Prognostic impact of lymph node involvement and loss of heterozygosity of 1p or 16q in stage III favorable histology Wilms tumor: A report from Children's Oncology Group Studies AREN03B2 and AREN0532.

INTRODUCTION: The prognostic impact of positive lymph nodes (LN+) and/or singular loss of heterozygosity (LOH) of 1p or 16q were assessed in children with stage III favorable histology Wilms tumor (FHWT) enrolled on AREN0532 or AREN03B2 alone. PATIENTS AND METHODS: A total of 635 stage III FHWT vincristine/dactinomycin/doxorubicin (DD4A)-treated patients met inclusion criteria. Event-free survival (EFS) and overall survival are reported overall and by LN sampling, LN status, LOH 1p, LOH 16q, and a combination of LN status and singular LOH. Patients with unknown or positive combined LOH of 1p and 16q status and AREN03B2-only patients with unknown outcomes or treatment other than DD4A were excluded. RESULTS: EFS did not differ by study, supporting pooling. Lack of LN sampling (hazard ratio [HR], 2.12; p = .0037), LN positivity (HR, 2.78; p = .0002), LOH 1p (HR, 2.18; p = .0067), and LOH 16q (HR, 1.72; p = .042) were associated with worse EFS. Compared with patients with both LN- and LOH-, those with negative nodes but positive LOH 1p or 16q and those with LN+ but LOH- for 1p or 16q had significantly worse EFS (HR, 3.05 and 3.57, respectively). Patients positive for both LN and LOH had the worst EFS (HR, 6.33; overall group factor, p < .0001). CONCLUSION: Findings confirm LN+ status as an adverse prognostic factor amplified by presence of singular LOH 1p or 16q, supporting study of intensified therapy for patients with LN+ in combination with singular LOH in a prospective clinical trial.

Imaging of pediatric pancreas tumors: A COG Diagnostic Imaging Committee/SPR Oncology Committee White Paper.

Primary pancreatic tumors in children are rare with an overall age-adjusted incidence of 0.018 new cases per 100,000 pediatric patients. The most prevalent histologic type is the solid pseudopapillary neoplasm, followed by pancreatoblastoma. This paper describes relevant imaging modalities and presents consensus-based recommendations for imaging at diagnosis and follow-up.

Similarities and controversies in imaging of pediatric renal tumors: A SIOP-RTSG and COG collaboration.

Malignant renal tumors are rare in children, and Wilms tumors (WTs) are the most common subtype. Imaging plays an essential role in the diagnosis, staging, and follow-up of these patients. Initial workup for staging is mainly performed by cross-sectional imaging modalities such as computed tomography (CT) and magnetic resonance imaging (MRI). Imaging approach within the two core international groups, the Children's Oncology Group (COG, North America) and the International Society of Pediatric Oncology - Renal Tumor Study Group (SIOP-RTSG, Europe), differs. Whereas abdominal ultrasound (US) is used for the initial diagnosis of a suspected pediatric renal tumor globally, COG protocols support the use of CT or MRI for locoregional staging, contrary to the preference for MRI over CT for abdominopelvic evaluation within the SIOP-RTSG. The purpose of this manuscript is to summarize current imaging approaches, highlighting differences and similarities within these core international groups, while focusing on future innovative efforts and collaboration within the HARMONICA initiative.

Early continuous renal replacement therapy during infant extracorporeal life support is associated with decreased lung opacification.

Lung opacification on chest radiography (CXR) is common during extracorporeal life support (ECLS), often resulting from pulmonary edema or inflammation. Concurrent use of continuous renal replacement therapy (CRRT) during ECLS is associated with improved fluid balance and cytokine filtration; through modification of these pathologic states, CRRT may modulate lung opacification observed on CXRs. We hypothesize that early CRRT use during infant ECLS decreases lung opacification on CXR. We conducted a retrospective cohort study comparing CXRs from infants receiving ECLS and early CRRT (n = 7) to matched infants who received ECLS alone (n = 7). The CXR obtained prior to ECLS, all CXRs obtained within the first 72 h of ECLS, and daily CXRs for the remainder of the ECLS course were analyzed. The outcome measure was the degree of opacification, determined by independent assessment of two, blinded pediatric radiologists using a modified Edwards et al.'s lung opacification scoring system (from Score 0: no opacification to Score 5: complete opacification). 220 CXRs were assessed (cases: 93, controls: 127). Inter-rater reliability was established (Cohen's weighted к = 0.74; p < 0.0001, good agreement). At baseline, the mean opacification score difference between cases and controls was 1 point (cases: 1.8, controls 2.8; p = 0.049). Using mixed modeling analysis for repeated measures accounting for differences at baseline, the average overall opacification score was 1.2 points lower in cases than controls (cases: 2.1, controls: 3.3; p < 0.0001). The overall distribution of scores was lower in cases than controls. Early CRRT utilization during infant ECLS was associated with decreased lung opacification on CXR.

