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A new generation of disease-specific molecular imaging agents is poised to revolutionize fluorescence-guided surgery. Pafolacianine has been approved for adult lung and ovarian cancers. We demonstrate a proof of concept for pediatric surgeons treating young adults with pulmonary metastatic sarcomas. Five successful fluorescence-guided pulmonary metastasectomy operations were performed in young adult patients with metastatic osteosarcoma or Ewing sarcoma following administration of pafolacianine. All osteosarcoma lesions identified using standard techniques were also markedly fluorescent in patients. Novel fluorescent molecular agents targeted to tumor-specific receptors have promise of increased sensitivity and specificity for detecting metastatic nodules and enhancing surgical clearance of disease., (© 2024 Wiley Periodicals LLC.)

Objective: To evaluate the impact of prophylactic antibiotics on early infectious complications after central venous access device (VAD) placement in children with cancer., Background: Despite the frequency of VAD procedures in children, the effectiveness of prophylactic antibiotics for reducing infectious complications is unknown., Methods: This was a retrospective cohort study of children with cancer undergoing central VAD placement identified in the Pediatric Health Information System database between 2017 and 2021. The primary outcome was the rate of early infectious complications (composite surgical site infections, central line-associated bloodstream infections, and bacteremia). Multivariable logistic regression was used to evaluate factors associated with early infection, and heterogeneity of treatment effect of prophylactic antibiotics was compared across subgroups., Results: A total of 9216 patients were included (6058 ports and 3158 tunneled lines). Prophylactic antibiotics were associated with lower early infectious complications overall [1.3% vs 2.4%; odds ratio (OR): 0.55 (95% CI: 0.39-0.79), P < 0.001], an effect demonstrated for tunneled lines (OR: 0.59, 95% CI: 0.41-0.84) but not ports (OR: 3.01, 95% CI: 0.66-13.78). On multivariate analysis, prophylactic antibiotics (OR: 0.67, 95% CI: 0.45-0.97) and solid tumors (OR: 0.38, 95% CI: 0.22-0.64) were associated with reduced odds of early infections, whereas tunneled lines (OR: 20.78, 95% CI: 9.83-43.93) and acute myelogenous leukemia (OR: 2.37, 95% CI: 1.58-3.57) had increased odds., Conclusions: Prophylactic antibiotics are associated with reduced early infectious complications after central VAD placement overall. Despite recommendations from multiple national organizations against prophylactic antibiotics, these findings suggest a benefit in children with malignancy undergoing tunneled line placement., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

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.)

Non-rhabdomyosarcoma soft tissue sarcoma (STS) comprises most STS in pediatric patients. It is a diverse set of over 30 histologic subtypes. Treatment is based on risk group determined by tumor size, grade, and the presence of metastases. Surgical resection is a cornerstone of therapy, as tumors are often resistant to chemotherapy or radiation. While patients with isolated tumors less than 5 cm may undergo upfront resection, strong consideration should be given to neoadjuvant chemoradiotherapy to ensure negative margins at surgical resection and optimal outcomes. Sentinel lymph node biopsy is strongly recommended for clear cell and epithelioid sarcomas. The most common metastatic site is the lung, and metastases should be resected at the end of therapy, when feasible. Unfortunately, many high-risk patients progress on therapy, and alternative strategies including earlier metastatic control require investigation., (© 2024 Wiley Periodicals LLC.)

Rhabdomyosarcoma is the most common pediatric soft tissue sarcoma, and 5-year overall survival exceeds 70%. With more long-term survivors, it is critical to understand the frequency of late events, including recurrence, second malignant neoplasm, and death, occurring 5 years after diagnosis, and the variables associated with these events. We report late events in patients enrolled on Intergroup Rhabdomyosarcoma Study Group and Children's Oncology Group trials from 1997 to 2013 including D9602, D9803, D9802, ARST0331, ARST0431, ARST0531, and ARST08P1. A late event occurred in 2.9% of 5-year event-free survivors supporting guidelines to limit surveillance for these events to 5 years from diagnosis., (© 2024 Wiley Periodicals LLC.)

Background and Aims: Indocyanine Green Fluorescence (ICG-F)- guided surgery is becoming an increasingly helpful tool in pediatric surgical care. This consensus statement investigates the utility of ICG-F in various pediatric surgical applications, primarily focusing on its evidence base, safety, indications, use across different surgical specialties and dosing strategies. The aim is to establish an international consensus for ICG-F use in pediatric surgery., Methods: An international panel of 15 pediatric surgeons from 9 countries was assembled. The structured process consisted of a rapid scoping review, iterative discussion sessions, mixed-methods studies with key stakeholders, and voting rounds on individual statements to create draft consensus statements., Results: 100 articles were identified during the review and summarized by application. Based on this condensed evidence, consensus statements were generated after 3 iterative rounds of anonymous voting. Key areas of agreement were quality of evidence, the safety of ICG, pediatric surgical indications, utilization per surgical specialty, and dosing of ICG., Conclusion: This consensus statement aims to guide healthcare professionals in managing ICG-F use in pediatric surgical cases based on the best available evidence, key stakeholder consultation, and expert opinions. Despite ICG-F's promising potential, the need for higher-quality evidence, prospective trials, and safety studies is underscored. The consensus also provides a framework for pediatric surgeons to utilize ICG-F effectively., Level of Evidence: III., Competing Interests: Conflict of interest All authors have no disclosures or conflicts of interest., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Study Objective: Isolated tubal torsion (ITT) is a surgical emergency that is less well characterized than ovarian torsion and presents with its own diagnostic challenges. We retrospectively examined patients with ITT and compared them to patients with ovarian torsion to better understand the unique features of this pathophysiologic process., Methods: The medical records of patients who were identified as having ITT or ovarian torsion intraoperatively between January 2019 and November 2022 were reviewed. Patient demographics, clinical documentation, diagnostic imaging reports, operative reports, and pathology results were collected., Results: A total of 82 patients met inclusion criteria of which 62 (75.6%) had ovarian torsion and 20 (24.4%) had ITT. Most patients (63.4%) underwent 2 or more imaging studies prior to the operating room. A diagnosis of torsion occurred more often on preoperative imaging for the ovarian torsion group (67.7% ovarian torsion vs 35.0% ITT, p=0.01). Doppler flow to the ovary was expectedly absent in 65.9% of ovarian torsion patients but interestingly also absent in 25% of ITT patients (p=0.01). Patients with ovarian torsion were more likely to have ovarian cysts (56.5% vs 10.0%, p<0.001), while patients with ITT were significantly more likely to have a paratubal cyst (90.0% vs 38.7%, p<0.001)., Conclusion: ITT is a surgical emergency that poses a risk to future fertility. Ultrasound studies, including doppler, may not be as definitive in patients with ITT and early cross-sectional imaging should be considered. When there is a high clinical suspicion for ovarian torsion or ITT, there should be a low threshold for diagnostic laparoscopy., Competing Interests: Declaration of competing interest The authors have nothing to disclose., (Copyright © 2024. Published by Elsevier Inc.)

