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Primary hepatic malignancies are relatively rare in the pediatric population, accounting for approximately 1%-2% of all pediatric tumors. Hepatoblastoma and hepatocellular carcinoma are the most common primary liver malignancies in children under the age of 5 years and over the age of 10 years, respectively. This paper provides consensus-based imaging recommendations for evaluation of patients with primary hepatic malignancies at diagnosis and follow-up during and after therapy.

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 manuscript describes relevant imaging modalities and presents consensus-based recommendations for imaging at diagnosis and follow up.

There is growing interest in the use of contrast-enhanced ultrasound (CEUS) in diagnostic and interventional radiology. CEUS applications in interventional radiology are performed with intravascular or intracavitary administration of microbubble-based US contrast agents to allow for real-time evaluation of their distribution within the vascular bed or in body cavities, respectively, providing additional information beyond gray-scale US alone. The most common interventional-radiology-related CEUS applications in children have been extrapolated from those in adults, and they include the use of CEUS to guide lesion biopsy and to confirm drain placement in pleural effusions and intra-abdominal fluid collections. Other applications are emerging in interventional radiology for use in adults and children, including CEUS to optimize sclerotherapy of vascular malformations, to guide arthrography, and for lymphatic interventions. In this review article we present a wide range of interventional-radiology-related CEUS applications, emphasizing the current and potential uses in children. We highlight the technical parameters of the CEUS examination and discuss the main imaging findings.

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.

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.

10003 Background: The addition of dinutuximab (DIN) in the post-consolidation setting led to improved event-free survival rates for patients with high-risk neuroblastoma. Chemoimmunotherapy including irinotecan, temozolomide, DIN and sargramostim (GM-CSF) in patients with recurrent or refractory neuroblastoma results in robust objective clinical responses. Evaluation of chemoimmunotherapy in the induction setting for patients with newly-diagnosed high-risk neuroblastoma (HR-NBL) warrants investigation. Methods: Children’s Oncology Group (COG) ANBL17P1 is a prospective, single arm, limited institution pilot study to assess the tolerability and feasibility of administering DIN (17.5mg/m2/dose, IV Days 2-5) and GM-CSF (250mcg/m2/dose, subcutaneous Days 6-count recovery) with COG Induction chemotherapy Cycles 3-5 for patients with newly-diagnosed high-risk neuroblastoma. The primary endpoint of tolerability included the number of toxic deaths and number of patients experiencing predefined unacceptable toxicities during Induction Cycles 3-5. Unacceptable toxicities included: hypotension requiring pressors > 24 hours, respiratory toxicity requiring ventilatory support > 24 hours, Grade 4 neuropathy that did not resolve prior to the next cycle, and failure to recover the ANC to > 750 mm3 by day 35. Feasibility was assessed as being able to receive > 75% of planned DIN doses administered during Induction Cycles 3-5. Revised International Neuroblastoma Response Criteria (INRC) were used to assess end of Induction (EOI) response. Results: Forty-two eligible and evaluable patients with newly-diagnosed high-risk neuroblastoma enrolled at 8 sites (22 [52.4%] males; median age 3.3 years at diagnosis) from January 14, 2019 to June 4, 2020. The most common DIN related Grade >3 toxicities observed during Induction Cycles 3-5 included fever (31.0%) and pain (9.5%). None of the patients experienced a toxic death or unacceptable toxicity during Induction Cycles 3-5. Thus, the regimen was deemed tolerable. Patients received 97.4% - 101.8% of the total DIN dose expected to be administered during Induction Cycles 3-5. Therefore, the regimen was deemed feasible. Thirty-eight of 42 patients completed the EOI evaluations, including 11 with complete response, 22 with partial response, 0 with minor response, 3 with stable disease and 2 with progressive disease. The overall EOI objective response rate (CR+PR+MR) was 86.8%. Conclusions: The administration of DIN and GM-CSF to COG Induction Cycles 3-5 for patients with newly-diagnosed high-risk neuroblastoma was tolerable and feasible. The objective response rate at EOI appears encouraging. This therapeutic regimen will be studied in a randomized phase 3 trial to further evaluate the efficacy of Induction phase chemoimmunotherapy for high-risk neuroblastoma. Clinical trial information: NCT03786783.

