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Background: Historically, surgical biopsy (SB) for diagnosis of pediatric solid tumors was considered necessary to provide adequate tissue for histologic and molecular analysis. Less invasive biopsy techniques such as image-guided core needle biopsy (CNB), have shown comparable accuracy with decreased morbidity in some adult studies. However, data regarding the safety and efficacy of CNB in pediatric tumors is limited. This study's aim was to assess the overall rate of successful diagnosis and safety of CNB compared to SB in children with malignancies., Methods: A PRISMA compliant systematic review was performed in MEDLINE via PubMed, Embase and CINAHL Plus database searches from 2010 to 2023. Studies were included with relevance to the following clinical question: For children with concern for malignancy requiring biopsy for diagnosis, how does CNB compare to open or laparoscopic/thoracoscopic SB in terms of safety and diagnostic efficacy? Data for patients ≤21 years requiring biopsy for diagnosis of liver tumors, neuroblastoma (NB), soft tissue sarcoma (STS), and lymphoma were included., Results: Twenty-seven studies including 2477 patients met inclusion criteria, with 2065 undergoing CNB and 412 SB. Of the 2477 patients, 820 patients had NB, 307 liver tumors, 96 STS, 151 lymphoma, and 1103 patients were from studies that included multiple diagnoses. The average complication rate for CNB was 2.9% compared to 21.4% for SB (p < 0.001). Bleeding was the most common complication in both groups, but significantly higher after SB (22.1% vs 2.3%) (p < 0.001). CNB was diagnostic in 90.8% of patients compared to 98.8% who underwent SB (p < 0.001)., Conclusions: Rates of successful diagnosis were greater than 90% for both CNB and SB, though significantly higher for SB. Conversely, complication rates were close to ten times higher after SB compared to CNB. Given its relatively lower risk profile, CNB can be a safe and useful diagnostic tool for children with solid malignancies. Research focused on enhancing CNB's diagnostic accuracy while maintaining low morbidity should be further explored., Level of Evidence: Treatment study, Level III., Competing Interests: Declaration of competing interest The authors have nothing to declare., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Children with end-stage solid tumors would benefit from established nonsurgical options for palliative site-directed local control with the intent of improving quality of life (QOL) and extending survival. We investigated the practice and tolerability of palliative ablation strategies in patients with pediatric solid tumors., Methods: Children and young adults with solid tumors ineligible for surgical site-directed control were treated with palliative ablative strategies between 2010 and 2020 at five institutions participating in the Pediatric Surgical Oncology Research Collaborative. Demographic, histologic, and clinical data were retrospectively evaluated, and assessment of patient reasons for choosing ablative strategies was qualitatively assessed., Results: A total of 69 patients underwent 89 ablative procedures for 132 lesions. More than 10 histologies were represented, the most common of which were Ewing sarcoma (n = 15 patients), osteosarcoma (n = 13 patients), and rhabdomyosarcoma (n = 9 patients). Ablation techniques included cryoablation (n = 9 procedures/16 lesions), radiofrequency ablation (RFA) (n = 4 procedures/5 lesions), microwave ablation (MWA) (n = 5 procedures/12 lesions), and stereotactic body radiotherapy (SBRT) (n = 68 procedures/99 lesions). Grade 3 or higher adverse events (AEs) included pain requiring IV medication in cryoablation (three out of 12 procedures), RFA (one out of four procedures), and MWA (two out of five procedures), bleeding in MWA (two out of five procedures), and skin burns requiring surgical intervention in SBRT (one out of 68 procedures). Patients were generally hospitalized after percutaneous ablation (19 out of 21 cryoablation, RFA, and MWA procedures) but the hospital stay was short (mean/SD 1 ± 4 days). Only four patients who were already hospitalized experienced hospitalization extension due to undergoing an ablative procedure. The primary reason for pursuing ablative measures could be broadly categorized into four groups: treatment of unresectable disease either due to surgical inaccessibility or metastasis (n = 31), symptom relief, either pain or mass effect (n = 25), specific patient QOL concerns unrelated to pain or life prolongation (n = 5), and physiologic instability for otherwise resectable disease (n = 4). All patients undergoing treatment for symptom relief received SBRT., Conclusion: In this cohort of patients undergoing palliative treatment of incurable or unresectable pediatric solid tumors, ablation strategies were well tolerated. Further research is needed to determine patient reported outcomes after ablation strategies are pursued and patient and family satisfaction with the choice for ablative strategies. An understanding of institutional availability and ability to employ these strategies would also be informative., (© 2024 Wiley Periodicals LLC.)

Background: Neuroblastoma is the most common extracranial solid tumor in children, with about half of cases classified as high risk. Treatment varies by risk level, with high-risk patients undergoing aggressive multimodal therapy. While long-term survival has improved, survivors face significant risks of late treatment effects, including adrenal insufficiency. This study investigates the incidence of adrenal insufficiency among neuroblastoma patients of varying disease risk., Procedure: This retrospective cohort study at a single tertiary children's hospital reviewed records from 1998 to 2021, identifying 370 neuroblastoma patients, with 137 having complete risk stratification. The primary outcome was the incidence of adrenal insufficiency, diagnosed via clinical evaluation, and compared by risk group., Results: Among 137 patients with neuroblastoma, nine (12.0%) high-risk and three (4.9%) non-high-risk patients were diagnosed with adrenal insufficiency. The cumulative incidence of adrenal insufficiency at 3 years after neuroblastoma diagnosis was 9.9% in high-risk and 3.5% in non-high-risk patients. Among high-risk neuroblastoma patients with adrenal insufficiency, median time to adrenal insufficiency diagnosis was 10.2 months from neuroblastoma diagnosis, with cases more commonly occurring in patients with adrenal primary tumors (p = 0.0234). There were no other significant differences in demographic or clinical characteristics between high-risk patients with and without adrenal insufficiency., Conclusions: Adrenal insufficiency occurs more commonly in children with high-risk neuroblastoma than in non-high-risk cases, particularly those with adrenal primary tumors. These findings underscore the need for vigilant monitoring and screening for adrenal insufficiency during and after treatment. Future research should include larger, multi-institutional cohorts to better understand risk factors and optimize screening protocols., (© The Author(s). Pediatric Blood & Cancer published by Wiley Periodicals LLC.)

Background: Differentiated thyroid carcinoma (DTC) is the most common pediatric thyroid malignancy, with papillary thyroid carcinoma (PTC) representing 90% of the cases. In 2015, the American Thyroid Association (ATA) developed management guidelines for pediatric DTC., Procedure: Patients less than 21 years of age diagnosed with DTC between 2000 and 2015 at Texas Children's Hospital, Seattle Children's Hospital, Children's Healthcare of Atlanta, Children's Hospital Colorado, and Nationwide Children's Hospital were retrospectively analyzed to evaluate treatment practices before the implementation of the ATA guidelines., Results: We included 216 pediatric patients with DTC. A majority were female (76%) with a median age at diagnosis of 14.8 years. Neck swelling (56%) and palpable nodules (49%) were common presenting symptoms. Diagnostic evaluations typically included thyroid ultrasound, with common features being microcalcifications (47.7%) and hypoechogenicity (39.0%). Fine-needle aspiration cytology confirmed diagnosis in 50.5%. The majority of patients underwent total thyroidectomy (78.7%) and lymph node dissection (75.5%). ATA risk stratification was low, intermediate, and high risk in 111 (51.4%), 29 (13.4%), and 76 patients (35.2%), respectively. Upfront radioactive iodine (RAI) was administered in 83%. At 1-year follow-up, 46% were disease-free (66% low risk, 59% intermediate risk, and 13% high risk). The 5-year progression-free survival for low, intermediate, and high risk were 86%, 53%, and 43%, respectively. The disease-related overall survival was 100%., Conclusions: Prior to the introduction of the ATA guidelines, a majority of patients underwent total thyroidectomy with lymph node dissection followed by RAI. Selective use of RAI for low-risk disease as recommended in the current guidelines may be appropriate., (© 2025 Wiley Periodicals LLC.)