Background: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. Despite bone metastases being present in 5% of patients at diagnosis, there are limited studies examining these outcomes. We sought to define the prognostic factors, clinical courses, and outcomes of children treated on Children's Oncology Group (COG) clinical trials with RMS metastatic to bone at diagnosis., Methods: We performed a retrospective analysis of patients diagnosed with bone metastatic RMS enrolled on COG RMS clinical trials (D9802, D9803, ARST0431, or ARST08P1) between 1997 and 2013., Results: RMS metastatic to bone was identified in 154 patients at a median age of 14.9 years at diagnosis. Fifty-eight percent of patients were male, 90% had metastases at additional sites, 74% had alveolar histology, and extremity was the most common primary site (31%). Eighty-six percent of patients (n = 133) received radiation therapy. The 3- and 5-year event-free survival (EFS) was 15.4% and 14.5%, respectively. The 3- and 5-year overall survival (OS) was 30.4% and 18.0%, respectively. We identified alveolar histology, FOXO1 fusion presence, unfavorable primary location, higher Oberlin score, and lack of radiation as poor prognostic characteristics for both EFS and OS in univariate analysis. Lack of radiation was not significant when excluding patients with events prior to 20 weeks., Conclusions: This study is the largest analysis of patients with bone metastatic RMS, and defines the poor overall outcomes and negative prognostic factors for these patients. They may be eligible for therapy deintensification for improved quality of life or pursuit of novel treatments/approaches, which are desperately needed., (© 2024 Wiley Periodicals LLC.)

Gastroschisis is a congenital anomaly characterized by herniation of abdominal contents via a defect in the anterior abdominal wall. Gastroschisis can manifest as simple or complex, with additional complications such as atresia, perforation, ischemia, necrosis, or volvulus. While prenatal screening and advancements in surgical techniques have improved outcomes, infants with complex gastroschisis cases pose significant challenges in neonatal care. Vanishing gastroschisis, a rare but dreaded complication with a mortality rate ranging from 10 to 70%, occurs when the abdominal wall closes around the herniated bowel, leading to strangulation. We present a case report focusing on the management of neonatal gastroschisis in a 36-week-old female infant with vanishing gastroschisis. The infant's clinical course, including surgical interventions, complications, and multidisciplinary management, is discussed in detail. This case underscores the importance of a multidisciplinary approach in optimizing outcomes for infants with complex gastroschisis. Via this case report, we aim to provide insights into the complexities of neonatal gastroschisis management and advocate for a collaborative approach involving neonatology, pediatric surgery, infectious disease, and palliative care to improve outcomes and quality of life for affected infants.

Competing Interests: Declaration of Interests M.J.B. has nothing to disclose. D.F. has nothing to disclose. E.L.T. reports funding from NIH/NCI T32 CA009560, NRSA F30HD107966-01, and T32GM008152 outside the submitted work. T.B.L. has nothing to disclose. M.M.L. reports funding from National Institutes of Health U01HD110336 and National Institutes of Health R01HD104683 outside the submitted work. E.E.R. has nothing to disclose.

Surgery is a crucial component of pediatric cancer treatment, but conventional methods may lack precision. Image-guided surgery, including fluorescent and radioguided techniques, offers promise for enhancing tumor localization and facilitating precise resection. Intraoperative molecular imaging utilizes agents like indocyanine green to direct surgeons to occult deposits of tumor and to delineate tumor margins. Next-generation agents target tumors directly to improve specificity. Radioguided surgery, employing tracers like metaiodobenzylguanidine (MIBG), complements fluorescent techniques by allowing for detection of tumors at a greater depth. Dual-labeled agents combining both modalities are under development. Three-dimensional modeling and virtual/augmented reality aid in preoperative planning and intraoperative guidance. The above techniques show great promise to benefit patients with pediatric tumors, and their continued development will almost certainly improve surgical outcomes., (© 2024 The Author(s). Pediatric Blood & Cancer published by 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.)

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.)

