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

Pediatric pulmonary malignancy can be primary or metastatic, with the latter being by far the more common. With a few exceptions, there are no well-established evidence-based guidelines for imaging pediatric pulmonary malignancies, although computed tomography (CT) is used in almost all cases. The aim of this article is to provide general imaging guidelines for pediatric pulmonary malignancies, including minimum standards for cross-sectional imaging techniques and specific imaging recommendations for select entities.

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.

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.

Neuroblastic Tumor Recurrence Associated With Opsoclonus Myoclonus Ataxia Syndrome Relapse a Decade After Initial Resection and Treatments.

Opsoclonus myoclonus ataxia syndrome (OMAS) is a rare disorder that causes significant neurodevelopmental sequelae in children. Approximately half of pediatric OMAS cases are paraneoplastic, typically associated with localized neuroblastic tumors. Since early persistence or relapse of OMAS symptoms is common even after tumor resection, OMAS relapses may not routinely prompt reevaluation for recurrent tumors. We report a 12-year-old girl with neuroblastic tumor recurrence associated with OMAS relapse a decade after initial treatment. Providers should be aware of tumor recurrence as a trigger for distant OMAS relapse, raising intriguing questions about the role of immune surveillance and control of neuroblastic tumors.

Congenital hyperinsulinism: localization of a focal lesion with 18F-FDOPA positron emission tomography.

Hyperinsulinemic hypoglycemia of infancy, also known as congenital hyperinsulinism, is a group of disorders characterized by dysregulated insulin release. Neonates with severe, persistent hyperinsulinemic hypoglycemia who are unresponsive to medical therapy require pancreatectomy to prevent brain damage from hypoglycemia. To date, multiple genetic mutations and syndromes and several unique histopathological entities have been identified in children with hyperinsulinism. Histopathology is characterized as diffuse, focal or atypical. Surgical resection of a focal lesion results in a cure in up to 97% of these children. Imaging with 6-fluoro-(18F)-L-3,4-dihydroxyphenylalanine (18F-FDOPA) positron emission tomography (PET) is the test of choice for identifying and localizing a focal lesion and has proved to be an invaluable guide for surgical resection. Genetic evaluation is essential for determining who will benefit from PET imaging. This article provides an approach to determine who should be imaged, how to set up a protocol and how to interpret the imaging findings. The diagnosis and management of this disorder require a multidisciplinary approach to prevent brain damage from hypoglycemia.

Working from home during the COVID-19 pandemic: surveys of the Society for Pediatric Radiology and the Society of Chiefs of Radiology at Children's Hospitals.

BACKGROUND: Due to the COVID-19 pandemic, some pediatric radiologists have shifted to working from home; the long-term ramifications for pediatric radiologists and departments have not yet been defined. OBJECTIVE: To characterize experiences of working from home associated with the COVID-19 pandemic and guide expectations after the pandemic is controlled, via separate surveys of Society for Pediatric Radiology (SPR) and Society of Chiefs of Radiology at Children's Hospitals (SCORCH) members. MATERIALS AND METHODS: Two separate surveys were conducted. In the first, SPR members were surveyed Jan. 11 through Feb. 8, 2021. The response rate was 17.0% (255 of 1,501). Survey questions included demographics, information on the ability to work from home and subjective experiences ranked on a scale of 0 to 10. The survey enabled segregation and comparison of responses between those with and without home PACS. In the second survey, SCORCH members were surveyed Dec. 8, 2020, through Jan. 8, 2021. The response rate was 51.5% (51/99). Survey questions included the logistics of working from home, technical specifications and the expectations on clinical duties performed from home. The Wilcoxon rank test was used to determine statistical significance of compared variables between respondents with and without home PACS in SPR members, and expectations between SPR and SCORCH members. Descriptive statistics summarized demographic questions and free text responses. RESULTS: The majority of member respondents (81.2%, 207/255) had a home PACS and most departments provided home PACS to faculty (94.1%, 48/51). Overall, radiologists who could work from home were satisfied with their ability to work from home (mean rating: 8.3/10) and were significantly more satisfied than predicted by those without home PACS (5.9/10, P<0.0001). Respondents overwhelmingly indicated they were less able to teach trainees (mean rating: 2.7/10) and had decreased emotional engagement (mean rating: 4.4/10), but had improved research productivity and cognitive ability for research when working from home (mean rating for both: 5.3/10). Regarding the expectations of the ability to work from home after no longer needing to address the pandemic, department chairs generally favored fewer rotations from home, with 97.9% (47/48) indicating working from home should be 60% or fewer assignments, compared with 84.1% (164/195) of individual radiologists (P=0.071). CONCLUSIONS: Due to the COVID-19 pandemic, there has been a shift to working from home using PACS. Results of these SPR and SCORCH member surveys can help inform future decisions regarding pediatric radiologists working from home once the pandemic has been controlled.