Introduction: Currently, few prospective guidelines exist for the surveillance of children with low-risk neuroblastic tumors (LRNBTs), including ganglioneuroma or ganglioneuroblastoma intermixed. This study aims to describe our institutional approach to LRNBT surveillance following surgical resection or nonoperative management. We hypothesize that length of surveillance can be reduced due to low recurrence risk., Methods: We conducted a retrospective review of patients diagnosed with LRNBT at our institution between January 1, 2010, and December 31, 2022. The majority of patients were followed in our multidisciplinary surgical oncology clinic. We summarized relevant patient data including follow-up imaging modalities and recurrences., Results: Thirty-eight patients met the inclusion criteria at a median age of diagnosis of 9 ys (interquartile range [IQR]: 5, 12.75). Thirty-seven patients underwent surgical resection, with tumors located in the posterior mediastinum (n = 18), retroperitoneum (n = 14), presacral space (n = 3), pelvis (n = 1), or combined thoracic or retroperitoneal location (n = 1). One patient underwent nonoperative management for a tumor located in the retroperitoneum. Histopathological results demonstrated 18 cases of ganglioneuroma and 20 cases of ganglioneuroblastoma intermixed. The median duration of postoperative follow-up was 27 mos (IQR: 14, 51) and the median clinical visits per year was 2.6 [IQR: 1.5, 3.4). Surveillance imaging was performed at a median frequency of 2.4 times per year (IQR: 1.6, 3.1). Chest radiographs were the most common modality used for thoracic locations (34% of all imaging) and ultrasound was used most commonly for abdominal location (24% of all imaging). During surveillance, one recurrent or residual ganglioneuroma was identified on magnetic resonance imaging at 6.7 mos from presacral resection. This patient underwent repeat surgical resection, and subsequent surveillance demonstrated no further recurrences., Conclusions: Recurrence was low in this cohort of LRNBT patients. Optimal surveillance strategies for this low-risk pathology would minimize radiation exposure and burden of health-care visits to patients. Further multi-institutional studies are warranted to determine the best imaging modality, as well as the frequency and duration of follow-up visits for LRNBT patients. Future studies should evaluate the acceptability of short-term surveillance for those who achieve complete resection., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Disaster events such as weather events and mass casualty events are increasing in frequency and severity. Caring for children during a surge requires a regional approach given limited pediatric inpatient capacity and expertise. During the 2024 American Academy of Pediatrics National Convention and Exhibition, the Section on Surgery and Council on Children and Disasters (COCD) partnered to present a joint symposium emphasizing importance of pediatric readiness and disaster preparedness and role of pediatric trauma surgeons in disaster preparedness and response in all communities., Competing Interests: Conflict of interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2025 Elsevier Inc. All rights reserved.)

Purpose: Thyroid nodules are uncommon in children and adolescents but carry an increased risk of malignancy when present. The Thyroid Imaging Reporting and Data System (TI-RADS) is an adult-validated ultrasound-based risk assessment providing a prediction of malignant potential for thyroid nodules, thereby guiding recommendations for fine needle aspiration biopsy (FNAB). Minimal data exist regarding the applicability of TI-RADS to predict malignancy in pediatric thyroid nodules. This study aims to analyze the performance of TI-RADS for children and adolescents with thyroid nodules, hypothesizing that applying TI-RADS criteria would improve accuracy and reduce the number of recommended FNAB compared to American Thyroid Association (ATA) size criteria alone., Methods: A multi-institutional retrospective analysis was conducted including patients ≤21 years with a thyroid nodule by sonographic thyroid imaging between 2015 and 2020. TI-RADS scores were assigned at each institution by a pediatric radiologist trained in thyroid imaging and TI-RADS criteria. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and overall accuracy of TI-RADS scoring were compared to existing ATA size-based recommendation for performing a FNAB. Accounting for relative size differences between adults and children, a novel PED TI-RADS category was developed and tested, recommending FNAB for thyroid nodules with a TI-RADS 3 and ≥ 1.5 cm, TI-RADS 4 and ≥ 1.0 cm, and TI-RADS 5 any feasible size., Results: 291 nodules from 260 patients (median age 14.9 years, 78.8% female) were assessed using TI-RADS. Applying adult TI-RADS criteria resulted in recommendation of FNAB for 35.1% of nodules, in contrast to 76.6% recommended by ATA guidelines (p < 0.0001) (Table). Utilizing the adult TI-RADS score ≥3 as an FNAB indicator resulted in 100% sensitivity and 28.5% specificity, with 0 cases of missed malignant nodules on pathology. When novel PED TI-RADS criteria were applied, 88 patients would have been spared an unnecessary FNAB with improved sensitivity and accuracy over ATA criteria., Conclusions: The application of adult and PED TI-RADS scoring to thyroid nodules in pediatric patients enhances the accuracy of malignancy prediction compared to current American Thyroid Association size criteria alone. The utilization of PED TI-RADS scoring eliminated unnecessary biopsies in many children while not missing a single thyroid malignancy., Level of Evidence: Level III., Competing Interests: Conflict of interset None., (Published by Elsevier Inc.)

Introduction: Laparoscopic cholecystectomy (Lap-C) is the standard of care for patients requiring cholecystectomy in the acute setting. Although robotic-assisted cholecystectomy (RA-C) performance has increased, utilization in the acute setting has not been widely reported. We describe the feasibility of RA-C for pediatric patients undergoing acute inpatient cholecystectomy. Methods: A single institutional retrospective review of patients receiving RA-C while admitted for acute cholecystitis and/or choledocholithiasis (June 2017-June 2022) was compared with a matched cohort who underwent traditional multiport Lap-C (June 2021-June 2022). Demographic, perioperative, and postoperative data were analyzed. Results: Fifty patients were included: 25 each in the RA-C and Lap-C groups. Fifty-four percent were female; 66% were non-Hispanic white. Median age (15.7 years [interquartile range, IQR 14.7, 17.3] versus 15.3 years [IQR 14.5, 16.9], P = .91) and preoperative weight (92.6 kg [IQR 60, 105.9] versus 72.3 kg [IQR 61.6, 85.6], P = .15) were similar between the RA-C and Lap-C groups, respectively. No differences were observed in median operating time (89 minutes [IQR 76, 103] versus 88 minutes [IQR 77, 137], P = .70), postoperative length of stay (22.5 hours [21.4, 24.9] versus 20.6 hours [18.0, 25.1], P = .06), or 30-day complications (12% versus 16%, P = .69). Although opioid utilization (.23 milliequivalents/kilogram [MME/kg] [IQR .03, .30] versus .03 MME/kg [0, .09], P = .02) was higher in the RA-C cohort overall, no differences were detected during an analysis of the most recent 2 years ( P = .96). Conclusion: RA-C in the acute setting can be performed safely in the pediatric population with comparable procedural times as well as perioperative and 30-day outcomes.