Background: Undifferentiated embryonal sarcoma of the liver (UESL) is a rare tumor for which there are few evidence-based guidelines. The aim of this study was to define current management strategies and outcomes for these patients using a multi-institutional dataset curated by the Pediatric Surgical Oncology Research Collaborative., Methods: Data were collected retrospectively for patients with UESL treated across 17 children's hospitals in North America from 1989 to 2019. Factors analyzed included patient and tumor characteristics, PRETEXT group, operative details, and neoadjuvant/adjuvant regimens. Event-free and overall survival (EFS, OS) were the primary and secondary outcomes, respectively., Results: Seventy-eight patients were identified with a median age of 9.9 years [interquartile range [IQR): 7-12]. Twenty-seven patients underwent resection at diagnosis, and 47 patients underwent delayed resection, including eight liver transplants. Neoadjuvant chemotherapy led to a median change in maximum tumor diameter of 1.6 cm [IQR: 0.0-4.4] and greater than 90% tumor necrosis in 79% of the patients undergoing delayed resection. R 0 resections were accomplished in 63 patients (81%). Univariate analysis found that metastatic disease impacted OS, and completeness of resection impacted both EFS and OS, while multivariate analysis revealed that R 0 resection was associated with decreased expected hazards of experiencing an event [hazard ratio (HR): 0.14, 95% confidence interval (CI): 0.04-0.6]. At a median follow-up of 4 years [IQR: 2-8], the EFS was 70.0% [95% CI: 60%-82%] and OS was 83% [95% CI: 75%-93%]., Conclusion: Complete resection is associated with improved survival for patients with UESL. Neoadjuvant chemotherapy causes minimal radiographic response, but significant tumor necrosis., (© 2024 Wiley Periodicals LLC.)

Introduction: The impact of established prognostic factors on survival outcomes for childhood rhabdomyosarcoma (RMS) have not been well described in the adolescent and young adult (AYA) RMS patient population., Methods: This is a retrospective analysis of patients with newly diagnosed RMS enrolled between 1997 and 2016 on seven previously reported Children's Oncology Group (COG) clinical trials. Demographics, clinical features, treatment details, and outcome data were collected. Five-year event-free survival (EFS) and overall survival (OS) were estimated for patients diagnosed at age 15-39 years and those diagnosed under age 15 years using the Kaplan-Meier method. Log-rank test was used to compare prognostic factors for EFS and OS. Factors significant in the univariable analysis were included in a Cox proportional hazards regression model. Nonsignificant covariates were removed from the multiple regression model., Results: Total 2151 patients including 402 AYAs were analyzed. AYAs were more likely to present with primary tumors ≥5 cm in size, metastatic disease, alveolar histology, and have FOXO1 fusions compared to children. Five-year EFS for the AYA cohort was 44.2% versus 67% for children (p < .001), and 5-year OS was 52% for the AYA cohort versus 78% for children (p < .001). Multivariable analysis revealed tumor site, size and invasiveness, clinical group, and histology were prognostic in AYAs., Conclusion: AYAs with RMS have a poorer prognosis compared to younger children due to multiple factors. Further research focused on AYAs to better understand RMS biology and improve treatments is critical to improve survival., (© 2024 Wiley Periodicals LLC.)

Surgery plays a crucial role in the treatment of children with solid malignancies. A well-conducted operation is often essential for cure. Collaboration with the primary care team is important for determining if and when surgery should be performed, and if performed, an operation must be done in accordance with well-established standards. The long-term consequences of surgery also need to be considered. Indications and objectives for a procedure vary. Providing education and developing and analyzing new research protocols that include aims relevant to surgery are key objectives of the Surgery Discipline of the Children's Oncology Group. The critical evaluation of emerging technologies to ensure safe, effective procedures is another key objective. Through research, education, and advancing technologies, the role of the pediatric surgeon in the multidisciplinary care of children with solid malignancies will continue to evolve., (© 2023 Wiley Periodicals LLC.)

Background: Intercostal nerve cryoablation (INC) has shown promise as an adjunct method for analgesia in adults undergoing thoracotomy, but has yet to be widely used in children for this indication. We hypothesize that INC decreases opioid utilization in children undergoing thoracotomy for cancer operations., Methods: A retrospective review was performed of children who underwent thoracotomy for cancer diagnosis at a freestanding children's hospital from 2018 to 2023. Patient characteristics, intraoperative data, and data on clinical course were collected. Patients were divided into those who underwent INC and those who underwent routine care for comparison., Results: Twenty-six patients underwent 38 procedures at a median age of 16 years (range 5-21 years). INC was performed in 23 cases over a median of five intercostal levels (range 2-7). Total oral morphine equivalents during inpatient admission were significantly lower in INC patients (137.6 vs. 514.5 mg, p = .002). Routine care patients were more likely to be discharged with an opioid prescription (30.4% vs. 80.0%, p = .008). Length of stay was similar between patients with INC and routine care (4 vs. 5 days, p = .15). There were no differences in rates of reoperation or 30-day re-admission (emergency department or inpatient)., Conclustions: INC is a feasible and safe adjunct for children undergoing thoracotomy for cancer. INC is associated with reduced postoperative opioid utilization with respect to both inpatient use and outpatient prescriptions., (© 2023 The Authors. Pediatric Blood & Cancer published by Wiley Periodicals LLC.)

Background: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. Metastatic disease occurs in 16% of all RMS cases and has a poor prognosis. There are limited studies examining the outcomes specific to patients with RMS metastatic to bone marrow despite an incidence of 6% at diagnosis. Our study aims to document the outcomes, prognostic factors, and clinical courses of children presenting with RMS metastatic to bone marrow treated on Children's Oncology Group (COG) cooperative trials., Methods: We performed a retrospective analysis of the patients diagnosed with RMS metastatic to bone marrow between 1997 and 2013 enrolled on one of four COG RMS clinical trials of D9802, D9803, ARST0431, and ARST08P1., Results: We identified 179 cases with RMS metastatic to bone marrow. Patients had a median age of 14.8 years, 58% were male, predominantly alveolar histology (76%), extremity was the most common primary site (32%), and 87% had metastatic disease to additional sites; 83% (n = 149) received radiation as a treatment modality. The 3- and 5-year event-free survival was 9.4% and 8.2%, respectively. The 3- and 5-year overall survival was 26.1% and 12.6%, respectively. Age ≥10 years, alveolar histology, FOXO1 fusion presence, unfavorable primary location, higher Oberlin score, and lack of radiation were identified as poor prognostic/predictive characteristics., Conclusions: This study represents the largest analysis of RMS metastatic to bone marrow, defining the poor prognostic outcome for these patients. These patients may be eligible for therapy deintensification or early pursuit of novel treatments/approaches that are desperately needed., (© 2023 Wiley Periodicals LLC.)

