Percutaneous biopsy for the diagnosis, risk stratification, and molecular profiling of neuroblastoma: A single-center retrospective study.

PURPOSE: To determine whether percutaneous core needle biopsy (PCNB) is adequate for the diagnosis and full molecular characterization of newly diagnosed neuroblastoma. MATERIALS AND METHODS: Patients with newly diagnosed neuroblastoma who underwent PCNB in interventional radiology at a single center over a 5-year period were included. Pre-procedure imaging and procedure details were reviewed. Rates of diagnostic success and sufficiency for International Neuroblastoma Pathology Classification (INPC), risk stratification, and evaluation of genomic markers utilized in the Children's Oncology Group risk stratification, and status of the anaplastic lymphoma kinase (ALK) gene were assessed. RESULTS: Thirty-five patients (13 females, median age 2.4 years [interquartile range, IQR: 0.9-4.4] and median weight 12.4 kg [IQR: 9.6-18]) were included. Most had International Neuroblastoma Risk Group Stage M disease (n = 22, 63%). Median longest axis of tumor target was 8.8 cm [IQR: 6.1-12]. A 16-gauge biopsy instrument was most often used (n = 20, 57%), with a median of 20 cores [IQR: 13-23] obtained. Twenty-five specimens were assessed for adequacy, and 14 procedures utilized contrast-enhanced ultrasound guidance. There were two post-procedure bleeds (5.7%). Thirty-four of 35 procedures (97%) were sufficient for histopathologic diagnosis and risk stratification, 94% (n = 32) were sufficient for INPC, and 85% (n = 29) were sufficient for complete molecular characterization, including ALK testing. Biologic information was otherwise obtained from bone marrow (4/34, 12%) or surgery (1/34, 2.9%). The number of cores did not differ between patients with sufficient versus insufficient biopsies. CONCLUSION: In this study, obtaining multiple cores with PCNB resulted in a high rate of diagnosis and successful molecular profiling for neuroblastoma.

Embolization for pediatric trauma.

BACKGROUND: The management of pediatric trauma with trans-arterial embolization is uncommon, even in level 1 trauma centers; hence, there is a dearth of literature on this subject compared to the adult experience. OBJECTIVE: To describe a single-center, level 1 trauma center experience with arterial embolization for pediatric trauma. MATERIALS AND METHODS: A retrospective review was performed to identify demographics, transfusion requirements, pre-procedure imaging, procedural details, adverse events, and arterial embolization outcomes over a 19-year period. Twenty children (age 4.5 months to 17 years, median 13.5 years; weight 3.6 to 108 kg, median 53 kg) were included. Technical success was defined as angiographic resolution of the bleeding-related abnormality on post-embolization angiography or successful empiric embolization in the absence of an angiographic finding. Clinical success was defined as not requiring additional intervention after embolization. RESULTS: Seventy-five percent (n=15/20) of patients required red blood cell transfusions prior to embolization with a mean volume replacement 64 ml/kg (range 12-166 ml/kg) and the median time from injury to intervention was 3 days (range 0-16 days). Technical success was achieved in 100% (20/20) of children while clinical success was achieved in 80% (n=16/20). For the 4 children (20%) with continued bleeding following initial embolization, 2 underwent repeat embolization, 1 underwent surgery, and 1 underwent repeat embolization and surgery. Mortality prior to discharge was 15% (n=3). A post-embolization mild adverse event included one groin hematoma, while a severe adverse event included one common iliac artery pseudoaneurysm requiring open surgical ligation. CONCLUSIONS: In this single-center experience, arterial embolization for hemorrhage control in children after trauma is feasible but can be challenging and the clinical failure rate of 20% in this series reflects this complexity. Standardization of pre-embolization trauma assessment parameters and embolic techniques may improve outcomes.

Endovascular closure of a congenital extrahepatic portosystemic shunt for the treatment of hepatopulmonary syndrome in an infant.

Congenital portosystemic shunts may result in the development of hepatopulmonary syndrome, typically presenting with progressive hypoxemia in later childhood. We describe a case of a 5-month-old male with heterotaxy with polysplenia presenting with new onset hypoxemia. Subsequent evaluation identified an extrahepatic portosystemic shunt arising from the confluence of the main portal and superior mesenteric veins draining into the left renal vein. To treat his hypoxemia and prevent future complications of shunting, the patient underwent a successful single-stage endovascular closure.