Background: Minimally invasive thoracic surgical techniques require effective lung isolation using one-lung ventilation (OLV). Verification of lung isolation may be confirmed by auscultation, visual confirmation using fiberoptic bronchoscopy (FOB), or more recently, point-of-care ultrasound (POCUS). The aim of this study was to prospectively compare lung ultrasound with clinical auscultation to confirm OLV before thoracic surgery in pediatric patients., Methods: This prospectively blinded feasibility study included 40 patients ranging in age from 0 to 20 years. After confirmation of lung separation by the primary anesthesia team using FOB, the sonographer and the auscultator, both blinded to the laterality of surgery and lung separation, entered the operating room. The sonographer evaluated for pleural lung sliding and the auscultator listened for breath sounds. Successful lung separation was definitively confirmed by direct visualization of lung collapse during the operation., Results: In confirming effective single-lung ventilation, lung ultrasound had a diagnostic accuracy of 95% (95% confidence interval [CI], 82.7%-98.5%). In contrast, auscultation could only reliably confirm lung isolation with 68% accuracy (95% CI, 51.5%-80.4%). The McNemar test showed a statistically significant difference between the use of lung ultrasound and auscultation ( P < .001). The median time to perform ultrasonography was 67 seconds (interquartile range [IQR], 46-142) and the median time to perform auscultation was 21 seconds (IQR, 10-32)., Conclusions: Based on the initial results of our feasibility trial, lung ultrasound proved to be a fast and reliable method to verify single-lung ventilation in pediatric patients presenting for thoracic surgery with a high degree of diagnostic accuracy., Competing Interests: Conflicts of Interest, Funding: Please see DISCLOSURES at the end of this article., (Copyright © 2024 International Anesthesia Research Society.)

Introduction: The focused use of a single-site port as an adjunct designed to decrease overall port site number and/or assist with specimen extraction in pediatric robotic surgery has not been fully elucidated. We aimed to describe the feasibility of using the single-site port as an adjunct during multi-port robotic-assisted minimally invasive surgery (RA-MIS). Methods: A single institution retrospective review of pediatric patients who underwent multiport RA-MIS with an adjunctive single-site (SS) port from August 2018 to October 2022 was performed. Demographic, perioperative, and postoperative variables were collected; descriptive analysis was performed. Results: A total of 13 patients were included; 46% were female, and 47% were Caucasian. Median age at surgery was 14.9 years of age (interquartile range [IQR] 10.6, 18); median weight was 61.1 kg (IQR 39.7, 73.6). Eleven patients (85%) underwent splenectomy; 2 patients (15%) underwent adrenalectomy. Four patients had a combined procedure (SS cholecystectomy with multi-port splenectomy [ n = 3], multi-port bilateral adrenalectomy [ n = 1]). The median total operative time was 197 minutes (IQR 131, 316); median console time was 59 minutes (IQR 40, 126). Two 8 mm robotic ports were utilized for all but 1 patient who required a third 8 mm port. The median length of stay was 2.1 days (IQR 2.0, 3.1). One readmission for fever occurred following a combined cholecystectomy/splenectomy. No hernias or wound infections were identified at the single-site port. Conclusion: Use of a SS port as an adjunct is a feasible option and should be considered for those with splenomegaly or need for combined procedures in different quadrants of the abdomen.

Introduction: Sacrococcygeal teratoma (SCT) is a rare congenital tumour. The risk of malignancy and recurrence is not well defined. Previous studies are small and report differing conclusions about the timing of surgery and the duration of follow-up. The authors studied the risk of malignant transformation and SCT recurrence after surgery to address these gaps., Methods: This was a global retrospective cohort study. Data of consecutive SCT patients was obtained from 145 institutes in 62 countries. Malignant transformation, defined as malignancy at initial resection, malignant recurrence or death due to malignancy, and its risk factors were analysed., Results: Of the 3612 included patients, 3407 entered analysis. The risk of malignant transformation of the initial tumour was 3.3, 5.1, 10.1, and 32.9% at age 3 months, 6 months, 1 year, and 2 years, respectively. After 6 years, the censored risk of malignancy (64%) did not further increase. Recurrent SCT was diagnosed in 349 (10.2%) children with 126 (36.1%) malignant recurrences. Risk factors for recurrence were Altman type II [odds ratio (OR): 1.6, 95% confidence interval (CI): 1.2-2.2], Altman type III (OR: 1.6, 95% CI: 1.2-2.3), initial immature histology (OR: 1.9, 95% CI: 1.4-2.6), and initial malignant histology (OR: 4.0, 95% CI: 2.9-5.4)., Conclusion: The risk of malignancy at initial resection in SCT increases with age reaching a plateau at 6 years of age. Recurrence after resection occurred in 10% of patients and 36% of these were malignant at that time. Altman type II or type III, and immature or malignant histology were associated with recurrence., Level of Evidence: Level III., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc.)

Purpose: The purpose of this study was to develop recommendations for the diagnostic evaluation and surgical management of cutaneous melanoma (CM) and atypical Spitz tumors (AST) and non-Spitz melanocytic tumors (melanocytomas) in pediatric (age 0-10 years) and adolescent (age 11-18 years) patients., Methods: A Children's Oncology Group-led panel with external, multidisciplinary CM specialists convened to develop recommendations on the basis of available data and expertise., Results: Thirty-three experts from multiple specialties (cutaneous/medical/surgical oncology, dermatology, and dermatopathology) established recommendations with supporting data from 87 peer-reviewed publications., Recommendations: (1) Excisional biopsies with 1-3 mm margins should be performed when feasible for clinically suspicious melanocytic neoplasms. (2) Definitive surgical treatment for CM, including wide local excision and sentinel lymph node biopsy (SLNB), should follow National Comprehensive Cancer Network Guidelines in the absence of data from pediatric-specific surgery trials and/or cohort studies. (3) Accurate classification of ASTs as benign or malignant is more likely with immunohistochemistry and next-generation sequencing. (4) It may not be possible to classify some ASTs as likely/definitively benign or malignant after clinicopathologic and/or molecular correlation, and these Spitz tumors of uncertain malignant potential should be excised with 5 mm margins. (5) ASTs favored to be benign should be excised with 1- to 3-mm margins if transected on biopsy. (6) Re-excision is not necessary if the AST does not extend to the biopsy margin(s) when complete/excisional biopsy was performed. (7) SLNB should not be performed for Spitz tumors unless a diagnosis of CM is favored on clinicopathologic evaluation. (8) Non-Spitz melanocytomas have a presumed increased risk for progression to CM and should be excised with 1- to 3-mm margins if transected on biopsy. (9) Re-excision of non-Spitz melanocytomas is not necessary if the lesion is completely excised on biopsy.

With an annual cumulative occurrence of approximately 15,000 in North America, all childhood cancers are rare. Very rare cancers as defined by both the European Cooperative Study Group for Rare Pediatric Cancers and the Children's Oncology Group fall into two principal categories: those so uncommon (fewer than 2 cases/million) that their study is challenging even through cooperative group efforts (e.g., pleuropulmonary blastoma and desmoplastic small round cell tumor) and those that are far more common in adults and therefore rarely studied in children (e.g., thyroid, melanoma, and gastrointestinal stromal tumor). Treatment strategies for these latter tumors are typically based on adult guidelines, although the pediatric variants of these tumors may harbor different genetic signatures and demonstrate different behavior. If melanoma and differentiated thyroid cancer are excluded, other rare cancer types account for only 2% of the cancers in children aged 0 to 14. This article highlights several of the most common rare tumor types., (© 2024 Wiley Periodicals LLC.)

As therapy for childhood malignancies becomes more sophisticated and survival has improved, long-term therapy-related sequelae have emerged. Loss of reproductive potential among childhood cancer survivors is one such concern that has become increasingly recognized among patients, families, and healthcare providers. The risk status for infertility based upon therapy received, state of current reproductive technology and outcomes, and an emphasis on adequate referral and counseling for fertility preservation options are reviewed. Contributing factors to infertility are discussed, and options for female and male preservation based upon age and pubertal status are summarized. This article highlights the current state of fertility opportunities for children and adolescents undergoing therapy for cancer. Providers caring for these young patients should be familiar with such options and should routinely initiate evaluations for eligibility of fertility preservation., (© 2024 Wiley Periodicals LLC.)