A survey of pediatric diagnostic radiologists in North America: current practices in fetal magnetic resonance imaging.

BACKGROUND: Fetal magnetic resonance imaging (MRI) is an imaging examination in evolution. Rapid developments over recent decades have led to better image quality, an increased number of examinations and greater impact on patient care. OBJECTIVE: To gather data regarding current practices among established programs in North America and provide information to radiologists interested in implementing or growing a fetal MRI service. MATERIALS AND METHODS: An electronic survey containing 15 questions relevant to the use of fetal MRI was submitted to pediatric radiologists and neuroradiologists. Items regarded scheduling and reporting logistics, magnet strength, patient positioning and patient preparation. Answers and comments were collected, and descriptive statistics were summarized. RESULTS: One hundred and six survey responses were evaluated. Of the survey responses, 62/106 (58.5%) allow fetal MR scheduling any time during the day and 72/105 (68.6%) exclusively use 1.5-T strength platforms for fetal MRI, while only 7/105 (6.7%) use exclusively 3 T. Patient positioning is variable: supine, 40/106 (37.8%); left lateral decubitus, 22/106 (20.8%), and, patient's choice, 43/106 (40.6%). Of the centers responding, 51/104 (49.0%) require no particular fasting instructions, while 20/104 (19.2%) request the patient avoid caffeine before the scanning. CONCLUSION: Logistical trends in performing fetal MRI may supplement the American College of Radiology's published technical standards and offer guidance to radiologists new to the field.

Updates in Diagnosis, Management, and Treatment of Neuroblastoma.

Neuroblastoma is an embryonic tumor of the peripheral sympathetic nervous system. It is the most common extracranial solid tumor of childhood and accounts for up to 15% of all pediatric cancer fatalities. The manifestation of neuroblastoma is variable depending on the location of the tumor and on the presence or absence of paraneoplastic syndromes. The prognosis of neuroblastoma is also highly variable, ranging from spontaneous regression to widespread metastatic disease that is unresponsive to treatment. The age of the patient, stage of disease, histopathologic results, and multiple biologic factors contribute to the presurgical and pretreatment risk stratification of a patient with neuroblastoma. Multimodality anatomic imaging with ultrasonography, computed tomography, and magnetic resonance imaging, as well as functional or metabolic nuclear imaging, are essential to determining the risk status of a patient with neuroblastoma. Patients at low risk of metastasis or death receive minimal intervention and those at high risk receive multimodality treatment. New immunotherapeutic techniques and nuclear medicine-targeted therapies have emerged and are demonstrating promising response rates for patients at high risk. This article reviews updates in the diagnosis, management, and treatment of neuroblastoma that have evolved over the past 2 decades, including emphasis on presurgical risk stratification, genetic evaluation of tumors, and the use of modern, high-quality, advanced imaging modalities. ©RSNA, 2018.

Pre-procedural scout radiographs are unnecessary for routine pediatric fluoroscopic examinations.

BACKGROUND: Although practice patterns vary, scout radiographs are often routinely performed with pediatric fluoroscopic studies. However few studies have evaluated their utility in routine pediatric fluoroscopy. OBJECTIVE: To evaluate the value of scout abdomen radiographs in routine barium or water-soluble enema, upper gastrointestinal (GI) series, and voiding cystourethrogram pediatric fluoroscopic procedures. MATERIALS AND METHODS: We retrospectively evaluated 723 barium or water-soluble enema, upper GI series, and voiding cystourethrogram fluoroscopic procedures performed at our institution. We assessed patient history and demographics, clinical indication for the examination, prior imaging findings and impressions, scout radiograph findings, additional findings provided by the scout radiograph that were previously unknown, and whether the scout radiograph contributed any findings that significantly changed management. RESULTS: We retrospectively evaluated 723 fluoroscopic studies (368 males and 355 females) in pediatric patients. Of these, 700 (96.8%) had a preliminary scout radiograph. Twenty-three (3.2%) had a same-day radiograph substituted as a scout radiograph. Preliminary scout abdomen radiographs/same-day radiographs showed no new significant findings in 719 (99.4%) studies. New but clinically insignificant findings were seen in 4 (0.6%) studies and included umbilical hernia, inguinal hernia and hip dysplasia. No findings were found on the scout radiographs that would either alter the examination performed or change management with regard to the exam. CONCLUSION: Pre-procedural scout abdomen radiographs are unnecessary in routine barium and water-soluble enema, upper GI series, and voiding cystourethrogram pediatric fluoroscopic procedures and can be substituted with a spot fluoroscopic last-image hold.