Pediatric Lung MRI: Currently Available and Emerging Techniques.

OBJECTIVE. The purpose of this article is to review currently available and emerging techniques for pediatric lung MRI for general radiologists. CONCLUSION. MRI is a radiation-free alternative to CT, and clearly understanding the strengths and limitations of established and emerging techniques of pediatric lung MRI can allow practitioners to select and combine the optimal techniques, apply them in clinical practice, and potentially improve early diagnostic accuracy and patient management.

Imaging sedation and anesthesia practice patterns in pediatric radiology departments - a survey of the Society of Chiefs of Radiology at Children's Hospitals (SCORCH).

BACKGROUND: There are few data describing practice patterns related to the use of sedation/anesthesia for diagnostic imaging in pediatric radiology departments. OBJECTIVE: To understand current practice patterns related to imaging with sedation/anesthesia in pediatric radiology departments based on a survey of the Society of Chiefs of Radiology at Children's Hospitals (SCORCH) in conjunction with the American College of Radiology's Pediatric Imaging Sedation and Anesthesia Committee. MATERIALS AND METHODS: A multi-question survey related to imaging with sedation/anesthesia in pediatric radiology departments was distributed to SCORCH member institutions in January 2019. A single reminder email was sent. Descriptive statistical analyses were performed. RESULTS: Of the 84 pediatric radiology departments, 23 (27%) completed the survey. Fifty-seven percent of the respondents self-identified as academic/university-affiliated and 13% as a division/section in an adult radiology department. Imaging sedation (excluding general anesthesia) is commonly performed by pediatric anesthesiologists (76%) and intensive care unit physicians (intensivists, 48%); only 14% of departments expect their pediatric radiologists to supervise imaging sedation. Ninety-six percent of departments use child life specialists for patient preparation. Seventy percent of departments have preparatory resources available on a website, including simulation videos (26%) and audio clips (17%). Nearly half (48%) of the departments have a mock scanner to aid in patient preparation. Imaging sedation/anesthesia is most often scheduled at the request of ordering clinicians (65%), while 57% of departments allow schedulers to place patients into imaging sedation/anesthesia slots based on specified criteria. CONCLUSION: Imaging sedation/anesthesia practice patterns vary among pediatric radiology departments, and understanding current approaches can help with standardization and practice improvement.

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.

Current trends in pediatric nuclear medicine: a Society for Pediatric Radiology membership survey.