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.)

Rhabdomyosarcoma (RMS), the most common soft tissue sarcoma in children, requires multimodal therapy which is determined by risk group stratification. Local control may be achieved by surgical resection, radiation, or both. Resection may occur upfront or following induction chemotherapy as a delayed primary excision. An R1 resection may allow a reduction in radiation exposure; however, debulking is not indicated nor is excision of residual masses at the end of therapy. Regional lymph node assessment is an important component of surgical care, as positive nodal basins require radiation. Depending on the tumor site and biology, sentinel lymph node biopsy vs biopsy of clinically or radiographically concerning nodes is indicated. Therapeutic lymph node dissection is never indicated. Familiarity with site-specific oncologic principles for RMS and participation in a multidisciplinary team including Pediatric Oncology and Radiation Oncology are necessary components of surgical care to ensure optimal outcomes., (Copyright © 2023. Published by Elsevier Inc.)

Innovation is essential to the advancement of the field of pediatric surgery. The natural skepticism toward new technologies in pediatrics leads to frequent confusion of surgical innovation and research. Using fluorescence-guided surgery as an archetype for this ethical discussion, we apply existing conceptual frameworks of surgical innovation to understand the distinction between innovation and experimentation, acknowledging the spectrum and "grey zone" in between. In this review, we discuss the role of Institutional Review Boards in evaluating surgical practice innovations, and the aspects of certain surgical innovations that are distinct from experimentation, including a thorough understanding of the risk profile, preexisting use in humans, and adaptation from related fields. Examining fluorescence-guided surgery through these existing frameworks as well as the concept of equipoise, we conclude that new applications of indocyanine green do not constitute human subjects research. Most importantly, this example gives practitioners a lens through which they may appraise potential surgical innovations to allow for a sensible and efficient improvement of the field of pediatric surgery. LEVEL OF EVIDENCE: V., Competing Interests: Conflicts of interest The authors have no conflicts of interest relevant to this article to disclose., (Copyright © 2023 Elsevier Inc. All rights reserved.)

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.)

Introduction: Rhabdomyosarcoma (RMS) of the chest wall presents unique management challenges and local control considerations. The benefit of complete excision is uncertain and must be weighed against potential surgical morbidity. Our aim was to assess factors, including local control modality, associated with clinical outcomes in children with chest wall RMS., Methods: Forty-four children with RMS of the chest wall from low-, intermediate-, and high-risk Children's Oncology Group studies were reviewed. Predictors of local failure-free survival (FFS), event-free survival (EFS), and overall survival (OS) were assessed, including clinical characteristics and staging, primary tumor anatomic locations, and local control modalities. Survival was assessed by Kaplan-Meier analysis and the log-rank test., Results: Tumors were localized in 25 (57%) and metastatic in 19 (43%), and they involved the intercostal region (52%) or superficial muscle alone (36%). Clinical group was I (18%), II (14%), III (25%), and IV (43%), and ultimately 19 (43%) patients had surgical resection (upfront or delayed), including 10 R0 resections. Five-year local FFS, EFS, and OS were 72.1%, 49.3%, and 58.5%, respectively. Univariate factors associated with local FFS included age, International Rhabdomyosarcoma Study (IRS) group, extent of surgical excision, tumor size, superficial tumor location, and presence of regional or metastatic disease. Other than tumor size, the same factors were associated with EFS and OS., Conclusions: Chest wall RMS has variable presentation and outcome. Local control is a significant contributor to EFS and OS. Complete surgical excision, whether upfront or after induction chemotherapy, is usually only possible for smaller tumors confined to the superficial musculature but is associated with improved outcomes. While overall outcomes remain poor for patients with initially metastatic tumors, regardless of local control modality, complete excision may be beneficial for patients with localized tumors if it can be achieved without excess morbidity., (© 2023 The Authors. Pediatric Blood & Cancer published by Wiley Periodicals LLC.)

The International Soft-Tissue Sarcoma Consortium (INSTRuCT) was founded as an international collaboration between different pediatric soft-tissue sarcoma cooperative groups (Children's Oncology Group, European Pediatric Soft-Tissue Sarcoma Group, and Cooperative Weichteilsarkom Studiengruppe). Besides other tasks, a major goal of INSTRuCT is to develop consensus expert opinions for best clinical treatment. This consensus paper for patients with rhabdomyosarcoma of the female genital tract (FGU-RMS) provides treatment recommendations for local treatment, long-term follow-up, and fertility preservation. Therefore, a review of the current literature was combined with recommendations of the treatment protocols of the appropriate clinical trials. Additionally, opinions of international FGU-RMS experts were incorporated into recommendations. Results were that the prognosis of FGU-RMS is favorable with an excellent response to chemotherapy. Initial complete surgical resection is not indicated, but diagnosis should be established properly. In patients with tumors localized at the vagina or cervix demonstrating incomplete response after induction chemotherapy, local radiotherapy (brachytherapy) should be carried out. In patients with persistent tumors at the corpus uteri, hysterectomy should be performed. Fertility preservation should be considered in all patients. In conclusion, for the first time, an international consensus for the treatment of FGU-RMS patients could be achieved, which will help to harmonize the treatment of these patients in different study groups., (© 2020 Wiley Periodicals LLC.)