Introduction: Robotic-assisted minimally invasive surgery (RA-MIS) for tumor resection is an emerging technology in the pediatric population with significant promise but unproven safety and feasibility., Methods: A multi-center retrospective review of patients ≤18 years undergoing RA-MIS tumor resection from December 2015-March 2023 was performed. Patient demographics, perioperative variables, and complication rates were analyzed., Results: Thirty-nine procedures were performed on 38 patients (17 thoracic, 22 abdominal); 37% female and 68% non-Hispanic White. Median age at surgery was 8.3 years (IQR 5.7, 15.7); the youngest was 1.7 years-old. Thoracic operations included resections of neuroblastic tumors (n = 16) and a single paraganglioma. The most common abdominal operations included resections of neuroblastic tumors (n = 5), pheochromocytomas (n = 3), and angiomyolipomas (n = 3). Six patients underwent retroperitoneal lymph node dissection (RPLND) for paratesticular tumors. Median operating time for the cohort was 2:52 h (IQR 2:04, 4:31). Two thoracic cases required open conversion due to poor visualization and lack of working domain. All patients underwent complete tumor resection; one had tumor spillage from a positive margin (Wilms tumor). Median LOS was 1.5 days (IQR 1.1, 3.0). Postoperatively, one patient developed a chyle leak requiring interventional radiology drainage, but none required a return to the operating room., Conclusions: Robotic-assisted surgery is safe and feasible for tumor resection in carefully selected pediatric patients, achieving complete resection with minimal morbidity and short LOS. Resection should be performed by those with robotic expertise for optimal outcomes., Level of Evidence: IV., Type of Study: Original Clinical Research., Competing Interests: Conflicts of interest Dr. Marc Michalsky is a stockholder and educational honorarium with Intuitive Surgical. He also is on the Childhood Obesity Advisory Board for Lilly USA, LLC. No other authors have any disclosures., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Tunneled central venous catheters (CVCs) are the cornerstone of modern oncologic practice. Establishing best practices for catheter management in children with cancer is essential to optimize care, but few guidelines exist to guide placement and management., Objectives: To address four questions: 1) Does catheter composition influence the incidence of complications; 2) Is there a platelet count below which catheter placement poses an increased risk of complications; 3) Is there an absolute neutrophil count (ANC) below which catheter placement poses an increased risk of complications; and 4) Are there best practices for the management of a central line associated bloodstream infection (CLABSI)?, Methods: Data Sources: English language articles in Ovid Medline, PubMed, Embase, Web of Science, and Cochrane Databases., Study Selection: Independently performed by 2 reviewers, disagreements resolved by a third reviewer., Data Extraction: Performed by 4 reviewers on forms designed by consensus, quality assessed by GRADE methodology., Results: Data were extracted from 110 manuscripts. There was no significant difference in fracture rate, venous thrombosis, catheter occlusion or infection by catheter composition. Thrombocytopenia with minimum thresholds of 30,000-50,000 platelets/mcl was not associated with major hematoma. Limited evidence suggests a platelet count <30,000/mcL was associated with small increased risk of hematoma. While few studies found a significant increase in CLABSI in CVCs placed in neutropenic patients with ANC<500Kcells/dl, meta-analysis suggests a small increase in this population. Catheter removal remains recommended in complicated or persistent infections. Limited evidence supports antibiotic, ethanol, or hydrochloric lock therapy in definitive catheter salvage. No high-quality data were available to answer any of the proposed questions., Conclusions: Although over 15,000 tunneled catheters are placed annually in North America into children with cancer, there is a paucity of evidence to guide practice, suggesting multiple opportunities to improve care., Level of Evidence: III. This study was registered as PROSPERO 2019 CRD42019124077., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Very rare tumors are usually divided into two groups: One includes tumors that are rare among both children and adults; the other one encompasses tumors that frequently occur in adults but are rarely observed in children. In this review, we focus on adrenocortical tumors, neuroendocrine tumors of the appendix, pheochromocytoma and paraganglioma, pancreatoblastoma and solid pseudopapillary tumors of the pancreas, with special attention to the role of surgery as main curative intervention or as part of the multimodal treatment., (© 2024 Wiley Periodicals LLC.)

The management of pediatric tumors is complex, with surgery, chemotherapy, and radiotherapy being cornerstones in their treatment. Tumor removal is increasingly performed by a minimally invasive approach, which allows for quicker postoperative recovery and less postoperative pain. The goal of this report is to give an overview of minimally invasive surgical approaches for common pediatric tumors, with a focus on technical considerations and postoperative outcomes., (© 2024 Wiley Periodicals LLC.)

Pediatric and adolescent ovarian lesions are common and are frequently managed by both pediatric surgeons and pediatric and adolescent gynecologists. During the 2023 American Academy of Pediatric Section on Surgery meeting, an educational symposium was delivered focusing on various aspects of management of pediatric and adolescent benign and malignant masses, borderline lesions, and fertility options for children and adolescents undergoing cancer therapies. This article highlights the discussion during this symposium., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Purpose: Our objectives were to compare overall survival (OS) and pulmonary relapse between patients with metastatic Ewing sarcoma (EWS) at diagnosis who achieve rapid complete response (RCR) and those with residual pulmonary nodules after induction chemotherapy (non-RCR)., Patients and Methods: This retrospective cohort study included children under 20 years with metastatic EWS treated from 2007 to 2020 at 19 institutions in the Pediatric Surgical Oncology Research Collaborative. Chi-square tests were conducted for differences among groups. Kaplan-Meier curves were generated for OS and pulmonary relapse., Results: Among 148 patients with metastatic EWS at diagnosis, 61 (41.2%) achieved RCR. Five-year OS was 71.2% for patients who achieved RCR, and 50.2% for those without RCR (p = .04), and in multivariable regression among patients with isolated pulmonary metastases, RCR (hazards ratio [HR] 0.42; 95% confidence interval [CI]: 0.17-0.99) and whole lung irradiation (WLI) (HR 0.35; 95% CI: 0.16-0.77) were associated with improved survival. Pulmonary relapse occurred in 57 (37%) patients, including 18 (29%) in the RCR and 36 (41%) in the non-RCR groups (p = .14). Five-year pulmonary relapse rates did not significantly differ based on RCR (33.0%) versus non-RCR (47.0%, p = .13), or WLI (38.8%) versus no WLI (46.0%, p = .32)., Discussion: Patients with EWS who had isolated pulmonary metastases at diagnosis had improved OS if they achieved RCR and received WLI, despite having no significant differences in rates of pulmonary relapse., (© 2024 Wiley Periodicals LLC.)