BACKGROUND: Pediatric nuclear medicine is evolving, and its practice patterns are uncertain. Knowledge of the current trends in pediatric nuclear medicine might be helpful to direct local decisions, including expectations for patient care, needs for capital acquisitions, and staffing recruitment strategies. OBJECTIVE: To provide data regarding the current practice of pediatric nuclear medicine via a Society for Pediatric Radiology (SPR) membership survey. MATERIALS AND METHODS: The SPR emailed our 25-question survey to all 1,847 SPR members in August 2018 and we accepted responses until April 2019. Questions focused on nuclear medicine staffing, positron emission tomography (PET) utilization, and radiotherapy availability. Respondents could indicate their affiliated hospital, which we used for data cataloguing only. Analysis of survey responses was blinded to reported institution. We analyzed response data using contingency tables. Independence testing between categorical variables based on proportions of physicians with additional nuclear medicine board certification was performed on a subset of questions regarding PET and therapy practices. RESULTS: Sixty-seven people from at least 29 hospitals responded to the survey, including all 10 of the 2018-2019 U.S. News & World Report best children's hospitals. The majority (48/67, 71.6%) of respondents indicated that pediatric nuclear medicine examinations were interpreted in the pediatric radiology department by pediatric radiologists and that most physicians interpreting the exams (43/67, 64.2%) did not have subspecialty certification in nuclear medicine or nuclear radiology. Most facilities offered PET/CT (57/67, 85.1%); few offered PET/MRI (12/67, 17.9%). Most facilities offered radiotherapies (57/67, 85.1%) but at most of these facilities (30/57, 52.6%), fewer than half the physicians who cover nuclear medicine were approved to administer therapies. In the subset analyses based on proportion of physicians with additional nuclear medicine board certification, there were statistically significant differences between the groups in availability of PET/MRI, lutetium-177 dotatate therapy programs, and percentages of physicians approved to administer therapies. CONCLUSION: Pediatric nuclear medicine is largely practiced by pediatric radiologists without subspecialty certification. Staffing, PET practices and therapies vary among practices, in part associated with the number of radiologists with nuclear medicine board certification.

Pediatric applications of Dotatate: early diagnostic and therapeutic experience.

In recent years, new somatostatin receptor agents (SSTRs) have become available for diagnostic imaging and therapy in neuroendocrine tumors. The novel SSTR ligand DOTA-DPhel-Tyr3-octreotate (Dotatate) in particular can be linked with 68Gallium for diagnostic imaging purposes, and with the ß-emitter 177Lutetium for radiotherapy in the setting of neuroendocrine tumors. Dotatate imaging offers distinct advantages in the evaluation of neuroendocrine tumors compared to standard techniques, including greater target-to-background ratio and lesion conspicuity, high sensitivity/specificity, improved spatial resolution with positron emission tomography (PET)/CT or PET/MR, and decreased radiation exposure. Although currently off-label in pediatrics, Dotatate theranostics in children are being explored, most notably in the setting of neuroblastoma and hereditary neuroendocrine syndromes. This article provides a multicenter case series of Dotatate imaging and therapy in pediatric patients in order to highlight the spectrum of potential clinical applications.

Clinical pediatric positron emission tomography/magnetic resonance program: a guide to successful implementation.

Children with malignancies undergo recurrent imaging as part of tumor diagnosis, staging and therapy response assessment. Simultaneous positron emission tomography (PET) and magnetic resonance (MR) allows for decreased radiation exposure and acts as a one-stop shop for disease in which MR imaging is required. Nevertheless, PET/MR is still less readily available than PET/CT across institutions. This article serves as a guide to successful implementation of a clinical pediatric PET/MR program based on our extensive clinical experience. Challenges include making scanners more affordable and increasing patient throughput by decreasing total scan time. With improvements in workflow and robust acquisition protocols, PET/MR imaging is expected to play an increasingly important role in pediatric oncology.

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.

Management of Refractory Pediatric Kaposiform Hemangioendothelioma With Sirolimus and Aspirin.

Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor characterized by aggressive local invasion and a syndrome of platelet trapping known as Kasabach-Merritt phenomenon that, through deposition of platelet derived growth factors, may perpetuate the growth of the tumor. Although many cases of KHE are successfully treated with local control or low-intensity chemotherapy, some cases are often refractory even to aggressive treatment. Herein, we describe a patient with a refractory, recurrent KHE despite multiple attempts at local control and intensive chemotherapy, that ultimately was successfully treated with rationally designed and low-intensity combination therapy of sirolimus and aspirin.

Management of adrenal masses in patients with Beckwith-Wiedemann syndrome.

