often undergo diagnostic imaging of the brain. OBJECTIVE: To catalogue neuroimaging abnormalities in patients with localized craniofacial scleroderma treated at our institution, review their clinical courses and compare this data with prior studies. MATERIALS AND METHODS: Following Institutional Review Board approval, an imaging database search identified 10 patients with localized craniofacial scleroderma and neuroimaging abnormalities treated at our institution. Neuroimaging exams and the electronic medical record were reviewed for each case. RESULTS: The most common indications for neuroimaging were headache or seizure (80% of cases). The most common neuroimaging abnormalities were T2-hyperintense, subcortical white matter lesions ipsilateral to the cutaneous lesion (90% of cases) on magnetic resonance imaging (MRI). Calcifications or blood products (50%), cysts (40%) and abnormal enhancement (20%) were also observed. A positron emission tomography (PET) scan obtained for a single case demonstrated diminished 18F-fluorodeoxyglucose (FDG) avidity corresponding to the dominant focus of signal abnormality on MRI. Progressive neuroimaging abnormalities were present in 30% of cases. There was no consistent relationship between changes in neurological symptoms following treatment and neuroimaging findings. CONCLUSION: Our results are similar to previously published data. In the absence of new or worsening neurological symptoms, the role of neuroimaging for follow-up of localized craniofacial scleroderma is unclear. Knowledge of intracranial neuroimaging abnormalities that are commonly associated with localized craniofacial scleroderma helps to distinguish these lesions from others that have similar appearance.