As the management of acute pain for children undergoing surgical procedures as well as recognition of the short and long term risks of exposure to opioids has evolved, multimodal and multidisciplinary approaches using organized pathways has resulted in improved perioperative outcomes and patient satisfaction. In this 2023 symposium held at the American Academy of Pediatrics on Surgery meeting, a multidisciplinary discussion on current enhanced recovery after surgery pathways, alternate methods of effective pain control and education and advocacy efforts for opioid reduction were discussed, and highlights are included in this article., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Introduction: The purpose of this study is to examine the outcomes in children with anaplastic bilateral Wilms tumor (BWT) from study AREN0534 in order to define potential prognostic factors and areas to target in future clinical trials., Methods: Demographic and clinical data from AREN0534 study patients with anaplasia (focal anaplasia [FA], or diffuse anaplasia [DA]) were compared. Event-free survival (EFS) and overall survival (OS) were reported using Kaplan-Meier estimation with 95% confidence bands, and differences in outcomes between FA and DA compared using log-rank tests. The impact of margin status was analyzed., Results: Twenty-seven children who enrolled on AREN0534 had evidence of anaplasia (17 DA, 10 FA) in at least one kidney and were included in this analysis. Twenty-six (96%) had BWT. Nineteen percent had anaplastic histology in both kidneys (four of 17 DA, and one of 10 FA). Forty-six percent with BWT had bilateral nephron-sparing surgery (NSS); one child who went off protocol therapy, eventually required bilateral completion nephrectomies. Median follow-up for EFS and OS was 8.6 and 8.7 years from enrollment. Four- and 8-year EFS was 53% [95% confidence interval (CI): 34%-83%] for DA; 4-year EFS was 80% [95% CI: 59%-100%], and 8-year EFS 70% [95% CI: 47%-100%] for FA. Three out of 10 children with FA and eight out of 17 children with DA had events. EFS did not differ statistically by margin status (p = .79; HR = 0.88). Among the six children who died (five DA, one FA), all experienced prior relapse or progression within 18 months., Conclusion: Events in children with DA/FA in the setting of BWT occurred early. Caution should be taken about interpreting the impact of margin status outcomes in the context of contemporary multimodal therapy. Future targeted investigations in children with BWT and DA/FA are needed., (© 2024 Wiley Periodicals LLC.)

This manuscript highlights the impressive advocacy work that members of the American Academy of Pediatrics have achieved and serves to inspire pediatric health care providers of all specialties to pursue such efforts beyond the acute physical need of the child. This article represents one of the Symposia presented at the 2023 American Academy of Pediatrics Section on Surgery., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Pediatric patients with metastatic and/or recurrent solid tumors have poor survival outcomes despite standard-of-care systemic therapy. Stereotactic ablative radiation therapy (SABR) may improve tumor control. We report the outcomes with the use of SABR in our pediatric solid tumor population., Methods: This was a single-institutional study in patients < 30 years treated with SABR. The primary endpoint was local control (LC), while the secondary endpoints were progression-free survival (PFS), overall survival (OS), and toxicity. The survival analysis was performed using Kaplan-Meier estimates in R v4.2.3., Results: In total, 48 patients receiving 135 SABR courses were included. The median age was 15.6 years (interquartile range, IQR 14-23 y) and the median follow-up was 18.1 months (IQR: 7.7-29.1). The median SABR dose was 30 Gy (IQR 25-35 Gy). The most common primary histologies were Ewing sarcoma (25%), rhabdomyosarcoma (17%), osteosarcoma (13%), and central nervous system (CNS) gliomas (13%). Furthermore, 57% of patients had oligometastatic disease (≤5 lesions) at the time of SABR. The one-year LC, PFS, and OS rates were 94%, 22%, and 70%, respectively. No grade 4 or higher toxicities were observed, while the rates of any grade 1, 2, and 3 toxicities were 11.8%, 3.7%, and 4.4%, respectively. Patients with oligometastatic disease, lung, or brain metastases and those who underwent surgery for a metastatic site had a significantly longer PFS. LC at 1-year was significantly higher for patients with a sarcoma histology (95.7% vs. 86.5%, p = 0.01) and for those who received a biological equivalent dose (BED10) > 48 Gy (100% vs. 91.2%, p = 0.001)., Conclusions: SABR is well tolerated in pediatric patients with 1-year local failure and OS rates of <10% and 70%, respectively. Future studies evaluating SABR in combination with systemic therapy are needed to address progression outside of the irradiated field.

Competing Interests: The authors report no conflicts of interest.

Introduction: Robotic-assisted surgery (RAS) is an increasingly utilized tool in children. However, utilization of RAS among infants and small children has not been well established. The purpose of this study was to review and characterize RAS procedures for children ≤15 kg. Methods: We performed a single institution retrospective descriptive analysis including all patients ≤15 kg undergoing RAS between January 2013 and July 2021. Data collection included procedure type, age, weight, gender, and surgical complications. Cases were further categorized according to surgical specialty: pediatric urology (PU), pediatric surgery (PS), and multiple specialties (MS). t -Tests were used for statistical analyses. Results: Since 2013, a total of 976 RAS were identified: 492 (50.4%) were performed by PU, 466 (47.8%) by PS, and 18 (1.8%) by MS. One hundred eighteen (12.1%) were performed on children ≤15 kg, consisting of 110 (93.2%) PU cases, 6 (5.1%) PS cases, and 2 (1.7%) MS cases. Procedures were significantly more common in the PU subgroup, mean of 12 cases/year, compared to PS subgroup, mean of 0.63 cases/year, ( P  < .01). The mean weight of PU patients (10.5 kg) was significantly less than PS patients (13.9 kg) ( P  < .01). Mean age was also significantly lower among PU patients (18.6 months) compared to PS (34.2 months) ( P  < .01). Conclusion: RAS among patients ≤15 kg is safe and feasible across pediatric surgical subspecialties. RAS was performed significantly more frequently by pediatric urologists in younger and smaller patients compared to pediatric surgeons. Further refinement of robotic technology and instrumentation should enhance the applicability of these procedures in this young group.

Study Objective: To assess the diagnostic performance of MRI to predict ovarian malignancy alone and compared with other diagnostic studies., Methods: A retrospective analysis was conducted of patients aged 2-21 years who underwent ovarian mass resection between 2009 and 2021 at 11 pediatric hospitals. Sociodemographic information, clinical and imaging findings, tumor markers, and operative and pathology details were collected. Diagnostic performance for detecting malignancy was assessed by calculating sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) for MRI with other diagnostic modalities., Results: One thousand and fifty-three patients, with a median age of 14.6 years, underwent resection of an ovarian mass; 10% (110/1053) had malignant disease on pathology, and 13% (136/1053) underwent preoperative MRI. MRI sensitivity, specificity, PPV, and NPV were 60%, 94%, 60%, and 94%. Ultrasound sensitivity, specificity, PPV, and NPV were 31%, 99%, 73%, and 95%. Tumor marker sensitivity, specificity, PPV, and NPV were 90%, 46%, 22%, and 96%. MRI and ultrasound concordance was 88%, with sensitivity, specificity, PPV, and NPV of 33%, 99%, 75%, and 94%. MRI sensitivity in ultrasound-discordant cases was 100%. MRI and tumor marker concordance was 88% with sensitivity, specificity, PPV, and NPV of 100%, 86%, 64%, and 100%. MRI specificity in tumor marker-discordant cases was 100%., Conclusion: Diagnostic modalities used to assess ovarian neoplasms in pediatric patients typically agree. In cases of disagreement, MRI is more sensitive for malignancy than ultrasound and more specific than tumor markers. Selective use of MRI with preoperative ultrasound and tumor markers may be beneficial when the risk of malignancy is uncertain., Concise Abstract: This retrospective review of 1053 patients aged 2-21 years who underwent ovarian mass resection between 2009 and 2021 at 11 pediatric hospitals found that ultrasound, tumor markers, and MRI tend to agree on benign vs malignant, but in cases of disagreement, MRI is more sensitive for malignancy than ultrasound., (Copyright © 2023. Published by Elsevier Inc.)