Beckwith-Wiedemann syndrome (BWS) is a genetic overgrowth and cancer predisposition syndrome, associated with both benign and malignant adrenal findings. Literature review and an institutional case series elucidate the wide spectrum of adrenal findings in BWS patients. The altered expression of the 11p15 region is likely related to adrenal gland hyperplasia and growth dysregulation. Given the absence of guidelines for managing adrenal findings in BWS, we propose a systematic approach to adrenal findings in BWS patients, to allow for maximum detection of potentially malignant pathology without posing additional risk to patients.

Medical Mimics of Child Abuse.

OBJECTIVE: Physicians have an ethical and legal mandate to identify abused children so that they may be protected from further harm and are simultaneously required to think broadly and objectively about differential diagnoses. The medical literature is replete with examples of medical diseases that mimic abuse, potentially leading to misdiagnoses and subsequent harm to children and families. CONCLUSION: This review highlights some of the common and uncommon diseases that mimic physical and sexual abuse of children.

Clinical updates and surveillance recommendations for DNA replication-repair deficiency syndromes in children and young adults.

Replication repair deficiency (RRD) is a pan-cancer mechanism characterized by abnormalities in the DNA mismatch repair (MMR) system due to pathogenic variants in the PMS2, MSH6, MSH2 or MLH1 genes, and/ or in the polymerase-proofreading genes, POLE and POLD1. RRD predisposition syndromes [constitutional MMR deficiency (CMMRD), Lynch, polymerase-proofreading associated polyposis (PPAP)] share overlapping phenotypic and biological characteristics. Moreover, cancers stemming from germline defects of one mechanism can acquire somatic defects in another, leading to complete RRD. Here we describe the recent advances in the diagnostics, surveillance, and clinical management for children with RRD syndromes. For patients with CMMRD, new data combining clinical insights and cancer genomics have revealed genotype-phenotype associations, helped in the development of novel functional assays, diagnostic guidelines, and surveillance recommendations. Recognition of non-gastrointestinal/ genitourinary malignancies, particularly aggressive brain tumors, in select children with Lynch and PPAP syndromes harbouring an RRD biology have led to new management considerations. Additionally, universal hypermutation and microsatellite instability have allowed immunotherapy to be a paradigm shift in the treatment of RRD cancers independent of their germline etiology. These advances have also stimulated a need for expert recommendations regarding genetic counselling for these patients and their families. Future collaborative work will focus on newer technologies such as quantitative measurement of circulating tumor DNA and functional genomics to tailor surveillance and clinical care, improving immune surveillance, develop prevention strategies, and deliver these novel discoveries to resource-limited settings to maximize benefits for patients globally.

Clinical features of three girls with mosaic genome-wide paternal uniparental isodisomy.

Here we describe three subjects with mosaic genome-wide paternal uniparental isodisomy (GWpUPD) each of whom presented initially with overgrowth, hemihyperplasia (HH), and hyperinsulinism (HI). Due to the severity of findings and the presence of additional features, SNP array testing was performed, which demonstrated mosaic GWpUPD. Comparing these individuals to 10 other live-born subjects reported in the literature, the predominant phenotype is that of pUPD11 and notable for a very high incidence of tumor development. Our subjects developed non-metastatic tumors of the adrenal gland, kidney, and/or liver. All three subjects had pancreatic hyperplasia resulting in HI. Notably, our subjects to date display minimal features of other diseases associated with paternal UPD loci. Both children who survived the neonatal period have displayed near-normal cognitive development, likely due to a favorable tissue distribution of the mosaicism. To understand the range of UPD mosaicism levels, we studied multiple tissues using SNP array analysis and detected levels of 5-95%, roughly correlating with the extent of tissue involvement. Given the rapidity of tumor growth and the difficulty distinguishing malignant and benign tumors in these GWpUPD subjects, we have utilized increased frequency of ultrasound (US) and alpha-fetoprotein (AFP) screening in the first years of life. Because of a later age of onset of additional tumors, continued tumor surveillance into adolescence may need to be considered in these rare patients.