Background: Margin status following surgery in children, adolescents, and young adults with soft tissue sarcomas is controversial and has been defined differently by various specialties, with definitions changing over time and by cooperative group. The International Soft Tissue Sarcoma Consortium (INSTRuCT) is a collaboration of the Children's Oncology Group (COG) Soft Tissue Sarcoma Committee, European pediatric Soft Tissue sarcoma Study Group (EpSSG), and the European Cooperative Weichteilsarkom Studiengruppe (CWS) devoted to improving patient outcomes by pooling and mining cooperative group clinical trial data., Methods: The INSTRuCT non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) working group aimed to develop international harmonized recommendations regarding surgical margin assessment and definitions in children and adolescents with soft tissue tumors., Results and Conclusion: This review addresses accepted principles and areas of controversy, including the perspectives of surgeons, pathologists, radiation oncologists, and pediatric oncologists, to develop a framework for building common guidelines for future research., (© 2023 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.)

RMS most commonly presents in children and adolescents, however a subset of tumors are diagnosed in infants under one year of age. Due to the rarity of infant RMS, utilization of different treatment approaches and goals, and small sample sizes, the published studies of infants with RMS have yielded heterogeneous results. In this review, we discuss the outcomes of infants with RMS treated in various clinical trials and the strategies that various international cooperative groups have employed to reduce the morbidity and mortality related to treatment without compromising the overall survival of this population. This review discusses the unique scenarios of diagnosing and managing congenitals or neonatal RMS, spindle cell RMS and relapsed RMS. This review concludes by exploring novel approaches to diagnosis and management of infants with RMS that are currently being studied by various international cooperative groups.

Background: Fluorescence-guided surgery (FGS) with indocyanine green (ICG) is a rapidly diffusing surgical innovation, but its utilization in pediatrics remains unknown. We present a cross-sectional descriptive analysis of trends from a national database., Methods: The Pediatric Health Information System (PHIS) database was queried for patient encounters between January 2016 and July 2021 with an associated ICG administration within 3 days prior to surgery. All procedure codes from each encounter were reviewed by two surgeons to determine the most likely associated FGS procedure and assign an operative category., Results: 1270 encounters were identified from 38 participating hospitals. The mean patient age (SD) was 8.3 (6.4) years, 54.5% were male, 63.8% were white, and 30.1% were Hispanic. The most common categories for ICG use were neurosurgery (21.3%), biliary (18.3%), perfusion (14.8%), urology (12.5%), gastrointestinal (10.8%), ophthalmology (8.8%), and thoracic (5.6%). Utilization over time increased for some categories (thoracic, visceral perfusion, and neurological procedures) or remained stable for other categories. Overall ICG utilization has increased in 2020 (n = 314) compared to 2016 (N = 83). The number of centers utilizing ICG has also increased from 14 hospitals in 2016 to 29 hospitals in 2020 though adoption remains unevenly distributed, with 5 high-utilization hospitals accounting for 56.8% of all ICG FGS cases., Conclusion: ICG is being used across a wide variety of pediatric surgical disciplines. Trends over time show increasingly frequent adoption across the country, with a few high-volume centers driving the innovation. Fluorescence-guided surgery is commercially available and is becoming more commonplace for pediatric surgeons. Dedicated efforts will now be needed to assess outcomes using this promising technology., Level of Evidence: Level IV., Study Type: Retrospective study., (Copyright © 2022 Elsevier Inc. All rights reserved.)

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.)

Background: Malignant melanoma is rare in the pediatric population and management is largely extrapolated from adult guidelines. Adult data have shown that immediate completion lymph node dissection (CLND) does not improve overall survival in selected patients with clinically node negative, sentinel lymph node-positive disease. Current nodal management in children is unknown., Methods: The National Cancer Database (NCDB) was queried for patients with melanoma from 2012-2017 and patients categorized as pediatric (≤18 years, n=962) or adult (n=327,987). Factors associated with CLND in children with positive SLNB were evaluated in multivariable analysis. Kaplan-Meier survival analysis was performed., Results: Compared to adults, children present with thicker primary tumors (T3 or T4 26.5% vs 15.5%, p<0.001), resulting in higher rates of nodal assessment with SLN biopsy or LND (60.2% vs 36.6%, p<0.001) and higher rates of regional nodal disease (35.1% vs 23.4%, p<0.001). Children underwent higher rates of CLND after SLN biopsy (10.4% vs 4.1%) and upfront lymph node dissection (15.2% vs 8.7%). A decreased rate of CLND was noted in 2017 compared to 2012 (odds ratio (OR) 0.16 (p=0.005). CLND was performed more often on multivariable analysis for older pediatric age (>12 years, OR=1.6, p=0.037) and lower extremity primary (OR=0.29, p<0.001). Children with regional nodal disease have improved 3-year overall survival compared to adults (96.5% vs 71.0%, p<0.001)., Conclusions: Children with melanoma have higher rates of nodal disease but better survival than adults. As in adults, there has been a recent increase in close nodal observation rather than CLND for patients with positive SLN. Further study of nodal surveillance for pediatric patients is warranted., Competing Interests: Declaration of Competing Interest The authors have no conflicts of interest to disclose., (Copyright © 2021. Published by Elsevier Inc.)