Background/purpose: The process of evaluating pediatric thyroid nodules at our institution was inconsistent with a high rate of negative biopsies raising concern of appropriate patient selection for biopsy. Our aim was to institute a standardized risk stratification reporting system for thyroid nodules to increase utilization and agreement of TI-RADS reporting at our institution., Methods: Radiology report data were collected and analyzed as part of a quality improvement project. A standardized TI-RADS dictation template was created, ultrasound technicians were trained, a multi-disciplinary conference initiated, and education provided for radiologists and clinicians. Control charts were used to track utilization and agreement of scoring of TI-RADS reporting based upon review by a radiologist trained in TI-RADS scoring., Results: From January 2019 to January 2021, 218 patients with a thyroid nodule had a thyroid ultrasound performed at our institution. TI-RADS was utilized in 0 % (0 of 57) of children in the four months prior to project initiation. Following creation of the template, utilization increased to 65 % (39 of 60) over 5 months. Utilization further increased after the first training conference and was maintained above 90 % for 13 months. Ultrasound reports were in agreement in 46.7 % (28 of 60) of children initially. Agreement in reporting improved to 71.4 % (10 of 14) in the 3 months following the first training and to 78.4 % (58 of 74) over 12 months. Agreement in reporting was maintained at 80 % in the following 6 months., Conclusions: A quality improvement initiative can improve utilization and agreement of scoring using the TI-RADS system in pediatrics. This may ultimately reduce unnecessary biopsies and sedation in children., Level of Evidence: Level III., Type of Study: Quality Improvement., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Objective: To determine the impact of nodal basin ultrasound (US) surveillance versus completion lymph node dissection (CLND) in children and adolescents with sentinel lymph node (SLN) positive melanoma., Background: Treatment for children and adolescents with melanoma are extrapolated from adult trials. However, there is increasing evidence that important clinical and biological differences exist between pediatric and adult melanoma., Methods: Patients ≤18 years diagnosed with cutaneous melanoma between 2010 and 2020 from 14 pediatric hospitals were included. Data extracted included demographics, histopathology, nodal basin strategies, surveillance intervals, and survival information., Results: Of 252 patients, 90.1% (n=227) underwent SLN biopsy (SLNB), 50.9% (n=115) had at least 1 positive node. A total of 67 patients underwent CLND with 97.0% (n=65/67) performed after a positive SLNB. In contrast, 46 total patients underwent US observation of nodal basins with 78.3% (n=36/46) of these occurring after positive SLNB. Younger patients were more likely to undergo US surveillance (median age 8.5 y) than CLND (median age 11.3 y; P =0.0103). Overall, 8.9% (n=21/235) experienced disease recurrence: 6 primary, 6 nodal, and 9 distant. There was no difference in recurrence (11.1% vs 18.8%; P =0.28) or death from disease (2.2% vs 9.7%; P =0.36) for those who underwent US versus CLND, respectively., Conclusions: Children and adolescents with cutaneous melanoma frequently have nodal metastases identified by SLN. Recurrence was more common among patients with thicker primary lesions and positive SLN. No significant differences in oncologic outcomes were observed with US surveillance and CLND following the identification of a positive SLN., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: The purpose of this study was to describe management and outcomes from a contemporary cohort of children with Wilms tumor complicated by inferior vena caval thrombus., Background: The largest series of these patients was published almost 2 decades ago. Since then, neoadjuvant chemotherapy has been commonly used to manage these patients, and outcomes have not been reported., Methods: Retrospective review of 19 North American centers between 2009 and 2019. Patient and disease characteristics, management, and outcomes were investigated and analyzed., Results: Of 124 patients, 81% had favorable histology (FH), and 52% were stage IV. IVC thrombus level was infrahepatic in 53 (43%), intrahepatic in 32 (26%), suprahepatic in 14 (11%), and cardiac in 24 (19%). Neoadjuvant chemotherapy using a 3-drug regimen was administered in 82% and postresection radiation in 90%. Thrombus level regression was 45% overall, with suprahepatic level showing the best response (62%). Cardiopulmonary bypass (CPB) was potentially avoided in 67%. The perioperative complication rate was significantly lower after neoadjuvant chemotherapy [(25%) vs upfront surgery (55%); P =0.005]. CPB was not associated with higher complications [CPB (50%) vs no CPB (27%); P =0.08]. Two-year event-free survival was 93% and overall survival was 96%, higher in FH cases (FH 98% vs unfavorable histology/anaplastic 82%; P =0.73). Neither incomplete resection nor viable thrombus cells affected event-free survival or overall survival., Conclusions: Multimodal therapy resulted in excellent outcomes, even with advanced-stage disease and cardiac extension. Neoadjuvant chemotherapy decreased the need for CPB to facilitate resection. Complete thrombectomy may not always be necessary., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc.)

Objective: To investigate whether perioperative calcium and 1,25 OH vitamin D supplementation (PCDS) influences the rates of postoperative hypocalcemia and length of stay (LOS) following pediatric thyroidectomy., Study Design: Retrospective Cohort Review., Setting: Tertiary children's hospital., Methods: 94 patients who underwent completion or total thyroidectomy with or without concomitant neck dissection from 2010 to 2020 at a single institution were included. Patients with pre-existing hypocalcemia or preoperative vitamin D insufficiency were excluded. Rates of postoperative hypocalcemia and LOS were compared for patients receiving PCDS to those receiving no supplementation., Results: Thirty percent of patients with PCDS had documented postoperative hypocalcemia compared to 64% of patients without PCDS (p = 0.01). Patients with PCDS had a median LOS of 30 h compared to 36 h (p = 0.002). Multivariable analyses confirmed that patients with PCDS had lower odds of postoperative hypocalcemia (OR: 0.32, CI: 0.11, 0.89) and shorter LOS by 17 h (SE: 8, p = 0.04) after adjustment for confounders., Conclusion: PCDS is associated with significantly lower risk of hypocalcemia and shorter LOS. Standardizing preoperative care for pediatric patients undergoing thyroidectomy may decrease variability and improve outcomes following surgery., Competing Interests: Declaration of competing interest None., (Copyright © 2024 Elsevier B.V. All rights reserved.)