PURPOSE Many novel therapies for relapsed and refractory neuroblastoma require tumor tissue for genomic sequencing. We analyze our experience with image-guided biopsy in these patients, focusing on safety, yield, adequacy for next-generation sequencing (NGS), and correlation of tumor cell percent (TC%) with quantitative uptake on 123I-meta-iodobenzylguanidine (MIBG) single-photon emission computed tomography with computed tomography (SPECT/CT). MATERIALS AND METHODS An 11-year retrospective review of image-guided biopsy on 66 patients (30 female), with a median age of 8.7 years (range, 0.9-49 years), who underwent 95 biopsies (55 bone and 40 soft tissue) of relapsed or refractory neuroblastoma lesions was performed. RESULTS There were seven minor complications (7%) and one major complication (1%). Neuroblastoma was detected in 88% of MIBG- or fluorodeoxyglucose-avid foci. The overall NGS adequacy was 69% (64% in bone and 74% in soft tissue, P = .37). NGS adequacy within neuroblastoma-positive biopsies was 88% (82% bone and 96% soft tissue, P = .11). NGS-adequate biopsies had a greater mean TC% than inadequates (51% v 18%, P = .03). NGS-adequate biopsies had a higher mean number of needle passes (7.5 v 3.4, P = .0002). The mean tissue volume from NGS-adequate soft-tissue lesions was 0.16 cm3 ± 0.12. Lesion:liver and lesion:psoas MIBG uptake ratios correlated with TC% (r = 0.74, r = 0.72, and n = 14). Mean TC% in NGS-adequate samples was 51%, corresponding to a lesion:liver ratio of 2.9 and a lesion:psoas ratio of 9.0. Thirty percent of biopsies showed an actionable ALK mutation or other therapeutically relevant variant. CONCLUSION Image-guided biopsy for relapsed or refractory neuroblastoma was safe and likely to provide NGS data to guide therapy decisions. A lesion:liver MIBG uptake ratio of ≥ 3 or a lesion:psoas ratio of > 9 was associated with a TC% sufficient to deliver NGS results.

To evaluate technical success and the incidences of, and risk factors for, mechanical and infectious complications of venous port placement in infants.This was a retrospective single-institution cohort study of port placement in infants (age1 y) from January 2006 through June 2016 (mean age, 7.5 mo ± 3.3; mean weight, 8.1 kg ± 1.9). Age, weight, sex, side of placement, tip position, and indication for placement (chemotherapy vs other) were recorded. Total catheter-days (CDs), mechanical complications, and central catheter-associated bloodstream infections (CCABSIs) were identified.During the study years, 64 ports were placed in 64 infants, with a technical success rate of 100%. The mean catheter life was 321 days (total range, 4-1,917 d; interquartile range [IQR], 107-421 d). There were 13 CCABSI events (0.63 per 1,000 CDs); of these, 8 (12.5% among 64 patients) required port removal for infection. There was an increase in CCABSIs in patients with left-sided port placement (relative risk [RR], 3.22; 95% confidence interval [CI], 1.02-10.14; P = .05). There were 8 mechanical complications of the port reservoir or catheter (0.39 per 1,000 CDs). Of these, 2 (3.1%) required removal. Patients in the lowest weight quartile were at an increased risk of mechanical complications (RR, 4.37; 95% CI, 1.09-17.48; P = .04).Venous ports can be placed with a high rate of technical success in infants. Left-sided ports and low weight are associated with increased infectious and mechanical complications, respectively.

Reduced-dose C-arm computed tomography (CT) uses flat-panel detectors to acquire real-time 3-D images in the interventional radiology suite to assist with anatomical localization and procedure planning. To describe dose-reduction techniques for C-arm CT at a pediatric institution and to provide guidance for implementation. We conducted a 5-year retrospective study on procedures using an institution-specific reduced-dose protocol: 5 or 8 s Dyna Rotation, 248/396 projection images/acquisition and 0.1–0.17 μGy/projection dose at the detector with 0.3/0.6/0.9-mm copper (Cu) filtration. We categorized cases by procedure type and average patient age and calculated C-arm CT and total dose area product (DAP). Two hundred twenty-two C-arm CT-guided procedures were performed with a dose-reduction protocol. The most common procedures were temporomandibular and sacroiliac joint injections (48.6%) and sclerotherapy (34.2%). C-arm CT was utilized in cases of difficult percutaneous access in less common applications such as cecostomy and gastrostomy placement, foreign body retrieval and thoracentesis. C-arm CT accounted for between 9.9% and 80.7% of the total procedural DAP. Dose-reducing techniques can preserve image quality for intervention while reducing radiation exposure to the child. This technology has multiple applications within pediatric interventional radiology and can be considered as an adjunctive imaging tool in a variety of procedures, particularly when percutaneous access is challenging despite routine fluoroscopic or ultrasound guidance.