Background: Hepatocellular carcinoma (HCC) is a rare cancer in children, with various histologic subtypes and a paucity of data to guide clinical management and predict prognosis., Methods: A multi-institutional review of children with hepatocellular neoplasms was performed, including demographic, staging, treatment, and outcomes data. Patients were categorized as having conventional HCC (cHCC) with or without underlying liver disease, fibrolamellar carcinoma (FLC), and hepatoblastoma with HCC features (HB-HCC). Univariate and multivariate analyses identified predictors of mortality and relapse., Results: In total, 262 children were identified; and an institutional histologic review revealed 110 cHCCs (42%; 69 normal background liver, 34 inflammatory/cirrhotic, 7 unknown), 119 FLCs (45%), and 33 HB-HCCs (12%). The authors observed notable differences in presentation and behavior among tumor subtypes, including increased lymph node involvement in FLC and higher stage in cHCC. Factors associated with mortality included cHCC (hazard ratio [HR], 1.63; P = .038), elevated α-fetoprotein (HR, 3.1; P = .014), multifocality (HR, 2.4; P < .001), and PRETEXT (pretreatment extent of disease) stage IV (HR, 5.76; P < .001). Multivariate analysis identified increased mortality in cHCC versus FLC (HR, 2.2; P = .004) and in unresectable tumors (HR, 3.4; P < .001). Disease-free status at any point predicted survival., Conclusions: This multi-institutional, detailed data set allowed a comprehensive analysis of outcomes for children with these rare hepatocellular neoplasms. The current data demonstrated that pediatric HCC subtypes are not equivalent entities because FLC and cHCC have distinct anatomic patterns and outcomes in concert with their known molecular differences. This data set will be further used to elucidate the impact of histology on specific treatment responses, with the goal of designing risk-stratified algorithms for children with HCC., Lay Summary: This is the largest reported granular data set on children with hepatocellular carcinoma. The study evaluates different subtypes of hepatocellular carcinoma and identifies key differences between subtypes. This information is pivotal in improving understanding of these rare cancers and may be used to improve clinical management and subsequent outcome in children with these rare malignancies., (© 2022 American Cancer Society.)

Background: Pulmonary nodules that are deep within lung parenchyma and/or small in size can be challenging to localize for biopsy. This study describes current trends in performance of image-guided localization techniques for pulmonary nodules in pediatric patients., Methods: A retrospective review was performed on patients < 21 years of age undergoing localization of pulmonary nodules at 15 institutions. Localization and resection success, time in interventional radiology (IR), operating room (OR) and total anesthesia time, complications, and technical problems were compared between techniques., Results: 225 patients were included with an average of 1.3 lesions (range 1-5). Median nodule size and depth were 4 mm (range 0-30) and 5.4 mm (0-61), respectively. The most common localization techniques were: wire + methylene blue dye (MBD) (28%), MBD only (25%), wire only (14%), technetium-99 only (11%), coil + MBD (7%) and coil only (5%). Localization technique was associated with institution (p < 0.01); technique and institution were significantly associated with mean IR, OR, and anesthesia time (all p < 0.05). Comparing techniques, there was no difference in successful IR localization (range 92-100%, p = 0.75), successful resection (94-100%, p = 0.98), IR technical problems (p = 0.22), or operative complications (p = 0.16)., Conclusions: Many IR localization techniques for small pulmonary nodules in children can be successful, but there is wide variability in application by institution and in procedure time., Level of Evidence: Retrospective review, Level 3., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background: Testicular germ cell tumors are uncommon tumors that are encountered by pediatric surgeons and urologists and require a knowledge of appropriate contemporary evaluation and surgical and medical management., Method: A review of the recommended diagnostic evaluation and current surgical and medical management of children and adolescents with testicular germ cell tumors based upon recently completed clinical trials was performed and summarized in this article., Results: In this summary of childhood and adolescent testicular germ cell tumors, we review the initial clinical evaluation, surgical and medical management, risk stratification, results from recent prospective cooperative group studies, and clinical outcomes. A summary of recently completed clinical trials by pediatric oncology cooperative groups is provided, and best surgical practices are discussed., Conclusions: Testicular germ cell tumors in children are rare tumors. International collaborations, data-sharing, and enrollment of patients at all stages and risk classifications into active clinical trials will enhance our knowledge of these rare tumors and most importantly improve outcomes of patients with testicular germ cell tumors., Level of Evidence: This is a review article of previously published and referenced level 1 and 2 studies, but also includes expert opinion level 5, represented by the American Pediatric Surgical Association Cancer Committee., (Copyright © 2021. Published by Elsevier Inc.)