Atypical accessory intraparietal sutures mimicking complex fractures in a neonate.

Partial or complete division of the parietal bones resulting in anomalous cranial sutures is a rare entity and may raise concern for fracture and potential abuse when identified on radiological examination in young children. We present a case of a 4-week-old male found to have anomalous intraparietal sutures originally interpreted as fractures during a comprehensive evaluation for nonaccidental trauma. Our goal is to raise awareness of a complex branching pattern of accessory intraparietal sutures, which has not been previously described. Additionally, we will review the characteristics that aid in the radiologic differentiation of accessory cranial sutures and fractures.

Open Partial Nephrectomy for Wilms' Tumor in a Cross-fused Pelvic Ectopic Kidney.

Wilms' tumor is the most common pediatric solid renal tumor. Cross-fused renal ectopia is a rare congenital anomaly in which the left and right kidneys become fused and fail to ascend from the pelvis and abdomen. We report a case of a 5-year-old girl that underwent open partial nephrectomy on a cross-fused ectopic kidney, "pancake kidney," after incidental discovery of a solid renal mass found to be a Wilms' tumor. Thorough review of the literature shows that this combination of Wilms' tumor in the setting of cross-fused renal ectopia has only been reported twice previously.

Desmoplastic small round cell tumor of the kidney with unusual imaging features.

Desmoplastic small round cell tumor (DSRCT) is a rare, high-grade sarcoma seen in children and young adults. DSRCT of the kidney is extremely uncommon, but has been described in both pediatric and adult populations. Here we present an unusual manifestation of renal DSRCT in a boy whose imaging studies revealed a well-circumscribed mass confined to the urinary collecting system, rather than a large aggressive-appearing mass typically associated with DSRCT, which highlights the varied clinical and imaging features of this disease.

Malignant degeneration of pulmonary juvenile-onset recurrent respiratory papillomatosis.

Juvenile-onset recurrent respiratory papillomatosis (JORRP) is a rare disease associated with the human papilloma virus (HPV) in which papillomas form along the aerodigestive tract in children. Pulmonary involvement is uncommon, but associated with worse clinical outcomes, including the rare complication of malignant transformation. We present a patient with JORRP in which lung disease underwent malignant transformation during adolescence. Our goal is to raise awareness of the potential for malignant transformation in children, as well as to familiarize pediatric radiologists with imaging features of malignant lung disease in JORRP. We advocate for the identification of the subgroup of JORPP patients with pulmonary disease who, due to increased risk for malignant transformation, may benefit from closer clinical and imaging surveillance by a multidisciplinary team.

Atypical squamous cells, cannot exclude HSIL: a review of original vs. duplicate thin-layer slides.

OBJECTIVE: To assess the utility of consensus review of original ThinPrep slides (Cytyc Corp., Boxborough, Masssachusetts) vs. duplicate slides and to identify the significance of the number of representative cells in classifying atypical squamous metaplastic cells, cannot exclude high grade squamous intraepithelial lesion (ASC-H). STUDY DESIGN: A duplicate ThinPrep slide was prepared from 19 cases of ASC-H. Both original and duplicate slides underwent blinded review by 4 pathologists. Consensus cytologic interpretations (75% agreement) were noted and compared with follow-up diagnoses. Dots with atypical metaplastic cells from each slide were counted and correlated with the consensus cytologic interpretation. RESULTS: Following review of 19 original and 19 duplicate slides, consensus interpretations were reached in 14 and 15 cases, respectively. Nine cases of squamous intraepithelial lesion (SIL) were confirmed by biopsies. Consensus interpretation of original and duplicate slides predicted 7 and 6 cases of SIL, respectively. Consensus interpretation of duplicate slides predicted 60% of histologically benign cases. Two-thirds of nonconsensus cases were histologically benign. Consensus interpretations for high grade SIL were reached more often on slides with greater numbers of atypical squamous metaplastic cells. Fewer abnormal cells were present on the duplicate slides. CONCLUSION: Consensus reviews of original and duplicate slides are equally useful in classifying atypical squamous cells, cannot exclude high grade squamous intraepithelial lesion, but may not be practical in all circumstances. HPV DNA testing of all such cases may better detect clinically significant cases.