Introduction: Surveillance following sacrococcygeal teratoma (SCT) resection varies. The purpose of this study was to describe the clinical characteristics and outcomes of patients undergoing SCT resection and examine current institutional practices to detect recurrence., Methods: A single-institution retrospective review of children who underwent resection of an SCT from January 1, 2010 to December 31, 2020 was performed. Data were summarized and surveillance strategies compared between histopathologic subtypes using nonparametric methods., Results: Thirty six patients (75.0% female) underwent SCT removal at a median age of 8 d. Histopathology revealed 27 mature teratomas (75.0%), eight immature teratomas (22.2%), and one malignant germ cell tumor (2.8%). Median postoperative follow-up was 3.17 y (interquartile range [IQR]: 2.31-4.38 y). Patients had a median of 2.32 clinic visits per year (IQR: 2.00-2.70), alpha-fetoprotein levels were obtained at a median of 2.01 times per year (IQR: 0-1.66), and surveillance imaging was performed at a median of 2.31 times per year (IQR: 0-2.84). Patients with immature teratomas had alpha-fetoprotein laboratories obtained more frequently than patients with mature teratomas (3.10 times/year versus 0.93 times/year, P = 0.001). There was no significant difference in the number of imaging studies obtained between groups. Two patients (5.6%) developed recurrence, which were identified on magnetic resonance imaging at 191 and 104 d postresection, respectively., Conclusions: Postoperative surveillance practices varied widely. Recurrence was noted in a single malignant case in the first year following resection. Multi-institutional studies are needed to determine the optimal surveillance strategy to detect recurrence of SCT., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: Multiple surgical specializations are involved in the operative management of pediatric thyroid disease, but current practice patterns remain unknown. The objective of this study was to examine current practice patterns in the operative management of pediatric thyroid disease, specifically comparing practices across different surgical specializations including pediatric surgery, pediatric otolaryngology, general surgery, adult otolaryngology, and endocrine-focused general surgery., Methods: Children 0-18 years-old undergoing thyroid surgery from 2015 to 2019 were identified using the Healthcare Cost and Utilization Project State Inpatient Databases and State Ambulatory Surgery and Services Databases across 6 states. Surgeon specialization was determined for all included surgeons. Patient and hospital characteristics were compared across surgical specializations. Clinical outcomes including hypocalcemia/hypoparathyroidism, recurrent laryngeal nerve injury, hematoma, and wound infection were assessed., Results: A total of 1241 pediatric thyroidectomies performed by 363 surgeons were included. Procedures were most frequently performed by pediatric surgeons (34.9%). Only 7.2% of procedures were performed by adult general surgeons. There were statistically significant differences in patient age, sociodemographics, surgical indications, and type of procedure performed between specializations (p < 0.05). Endocrine-focused general surgeons had the highest average annual thyroid procedure volume with 78.2 cases/year, and pediatric surgeons and pediatric otolaryngologists had the lowest volumes with 0.7 and 0.6 cases/year, respectively. Overall complication rates were low., Conclusions: Operative management of pediatric thyroid disease was most frequently performed by pediatric surgery. Pediatric specializations are more likely to operate on low-income, minority children with public insurance and patients with Graves' disease. Overall complications were low., Level of Evidence: III., Competing Interests: Declarations of interest All authors declare they have no conflicts of interest or financial disclosures, including financial, consultant, institutional and other relationships that may lead to bias or conflict of interest., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: Nephrogenic rests (NR) may represent precursor lesions for Wilms tumor (WT), but their clinical course is not fully understood and no guidelines for treatment exist. This study sought to evaluate the outcomes of pediatric patients with NRs related to traditional chemotherapy and surgery., Methods: A PRISMA-P-compliant literature search was conducted in MEDLINE, Embase, CINAHL, Web of Science, COCHRANE, and SCOPUS from inception to June 2021. Clinical questions regarding the treatment of NRs, including chemotherapy and surgery, were developed in the population, intervention, comparison, and outcome format., Results: Twenty-five studies including 1445 patients met inclusion criteria for evaluating chemotherapy compared to observation for NRs. Eighteen studies including 1392 patients met inclusion criteria for evaluating the role of surgery for NRs. Patients with isolated NRs who underwent observation progressed to WT 33% of the time; chemotherapy reduced the rate of WT to 3.9%. Observation of multiple NRs and diffuse hyperplastic perilobar nephroblastomatosis (DHPLN) led to progression to WT 50% and 100% of the time, respectively. Chemotherapy reduced the rate of WT to 34% for multiple NRs and 46% for DHPLN. Surgery for isolated NRs reduced the risk of WT development from 23.7% in patients with incomplete excision to 3.3% with complete excision; however, 96% of patients with incompletely excised NRs had bilateral disease., Conclusions: Observation with close surveillance for isolated NRs is safe. Treatment with chemotherapy is recommended for patients with multiple NRs and DHPLN. Surgical management of NRs should focus on renal function preservation., Level of Evidence: Treatment study, Level III., Competing Interests: Conflicts of interest The authors have nothing to declare., (Copyright © 2023 The Author(s). Published by Elsevier Inc. All rights reserved.)

Although survival for many pediatric cancers has improved with advances in conventional chemotherapeutic regimens and surgical techniques in the last several decades, it remains a leading cause of disease-related death in children. Outcomes in patients with recurrent, refractory, or metastatic disease are especially poor. Recently, the advent of alternative classes of therapies, including immunotherapies, have revolutionized systemic treatment for pediatric malignancies. Several classes of immunotherapies, including chimeric antigen receptor (CAR) T cell therapy, transgenic T-cell receptor (TCR)-T cell therapy, bispecific T-cell engagers, and monoclonal antibody checkpoint inhibitors have been FDA-approved or entered early-phase clinical trials in children and young adults. The pediatric surgeon is likely to encounter these therapies during the care of children with malignancies and should be familiar with the classes of therapy, indications, adverse events, and potential need for surgical intervention in these cases. This review from the APSA Cancer Committee offers a brief discussion of the three most encountered classes of immunotherapy in children and young adults and discusses surgical relevance. LEVEL OF EVIDENCE: IV., Competing Interests: Conflict of interest The authors have no competing interests in this work., (Copyright © 2023 Elsevier Inc. All rights reserved.)

As non-operative management of acute appendicitis in children has become more common, missed incidental appendiceal pathology can be an unintended consequence. We assessed the prevalence of neuroendocrine tumors in appendectomy specimens from eight US children's hospitals from 2012 to 2021. The prevalence of neuroendocrine tumors (NET) was found to be 1:271, with a median age of 14 years and 62% female. Most tumors were small (median 6 mm; interquartile range [IQR]: 3-10), and no recurrence was noted during the follow-up period (median 22.5 months; IQR: 3-53). The possibility of delayed diagnosis of these tumors should be part of the discussion for non-operative management of pediatric acute appendicitis., (© 2023 Wiley Periodicals LLC.)

Children with renal masses require surgical management to provide accurate surgical staging and skilled resection of the tumor. This document includes evidence-based recommendations for pediatric surgeons regarding the resection, staging, and proper nodal basin evaluation., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Neuroblastoma (NB) is the most common solid extracranial malignancy of childhood with an incidence of 1 per 100,000 in the United States compromising approximately 10 % of childhood cancer. Unfortunately, patients with high-risk NG continue to have long-term survival less than 50 %. Both Children's Oncology Group and the International Society of Paediatric Oncology have demonstrated the important role of surgery in the treatment of high-risk NB. Herein, we compose the results of an extensive literature review as well as expert opinion from leaders in pediatric surgical oncology, to present the critical elements of effective surgery for high-risk neuroblastoma., (Copyright © 2023. Published by Elsevier Inc.)

Every year, approximately 600 infants, children, and adolescents are diagnosed with renal cancer in the United States. In addition to Wilms tumor (WT), which accounts for about 80% of all pediatric renal cancers, clear cell sarcoma of the kidney, renal cell carcinoma, malignant rhabdoid tumor, as well as more rare cancers (other sarcomas, rare carcinomas, lymphoma) and benign tumors can originate within the kidney. WT itself can be divided into favorable histology (FHWT), with a 5-year overall survival (OS) exceeding 90%, and anaplastic histology, with 4-year OS of 73.7%. Outcomes of the other pediatric renal cancers include clear cell sarcoma (5-year OS: 90%), malignant rhabdoid tumor (5-year OS: 10% for stages 3 and 4), and renal cell carcinoma (4-year OS: 84.8%). Recent clinical trials have identified novel biological prognostic markers for FHWT, and a series of Children's Oncology Group (COG) trials have demonstrated improving outcomes with therapy modification, and opportunities for further care refinement., (© 2023 Wiley Periodicals LLC.)