Background/purpose: Cancer predisposition syndromes (CPS) are a heterogeneous group of inherited disorders that greatly increase the risk of developing malignancies. CPS are particularly relevant to pediatric surgeons since nearly 10% of cancer diagnoses are due to inherited genetic traits, and CPS often contribute to cancer development during childhood., Materials/methods: The English language literature was searched for manuscripts, practice guidelines, and society statements on "cancer predisposition syndromes in children". Following review of these manuscripts and cross-referencing of their bibliographies, tables were created to summarize findings of the most common CPS associated with surgically treated pediatric solid malignancies., Results: Pediatric surgeons should be aware of CPS as the identification of one of these syndromes can completely change the management of certain tumors, such as WT. The most common CPS associated with pediatric solid malignancies are outlined, with an emphasis on those most often encountered by pediatric surgeons: neuroblastoma, Wilms' tumor, hepatoblastoma, and medullary thyroid cancer. Frequently associated non-tumor manifestations of these CPS are also included as a guide to increase surgeon awareness. Screening and management guidelines are outlined, and published genetic testing and counseling guidelines are included where available., Conclusion: Pediatric surgeons play an important role as surgical oncologists and are often the first point of contact for children with solid tumors. In their role of delivering a diagnosis and developing a follow-up and treatment plan as part of a multidisciplinary team, familiarity with common CPS will ensure evidence-based practices are followed, including important principles such as organ preservation and intensified surveillance plans. This review defines and summarizes the CPS associated with common childhood solid tumors encountered by the pediatric surgeon, as well as common non-cancerous disease stigmata that may help guide diagnosis., Type of Study: Summary paper., Level of Evidence: 5., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background: Most children with cancer utilize a central venous line (CVL) for treatment. Complications often necessitate early replacement, revision, or addition (RRA), but the rate of these procedures is not known. This study sought to determine rates of RRA in pediatric oncology patients, and associated risk factors., Materials and Methods: Data queried from the Pediatric Health Information System including patients ≤18 years old with malignancy and CVL placement. Analysis included: first CVL placement of the calendar year and subsequent procedures for 6 months thereafter., Results: A total of 6553 children met inclusion criteria (55.9% male, median age 6 years, interquartile range: 2 to 12). RRA within 6 months was required in 25.6% of patients, with 1.7% requiring 5 or more lines. Patients with Central Line-Associated Bloodstream Infection (CLABSI) were 2.78 times more likely to require RRA within 6 months of initial CVL placement, but accounted for only 16% of RRA patients. Factors associated with RRA were age below 1 year, CLABSI, hematologic malignancy, malnutrition, clotting disorder, deep vessel thromboembolism, and obesity. Patients with implantable ports as initial CVL (42%) were less likely to need RRA., Conclusion: Twenty-five percent require at least 1 RRA within 6 months, with associated morbidity and costs. Though strongly associated, most revisions were not related to CLABSI episodes., Competing Interests: The authors declare no conflict of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose While central venous port placement is common, there remains variation in placement technique and rates of technical complications. The aim of this study was to assess variability in techniques and identify predictors of complications for children undergoing port placement. Methods We retrospectively reviewed all 331 patients who underwent venous port placement at a single tertiary children's hospital from May 2018 to June 2020. The primary outcome was early revision or replacement (R/R) for complications occurring within 30 days. Secondary outcomes included radiation exposure and rate of intraoperative conversion to a secondary site. Results The median age was 7 years (Interquartile Range 3-13 years) and the most common diagnoses were leukemia (30.2%), solid tumors (27.8%), and brain tumors (16.9%). Initial approach for port placement was ultrasound-guided internal jugular (IJV) in 255 (147 by surgery and 108 by interventional radiology [IR]) and landmark subclavian vein (SCV) in 76 (all by surgery). Early R/R occurred in 5.1%, including 9.0% of patients with leukemia but 1.1% with solid tumors. Individual proceduralist volume ranged from 2 to 98 cases and was inversely correlated with early R/R (r = -0.12, p = 0.30). In univariate analysis, ports placed by IR had an increased rate of early R/R (9.3%, n = 10) compared to those placed by surgery (3.2%, n = 7, p = 0.036) but this was not significant in multivariable regression controlling for diagnosis and age (Hazard Ratio 2.04; p = 0.19). Mean fluoroscopy time was significantly longer for ports placed by IR (59.9 s) compared to those placed by surgery (15.1 s, p < 0.001). Initial SCV access was associated with an increased (14.5 vs 0.4%) rate of conversion to a secondary site. Conclusions Though venous port placement is a largely safe procedure in children, a substantial minority of patients, particularly those with leukemia, require early R/R. Proceduralist volume and training may influence early R/R, fluoroscopy exposure, and anatomic site preferences., (Copyright © 2021. Published by Elsevier Inc.)

Care for pediatric patients with serious or potentially life-limiting illness involves the interplay of multiple medical and surgical teams within the hospital. Pediatric surgeons are capable of performing procedures that can improve the quality of life for children facing serious illness, but which also carry the potential for significant risk and burden. Patients and families are often faced with decisions about invasive surgical procedures and interventions, stressing the need for seamless collaboration between palliative care and surgical providers. Equally important is the need for clear and open-ended communication with patients and families by all medical teams to determine if potential surgical procedures and interventions align with their goals and to ensure that the perceived benefits of interventions outweigh any risks. Over the last two decades, pediatric palliative care has grown into a thriving medical subspecialty Despite the importance of collaborative care, there is lack of literature on the interaction of pediatric surgery and palliative care and the role of pediatric surgeons in providing primary palliative care. This review defines surgical pediatric palliative care, and provides an in-depth discussion of the unique complexities involved in caring for children with serious and potentially life-limiting illness, while highlighting specific challenges through detailed case presentations.