Prevalence, management and efficacy of treatment in portal vein obstruction after paediatric liver transplantation: protocol of the retrospective international multicentre PORTAL registry.

INTRODUCTION: Portal vein obstruction (PVO) consists of anastomotic stenosis and thrombosis, which occurs due to a progression of the former. The aim of this large-scale international study is to assess the prevalence, current management practices and efficacy of treatment in patients with PVO. METHODS AND ANALYSIS: The Portal vein Obstruction Revascularisation Therapy After Liver transplantation registry will facilitate an international, retrospective, multicentre, observational study, with 25 centres around the world already actively involved. Paediatric patients (aged <18 years) with a diagnosed PVO between 1 January 2001 and 1 January 2021 after liver transplantation will be eligible for inclusion. The primary endpoints are the prevalence of PVO, primary and secondary patency after PVO intervention and current management practices. Secondary endpoints are patient and graft survival, severe complications of PVO and technical success of revascularisation techniques. ETHICS AND DISSEMINATION: Medical Ethics Review Board of the University Medical Center Groningen has approved the study (METc 2021/072). The results of this study will be disseminated via peer-reviewed publications and scientific presentations at national and international conferences. TRIAL REGISTRATION NUMBER: Netherlands Trial Register (NL9261).

Percutaneous Nephrostomy in Neonates and Young Infants.

PURPOSE: To describe a single-center experience of placing percutaneous nephrostomy (PCN) tubes in neonates and young infants aged ≤3 months. MATERIALS AND METHODS: This retrospective study evaluated PCN placement during a 19-year period. Medical records were reviewed for patient demographics, indications, procedure details, catheter-related adverse events, and outcomes. A total of 45 primary PCN insertions were attempted in 29 children (median age, 11 days [range, first day of life to 3 months]; median weight, 3.5 kg [range, 1.4-7.0 kg]). Salvage procedures resulted in 13 secondary catheters in 6 children. The most common indication was ureteropelvic junction obstruction (40.0%), and the most common urinary tract dilation classification was P3 (88.9%). RESULTS: Technical success for primary placements was 95.6%; both technical failures were due to loss of access in the same patient. Of primary placements, 76.7% were electively removed, 6.9% were dislodged but not replaced, and the remaining 16.3% required salvage procedures. Mechanical adverse events occurred in 20.9% of primary and 53.8% of secondary catheters, including partial retraction, complete dislodgement, and occlusion. Urinary tract infections (UTIs) occurred in 18.6% of primary and 15.4% of secondary catheters. Urosepsis occurred in 2.3% of primary and 7.7% of secondary catheters. Median primary catheter dwell time was 41 days (range, 1-182 days) and median secondary catheter dwell time was 31 days (range, 10-107 days). CONCLUSION: PCN placement in neonates and young infants has a high technical success rate, although not without particular procedural and management challenges of catheter malfunction and UTI.

Novel method to biopsy aneurysmal bone cysts in children.

PURPOSE: The thin and friable septations composing aneurysmal bone cysts (ABC) may be challenging to target for percutaneous biopsy. The purpose of this study was to describe and evaluate a novel method of ABC biopsy using endomyocardial biopsy forceps as an attempt to capture larger fragments of tissue for diagnosis. METHODS AND MATERIALS: This was a retrospective study performed over a 17-year period. Patients <18 years old who underwent percutaneous biopsy for a presumed ABC, based on pre-procedure imaging, were included. Medical records were reviewed to identify age, sex, lesion location, biopsy procedure details, complications, and pathology results. A diagnostic biopsy was defined as conclusive histologic confirmation. Inconclusive findings or findings suggestive of but not diagnostic of an ABC were treated as non-diagnostic, even if imaging and clinical findings were characteristic. Biopsy device selection and quantity of tissue obtained was at the discretion of the pediatric interventional radiologist. Fisher's exact test was used to compare the diagnostic yield of standard biopsies to those employing biopsy forceps. RESULTS: Twenty-three biopsies were performed in 18 patients (11 female) with a median age of 14.7 years (IQR 10.6-15.6). Lesions were located in the extremities (7, 30.4%), chest (6, 26.1%), pelvis (5, 21.7%), spine (4, 17.4%), and mandible (1, 4.3%). Specimens were obtained using a 13- or 15-gauge bone coring needle (11, 47.8%); 14-, 16-, or 18-gauge soft tissue needle (6, 26.1%); or a combination of bone and soft tissue (4, 17.4%) needles. Endomyocardial biopsy forceps were utilized in 7 cases (30.4%), 2 in which it was the only device used. Overall, a conclusive pathologic diagnosis was made in 13/23 (56.5%) biopsies. Of the diagnostic biopsies, 1 was a unicameral bone cyst and all others were ABCs. No malignancy was identified. Compared to the standard approach, the use of forceps was more likely to result in a diagnostic biopsy (40.0% vs 100.0%, p = 0.008). There were no complications. CONCLUSIONS: Endomyocardial biopsy forceps offer an additional, novel technique to biopsy presumed ABCs and may improve diagnostic yield.