Purpose To study the factors that might impact infarction of individual uterine leiomyomas and total tumor burden after uterine artery embolization (UAE). Materials and Methods This retrospective study included 91 patients (mean age, 44 y [range, 34–54 y]) who underwent UAE with tris-acryl gelatin microspheres (TAGMs) or nonspherical polyvinyl alcohol (PVA) particles. Twenty-one patients were treated with PVA (23%) and 70 were treated with TAGMs (77%). A total of 356 uterine leiomyomas were assessed, with a median uterine volume of 533 cm 3 (range, 321–848 cm 3 ). A reader masked to demographic and technical details reviewed contrast-enhanced magnetic resonance images before and 3 months after UAE to estimate the extent of tumor infarction. Results There was no significant difference in global or individual tumor infarction rate between embolizations with TAGMs and PVA particles ( P = .73 and P = .3, respectively). Global infarction was not affected by age ( P = .53), race ( P = .12), number of leiomyomas ( P = .72), or uterine volume ( P = .74). Leiomyoma size did not influence individual tumor infarction ( P = .41). Leiomyoma location was the sole factor that influenced individual tumor infarction rates, with pedunculated serosal tumors significantly less likely to show complete infarction than transmural tumors (odds ratio, 0.24; P = .01). Conclusions Nonspherical PVA particles and TAGMs produce similar rates of uterine leiomyoma infarction. Complete infarction of individual tumors is less likely in serosal and pedunculated serosal tumors.

Lymphatic malformations are slow-flow vascular anomalies composed of dilated lymphatic channels and cysts of varying sizes. Percutaneous treatments, particularly sclerotherapy, play an important role in the treatment of these lesions, often obviating the need for surgical intervention. Owing to the complex nature of these lesions, a multidisciplinary approach should be used to guide diagnosis and management. This submission focuses on the workup and treatment of pediatric lymphatic malformations at our institution, with a focus on sclerotherapy. Therapeutic outcomes and the management of postprocedural complications are also discussed.

INTRODUCTION Totally implantable venous access devices (TIVADs) are the preferred devices for patients with advanced lung disease who require long-term venous access. The primary purpose of this study was to describe the natural history of TIVADs left in place at the time of transplant. METHODS This multicenter retrospective cohort study evaluated pediatric and adult lung transplant recipients from 5/5/2005 to 12/31/17 with pretransplant TIVAD. Incident rates (IR) for infectious and mechanical complications were calculated. Poisson regression models were used to identify TIVAD characteristics associated with complications. RESULTS Of 1253 transplant recipients, 82 (6.5%) had pretransplant TIVAD. Five (6.1%) TIVADs were removed at transplantation. Fifty-five (67.1%) TIVADs were eventually removed, most commonly because they were no longer required (50.9%) or because of infection (25.5%). Overall incident rates (IR) of infectious or mechanical complications were 0.33 and 0.14, respectively. The IR of infection was highest within one year of transplant, particularly during the index hospitalization (IR = 1.67). Youngest tertile (

Venous malformations are the most common type of congenital vascular malformation. The diagnosis and management of venous malformations may be challenging, as venous malformations may be located anywhere in the body and range from small and superficial to large and extensive lesions. There are many treatment options for venous malformations including systemic targeted drugs, open surgery, sclerotherapy, cryoablation, and laser photocoagulation. This article reviews the natural history, clinical evaluation, imaging diagnosis, and treatment modalities of venous malformations.

MR enterography is traditionally used to evaluate inflammatory bowel disease (IBD) both at initial presentation and at follow-up. MR enterography can also be used to evaluate non-IBD conditions such as polyps or other masses of the gastrointestinal tract. In this article, we emphasize how to recognize bowel conditions beyond IBD on conventional abdominal MRI without a specific enterographic technique. In this overview we discuss common and uncommon pediatric bowel conditions beyond IBD seen on MRI including infectious and inflammatory conditions, congenital diseases and tumor and tumor-like conditions. Radiologists should become familiar with the salient imaging features of these bowel conditions to help guide management.

Image-guided percutaneous core needle biopsy (PCNB) of soft-tissue lesions is a minimally invasive technique that can provide a definitive diagnosis for treatment. Anatomical and age considerations in children require judicious use of safe percutaneous biopsy techniques. There are, however, limited data on the determinants of diagnostic yield in soft-tissue lesions in this population. To assess lesion-related and technical factors that affect diagnostic yield and safety in PCNB of pediatric soft-tissue lesions. This was a retrospective cohort study of 205 PCNB performed from January 2000 to July 2014. Diagnostic yield and its associations with technical and lesion-related factors were evaluated using bivariate analysis. The mean patient age was 11.1 ± 6.9 years (interquartile range [IQR]: 4.3-16 years). Ultrasound guidance alone was used in 91% of cases. The mean number of passes was 7.5 ± 3.2 (IQR: 5–9 passes) per case. The overall diagnostic yield was 75% and the overall accuracy was 88%. Performing fewer than four passes was associated with a nondiagnostic biopsy (P = 0.001). There were no lesion or other technical factors that predicted a diagnostic biopsy. There were two complications (1%) over 14 years. Image-guided PCNB is a safe and effective means for the diagnosis of pediatric soft-tissue abnormalities. Aside from performing at least four passes, our data show no technical or lesion-related factors that increase diagnostic yield in pediatric PCNB of soft-tissue lesions.