Objective: To describe utilization and long-term outcomes of pneumonectomy in children and adolescents with cancer., Summary Background Data: Pneumonectomy in adults is associated with significant morbidity and mortality. Little is known about the indications and outcomes of pneumonectomy for pediatric tumors., Methods: The Pediatric Surgical Oncology Research Collaborative (PSORC) identified pediatric patients <21 years of age who underwent pneumonectomy from 1990 to 2017 for primary or metastatic tumors at 12 institutions. Clinical information was collected; outcomes included operative complications, long-term function, recurrence, and survival. Univariate log rank, and multivariable Cox analyses determined factors associated with survival., Results: Thirty-eight patients (mean 12 ± 6 yrs) were identified; median (IQR) follow-up was 19 (5-38) months. Twenty-six patients (68%) underwent pneumonectomy for primary tumors and 12 (32%) for metastases. The most frequent histologies were osteosarcoma (n = 6), inflammatory myofibroblastic tumors (IMT; n = 6), and pleuropulmonary blastoma (n = 5). Median postoperative ventilator days were 0 (0-1), intensive care 2 (1-3), and hospital 8 (5-16). Early postoperative complications occurred in 10 patients including 1 death. Of 25 (66%) patients alive at 1 year, 15 reported return to preoperative pulmonary status. All IMT patients survived while all osteosarcoma patients died during follow-up. On multivariable analysis, metastatic indications were associated with nonsurvival (HR = 3.37, P = 0.045)., Conclusion: This is the largest review of children who underwent pneumonectomy for cancer. There is decreased procedure-related morbidity and mortality than reported for adults. Survival is worse with preoperative metastatic disease, especially osteosarcoma., Competing Interests: The authors report no conflicts of interest., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Decisions about fertility preservation can be difficult in general but the recent application of preservation techniques to pediatric patients has ushered in a host of new ethical considerations. Fertility preservation (FP) may be considered for all patients who are at risk for infertility due to their medical diagnosis or treatment, including those undergoing gonadotoxic chemotherapy, those with differences of sex development (DSD) undergoing gonadectomy, 1-3 and transgender patients undergoing gender affirming surgery. The focus of this paper is to review the ethical issues involved in offering FP to pediatric oncology patients and, to a lesser extent, the new ethical issues that apply to patients with DSD. Some of the techniques and approach to counseling will also apply to transgender individuals, although that is beyond the scope of this work. We aim to discuss several barriers to offering FP and to advise how to counsel families in the setting of rapid changes in this field. Families should be educated about:Specific guidance for clinicians regarding some of these points was recently published in an American Academy of Pediatrics Clinical Report, 1 and we will illustrate the use of these guidelines in four case presentations., (Copyright © 2021. Published by Elsevier Inc.)

Objective: To examine the association between perioperative red blood cell (RBC) transfusion and postoperative venous thromboembolism (VTE) in pediatric surgical patients., Methods: Retrospective cohort study using the National Surgical Quality Improvement Project Pediatric, a validated registry of 118 United States children's hospitals. Patients under 19 years of age undergoing a surgical procedure between 2012 and 2017 were included, with the main exposure being RBC transfusion in the perioperative period (48 hours prior to operation to 72 hours after operation). The primary 30-day outcome of interest was a postoperative VTE requiring therapy. Risk-adjusted odds ratios (aOR) were calculated using multiple logistic regression. Subgroup analyses were performed across multiple surgical specialties. Sensitivity analyses were performed after (a) imputation for missing variables and (b) propensity score matching., Results: During the study years, 482 867 pediatric patients (56.7% male; median age, 6 years [interquartile range, 1-12 years]) underwent an operation. Of these, 30 879 (6.4%) received at least one perioperative RBC transfusion. Postoperative VTE requiring therapy occurred in 618 patients (0.13%). After adjustment for multiple risk factors, perioperative RBC transfusion was associated with an increased risk of VTE (aOR 2.4; 95% CI, 1.9-3.0). The increased VTE risk persisted after imputation of missing demographic and clinical data as well as after 1:1 propensity score matching (29 811 matched pairs, aOR 2.2; 95% CI, 1.7-2.8)., Conclusions: Perioperative RBC transfusion is associated with an increased, albeit still very low, risk of postoperative VTE in pediatric patients. Patients receiving blood in the perioperative period may benefit from additional monitoring or VTE prophylaxis., (© 2019 Wiley Periodicals, Inc.)

Background: Hirschsprung-associated enterocolitis (HAEC) is a serious potential complication after primary pull-through surgery for Hirschsprung's disease (HSCR). Administration of anal botulinum toxin (BT) injection may improve obstructive symptoms at the internal anal sphincter, leading to improved fecal passage. The timing of administration and effects on delay or prevention of HAEC are unknown. We hypothesized that BT administration increased the postoperative time to HAEC and aimed to investigate whether anal BT administration after primary pull-through surgery for HSCR is associated with increased time to inpatient HAEC admission development., Methods: We performed a retrospective cohort study examining children with HSCR at US children's hospitals from 2008 to 2018 using the Pediatric Health Information System database with an associated primary pull-through operation performed before 60 d of age. The intervention assessed was the administration of BT concerning the timing of primary pull-through, and two groups were identified: PRO (received BT at or after primary pull-through, before HAEC) and NOT (never received BT, or received BT after HAEC). The primary outcome was time from pull-through to the first HAEC admission. The Cox proportional hazards model was developed to examine the BT administration effect on the primary outcome after controlling for patient-level covariates., Results: We examined a total of 1439 children (67 in the PRO and 1372 in the NOT groups). A total of 308 (21.4%) developed at least one episode of HAEC, including 76 (5.3%) who had two or more episodes. Between 2008 and 2018, the frequency of BT administration has increased from three to 20 hospitals with a frequency of administration between 2.2% and 16.2%. Prophylactic BT (PRO) was not associated with increased time to HAEC event on adjusted analysis., Conclusions: Among children with HSCR undergoing primary pull-through surgery, prophylactic BT administration did not demonstrate increased time to first HAEC event. A better-powered study with prophylactic BT is required to determine the effect on HAEC occurrence and timing., Level of Evidence: Level II (retrospective cohort study)., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Survivors of pediatric cancer are at increased risk for infertility and premature hormonal failure. Surgeons caring for children with cancer have an important role to play in understanding this risk, as well as advocating for and performing appropriate fertility preservation procedures. Fertility preservation options in males and females vary by pubertal status and include nonexperimental (oocyte harvest, ovarian tissue cryopreservation, sperm cryopreservation) and experimental (testicular tissue cryopreservation) options. This review summarizes the basics of risk assessment and fertility preservation options and explores unique considerations in pediatric fertility preservation., Competing Interests: Disclosure The authors have no commercial or financial conflicts of interest to disclose. The authors have no sources of funding to report., (Copyright © 2020 Elsevier Inc. All rights reserved.)