Pathogenic variants in PIK3CA are associated with clinical phenotypes of kaposiform lymphangiomatosis, generalized lymphatic anomaly, and central conducting lymphatic anomaly.

Complex lymphatic anomalies are debilitating conditions characterized by aberrant development of the lymphatic vasculature (lymphangiogenesis). Diagnosis is typically made by history, examination, radiology, and histologic findings. However, there is significant overlap between conditions, making accurate diagnosis difficult. Recently, genetic analysis has been offered as an additional diagnostic modality. Here, we describe four cases of complex lymphatic anomalies, all with PIK3CA variants but with varying clinical phenotypes. Identification of PIK3CA resulted in transition to a targeted inhibitor, alpelisib. These cases highlight the genetic overlap between phenotypically diverse lymphatic anomalies.

In utero torsion of an accessory hepatic lobe mimicking a congenital tumor.

Accessory hepatic lobes are rare anatomic variants connected to the liver by a fibrous stalk or parenchymal attachments. They are usually detected incidentally, but torsion is a rare complication. Here, we report torsion of an accessory hepatic lobe occurring in utero with a focus on the MRI findings. The lesion mimicked a congenital tumor, and we provide potential clues that may have narrowed the differential diagnosis prior to surgical exploration.

Length of Bowel Rest Does Not Predict Gastrojejunostomy Tube-Associated Intussusception Recurrence.

OBJECTIVES: One potential treatment for gastrojejunostomy (GJ) tube-related intussusception is bowel rest, whereby the GJ tube is replaced with a gastrostomy tube. The aim of this study was to determine whether bowel rest length was associated with decreased risk of re-intussusception. METHODS: Pediatric patients with GJ tube-related intussusceptions were identified during the study period of January 1, 2010 and August 1, 2021. Records were reviewed for demographics, symptoms, need for central access to initiate parenteral nutrition, and length of stay. Comparison was made between patients with intussusception recurrence within 30 days and those without. Those undergoing earlier replacement, defined as the first quartile of rest time, or 72 hours, were then compared to longer periods of bowel rest. RESULTS: Forty-six intussusceptions were included, with a median age of 2.8 years (interquartile range, IQR: 1.4-4.1) and weight of 12 kg (IQR: 8.9-15.4). All patients diagnosed as outpatient (54.3%) required hospital admission and 9 of 46 (19.6%) required central access. There were 7 recurrences (15.2%). There was no difference in recurrence based on time of bowel rest (5 days in those without vs 6 days in those with, P = 0.30) nor a difference in recurrence with <72 hours of bowel rest compared to >72 hours (1/15, 6.7% vs 6/31, 19.3%). Patients undergoing earlier exchange had a shorter median length of hospital stay (3 vs 8.5 days, P = 0.003). CONCLUSION: Length of bowel rest was not associated with recurrent GJ tube-related intussusceptions. If bowel rest is utilized, <72 hours may be sufficient to decrease length of hospital stay.

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

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.

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

Pediatric soft tissue tumors of the extremity include rhabdomyosarcoma and nonrhabdomyosarcoma neoplasms. This manuscript provides consensus-based imaging recommendations for imaging evaluation at diagnosis, during treatment, and following completion of therapy for patients with a soft tissue tumor of the extremity.

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.

Adjunctive techniques for percutaneous enteral access in children: a pictorial review.

Primary percutaneous gastrostomy and gastrojejunostomy tube placements are fundamental procedures performed in pediatric interventional radiology, with both antegrade and retrograde techniques described. In pediatric patients, however, challenges may arise due to smaller patient size and anatomical variations. Several adjunctive techniques may facilitate safe percutaneous access in the setting of a limited percutaneous gastric access window. These include the intra-procedural use of cone beam computed tomography (CT), percutaneous needle decompression in the setting of distended air-filled bowel interposed between the stomach and abdominal wall, post-pyloric balloon occlusion to facilitate gastric distension, ultrasound-guided gastric puncture, and intra-gastric contrast-enhanced ultrasound (ceUS) to define the relationship of the gastric wall and the anterior abdominal wall. Adjunctive techniques may increase successful primary percutaneous gastroenteric tube placement and may improve operator confidence in safe placement.