Background: Intraoperative nerve monitoring (IONM) is a technique used to decrease the possibility of nerve-associated morbidity and damage to nearby neural structures during complex surgical procedures. The use and potential benefits of IONM in pediatric surgical oncology are not well-described., Methods: An overview of the current literature was performed to elucidate the various techniques that may be useful to pediatric surgeons for resection of solid tumors in children., Results: The physiology and common types of IONM relevant to the pediatric surgeon are described. Important anesthetic considerations are reviewed. Specific applications for IONM that may be useful in pediatric surgical oncology are then summarized, including its use for monitoring the recurrent laryngeal nerve, the facial nerve, the brachial plexus, spinal nerves, and lower extremity nerves. Troubleshooting techniques regarding common pitfalls are then proposed., Conclusion: IONM is a technique that may be beneficial in pediatric surgical oncology to minimize nerve injury during extensive tumor resections. This review aimed to elucidate the various techniques available. IONM should be considered as an adjunct for the safe resection of solid tumors in children in the proper setting with the appropriate level of expertise. A multidisciplinary approach is advised. Additional studies are necessary to further clarify the optimal use and outcomes in this patient population., Levels of Evidence: Level III., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: Postoperative pneumonia is the third most common surgical complication and can seriously impair surgical rehabilitation and lead to related morbidity and mortality. We evaluated the temporal trends in racial and ethnic disparities in postoperative pneumonia and quantified the economic burden resulting from these inequalities in the United States., Methods: This population-based study includes 195 028 children (weighted to 964 679) admitted for elective surgery across 5340 US hospitals reporting to the Nationwide Inpatient Sample between 2010 and 2018. We estimated the risk-adjusted incidence of postoperative pneumonia, comparing racial and ethnic groups. We also quantified the inflation-adjusted hospital costs attributable to racial and ethnic disparities in postoperative pneumonia., Results: The risk-adjusted rates of pneumonia declined across all racial and ethnic categories, with Black children having the lowest annual rate of decline (Black: 0.03 percentage points, Hispanic: 0.05 percentage points, white: 0.05 percentage points). The risk-adjusted rates of pneumonia trended consistently higher for Black and Hispanic children, relative to white children, throughout the study period (Black versus white: relative risk, 1.31 (95% confidence interval, 1.14-1.51), P < .01; Hispanic versus white: relative risk, 1.16 (95% confidence interval, 1.02-1.32), P = .02). These disparities did not narrow significantly over time. During the study period, the excess hospitalization cost attributable to racial and ethnic disparities in postoperative pneumonia was $24 533 458 for Black children and $26 200 783 for Hispanic children (total, $50 734 241)., Conclusions: Against the backdrop of decreasing postoperative pneumonia, Black and Hispanic children continue to experience higher rates compared with white children. These persistent disparities in postoperative pneumonia were associated with considerable excess cost of surgical care., (Copyright © 2023 by the American Academy of Pediatrics.)

The diagnosis of multiple or diffuse renal lesions in a child is challenging by imaging and/or pathology. Optimal management requires distinguishing benign lesions such as nephrogenic rests from cancerous lesions such as Wilms tumor, but this is often difficult or impossible. This difficulty is compounded by the overlapping nature of our current radiologic and pathologic definitions of lesions along the spectrum of nephrogenic rests/nephroblastomatosis. We provide a review of these issues, as a collaborative effort between the Children's Oncology Group Renal Tumor Committee and International Society of Pediatric Oncology Renal Tumor Study Group. Our aim is to discuss current challenges in diagnosis and management of these renal lesions, encouraging future work toward consensus definitions for research and patient care., (© 2022 Wiley Periodicals LLC.)

Study Objective: Describe the current practice patterns and diagnostic accuracy of frozen section (FS) pathology for children and adolescents with ovarian masses DESIGN: Prospective cohort study from 2018 to 2021 SETTING: Eleven children's hospitals PARTICIPANTS: Females age 6-21 years undergoing surgical management of an ovarian mass INTERVENTIONS: Obtaining intraoperative FS pathology MAIN OUTCOME MEASURE: Diagnostic accuracy of FS pathology RESULTS: Of 691 patients who underwent surgical management of an ovarian mass, FS was performed in 27 (3.9%), of which 9 (33.3%) had a final malignant pathology. Among FS patients, 12 of 27 (44.4%) underwent ovary-sparing surgery, and 15 of 27 (55.5%) underwent oophorectomy with or without other procedures. FS results were disparate from final pathology in 7 of 27 (25.9%) cases. FS had a sensitivity of 44.4% and specificity of 94.4% for identifying malignancy, with a c-statistic of 0.69. Malignant diagnoses missed on FS included serous borderline tumor (n = 1), mucinous borderline tumor (n = 2), mucinous carcinoma (n = 1), and immature teratoma (n = 1). FS did not guide intervention in 10 of 27 (37.0%) patients: 9 with benign FS underwent oophorectomy, and 1 with malignant FS did not undergo oophorectomy. Of the 9 patients who underwent oophorectomy with benign FS, 5 (55.6%) had benign and 4 (44.4%) had malignant final pathology., Conclusions: FSs are infrequently utilized for pediatric and adolescent ovarian masses and could be inaccurate for predicting malignancy and guiding operative decision-making. We recommend continued assessment and refinement of guidance before any standardization of use of FS to assist with intraoperative decision-making for surgical resection and staging in children and adolescents with ovarian masses., (Copyright © 2022. Published by Elsevier Inc.)

Objective: To determine the impact of tumor characteristics and treatment approach on (1) local recurrence, (2) scoliosis development, and (3) patient-reported quality of life in children with sarcoma of the chest wall., Summary of Background Data: Children with chest wall sarcoma require multimodal therapy including chemotherapy, surgery, and/or radiation. Despite aggressive therapy which places them at risk for functional impairment and scoliosis, these patients are also at significant risk for local recurrence., Methods: A multi-institutional review of 175 children (median age 13 years) with chest wall sarcoma treated at seventeen Pediatric Surgical Oncology Research Collaborative institutions between 2008 and 2017 was performed. Patient-reported quality of life was assessed prospectively using PROMIS surveys., Results: The most common diagnoses were Ewing sarcoma (67%) and osteosarcoma (9%). Surgical resection was performed in 85% and radiation in 55%. A median of 2 ribs were resected (interquartile range = 1-3), and number of ribs resected did not correlate with margin status ( P = 0.36). Local recurrence occurred in 23% and margin status was the only predictive factor(HR 2.24, P = 0.039). With a median follow-up of 5 years, 13% developed scoliosis (median Cobb angle 26) and 5% required corrective spine surgery. Scoliosis was associated with posteriorrib resection (HR 8.43; P= 0.003) and increased number of ribs resected (HR 1.78; P = 0.02). Overall, patient-reported quality of life is not impaired after chest wall tumor resection., Conclusions: Local recurrence occurs in one-quarter of children with chest wall sarcoma and is independent of tumor type. Scoliosis occurs in 13% of patients, but patient-reported quality of life is excellent., Competing Interests: The authors declare no conflict of interest., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Wilms tumor (WT) is the most common renal malignancy in children. Children with favorable histology WT achieve survival rates of over 90%. Twelve percent of patients present with metastatic disease, most commonly to the lungs. The presence of a pleural effusion at the time of diagnosis of WT may be noted on staging imaging; however, minimal data exist regarding the significance and prognostic importance of this finding. The objectives of our study are to identify the incidence of pleural effusions in patients with WT, and to determine the potential impact on oncologic outcomes. A multi-institutional retrospective review was performed from January 2009 to December 2019, including children with WT and a pleural effusion on diagnostic imaging treated at Pediatric Surgical Oncology Research Collaborative (PSORC) participating institutions. Of 1259 children with a new WT diagnosis, 94 (7.5%) had a pleural effusion. Patients with a pleural effusion were older than those without (median 4.3 vs 3.5 years; P = .004), and advanced stages were more common (local stage III 85.9% vs 51.9%; P < .0001). Only 14 patients underwent a thoracentesis for fluid evaluation; 3 had cytopathologic evidence of malignant cells. Event-free and overall survival of all children with WT and pleural effusions was 86.2% and 91.5%, respectively. The rate and significance of malignant cells present in pleural fluid is unknown due to low incidence of cytopathologic analysis in our cohort; therefore, the presence of an effusion does not appear to necessitate a change in therapy. Excellent survival can be expected with current stage-specific treatment regimens., (© 2022 UICC.)