Multifocal Kaposiform Hemangioendothelioma in a Newborn With Confirmatory Histopathology.

Kaposiform hemangioendothelioma is classified as a locally aggressive vascular tumor of childhood resulting from abnormal angiogenesis and lymphangiogenesis. Most commonly, KHE presents as a single tissue mass, ranging from an erythematous papule to a violaceous indurated tumor. Definitive diagnosis requires tissue sampling with the demonstration of ill-defined nodules and fascicles of spindle-shaped D2-40 positive endothelial cells, forming slit-like vascular channels. This newborn presented with multifocal cutaneous Kaposiform hemangioendothelioma associated with Kasabach-Merritt phenomenon confirmed on histopathology with immunostaining.

Guideline for Children With Cancer Receiving General Anesthesia for Procedures and Imaging.

Children with cancer and those undergoing hematopoietic stem cell transplantation frequently require anesthesia for imaging as well as diagnostic and therapeutic procedures from diagnosis through follow-up. Due to their underlying disease and side effects of chemotherapy and radiation, they are at risk for complications during this time, yet no published guideline exists for preanesthesia preparation. A comprehensive literature review served as the basis for discussions among our multidisciplinary panel of oncologists, anesthesiologists, nurse practitioners, clinical pharmacists, pediatric psychologists, surgeons and child life specialists at the Children's Hospital of Philadelphia. Due to limited literature available, this panel created an expert consensus guideline addressing anesthesia preparation for this population.

Impact of routine chest radiographs after removal of pigtail chest tubes placed by pediatric interventional radiology.

BACKGROUND: Chest radiographs are commonly obtained after chest tube removal to assess for complications. The benefit of this practice in children is uncertain. OBJECTIVE: To determine the clinical impact of a routine chest radiograph following removal of chest tubes placed by pediatric interventional radiology. MATERIALS AND METHODS: This single-center retrospective study evaluated 200 chest tube removals in 176 patients (median age: 4 years, interquartile range [IQR]: 1.2-12; median weight: 17.2 kg, IQR: 10.67-37.6), who had a chest tube placed and removed by pediatric interventional radiology over a 16-year period. A chest radiograph obtained on the day of removal was compared to the preceding study. For patients with imaging changes, medical records were reviewed to determine whether clinical actions occurred as a result. All records were reviewed for 7 days after tube removal or hospital discharge, whichever occurred first. RESULTS: The most common indication for chest tube insertion was simple effusion (53%, 106/200) and the most common tube size was 10.2 French (38.7%, 81/209). The median tube dwell time was 8 days (IQR: 5-17). There was a median of 14 h (IQR: 7-33.5) between imaging before and after tube removal. Imaging changes occurred in 10% (n = 20/200) of chest tube removals. Three of 200 (1.5%) of these were symptomatic after removal and only 0.5% (1/200) required chest tube reinsertion. For the remaining removals resulting in chest radiograph changes, patients were asymptomatic and required no change in clinical management. CONCLUSION: For chest tubes placed by pediatric interventional radiology, these findings do not support the practice of a routine chest radiograph after removal in asymptomatic children.

Image-Guided Biopsy for Relapsed Neuroblastoma: Focus on Safety, Adequacy for Genetic Sequencing, and Correlation of Tumor Cell Percent With Quantitative Lesion MIBG Uptake.

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.

Management of Venous Malformations.

Venous malformations (VMs) are slow-flow, congenital vascular anomalies that are a result of vascular dysgenesis. Clinical presentation and morbidity depend on size, location, and association with other syndromes. VMs are the most common symptomatic vascular anomaly referred for intervention, usually due to thrombosis, swelling, mass effect, functional compromise, or cosmetic concerns. Treatment for larger lesions can be challenging and a multidisciplinary approach involving medical, interventional, and surgical input is critical for comprehensive care. This article will assist the interventional radiologist in patient assessment and will discuss current techniques for treatment, means to minimize adverse events, and expected outcomes.

Contrast-enhanced ultrasound in pediatric interventional radiology.

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.