Search

Background and Objectives: Solid pseudopapillary neoplasm (SPN) of the pancreas demonstrates an indolent disease course; however, some patients present with a "malignant" phenotype, including distant metastases resistant to chemotherapy. This analysis identifies molecular drivers of metastatic SPN using the world's largest clinicogenomics database., Methods: The American Association for Cancer Research Project Genomics Evidence Neoplasia Information Exchange was queried for primary and metastatic SPN samples. Sample-level genomic alterations were compared. A pan-pancreatic cancer analysis assessed relevant mutations among all metastatic pancreatic malignancies., Results: Among 28 SPN samples identified (n = 17 primary, n = 11 metastatic), the most commonly mutated gene was CTNNB1, (24/28 samples; 85.7%). Most mutations were missense (21/24; 87.5%) or in-frame deletions (3/24; 12.5%). The most common CTNNB1 mutations in primary SPN were exon 3 S37F/C missense mutations (6/16 profiled patients, 37.5%), contrasting exon 3 D32N/Y/H missense mutations in metastatic samples (6/11 profiled patients, 54.5%). Metastatic SPN had higher rates of CTNNB1 mutations than metastases from pancreatic ductal adenocarcinoma (72.7% vs. 1.1%; q < 0.0001), pancreatic neuroendocrine tumor (72.7% vs. 2.5%; q < 0.0001), and pancreatic acinar cell carcinoma (72.7% vs. 11.5%; q = 0.0254)., Conclusions: Missense mutations along exon 3 of CTNNB1 predominate metastatic SPN, differentiating these patients from those with metastases from analogous pancreatic malignancies., (© 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.)

Background: The role of hepatectomy for metastatic disease in children is controversial. Rationales include potential cure, obtaining a diagnosis, and guiding chemotherapy decisions. This study examines the safety and utility of hepatic metastasectomy for children at a single institution., Methods: After IRB approval (#22-1258), medical records were reviewed from 1995 to 2022 for children undergoing hepatic metastasectomy. En-bloc hepatectomies during primary tumor resection were excluded., Results: Hepatic metastasectomy was performed in 16 patients for a variety of histologies. Median patient age was 12.2 years [IQR 6.9-22.6], and 13/16 patients were female (81 %). Number of hepatic metastases ranged from 1 to 23 and involved between 1 and 8 Couinaud segments. Anatomic resections included 4 hemihepatectomies and 1 sectionectomy. All other resections were nonanatomic. 3/6 resections for germ cell tumor (GCT) revealed only mature teratoma, driving adjuvant therapy decisions. When indicated, median time to adjuvant chemotherapy was 19 days [IQR 11-22]. No patients had Clavien-Dindo Class III or higher perioperative morbidity. Three patients (1 GCT, 1 adrenocortical carcinoma (ACC), and 1 gastric neuroendocrine tumor (GNET) experienced hepatic relapse. The patients with relapsed GCT and GNET are alive with disease at 17 and 135 months, respectively. The patient with ACC died of disease progression and liver failure. One patient with Wilms tumor experienced extrahepatic, retroperitoneal recurrence and died. With a median follow-up of 38 months, 10-year disease-specific and disease-free survival were 77 % and 61 %, respectively., Conclusions: Hepatic metastasectomy can be accomplished safely in children, may guide adjuvant therapy decisions, and is associated with long-term survival in selected patients., Level of Evidence: Level IV., Type of Study: Treatment Study, Case series with no comparison group., Competing Interests: Conflicts of interest The authors have no conflicts of interest to disclose., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: Pancreatic solid pseudopapillary neoplasms (SPN) are generally indolent; however, some patients present with "malignant" SPN. An orthogonal analysis of multiple datasets was performed to investigate the utility of complete surgical resection (CSR) for malignant SPN., Methods: A systematic review was performed for cases of malignant SPN, defined as T4, N1, and/or M1. Malignant SPN was analyzed within the National Cancer Database (NCDB) and compared with T1-3N0M0 SPN. Predictors of malignant SPN were assessed, and treatments were analyzed by using survival analysis., Results: The systematic review yielded 164 cases of malignant SPN. Of 31 children, only one died due to malignant SPN. Among adults, CSR was associated with improved disease-specific survival (DSS) (P = 0.0002). Chemotherapy did not improve malignant SPN DSS, whether resected (P = 0.8485) or not (P = 0.2219). Of 692 adults with SPN within the NCDB, 93 (13.4%) had malignant SPN. Pancreatic head location (odds ratio [OR] 2.174; 95% confidence interval [CI] 1.136-4.166; P = 0.0186) and tumor size (OR 1.154; 95% CI 1.079-1.235; P < 0.0001) associated with the malignant phenotype. Malignant SPN predicted decreased overall survival (OS) compared with T1-3N0M0 disease (P < 0.0001). Resected malignant SPN demonstrated improved OS (P < 0.0001), including resected stage IV malignant SPN (P = 0.0003). Chemotherapy did not improve OS for malignant SPN, whether resected (P = 0.8633) or not (P = 0.5734). Within a multivariable model, resection was associated with decreased hazard of death (hazard ratio 0.090; 95% CI 0.030-0.261; P < 0.0001)., Conclusions: Approximately 13% of patients with SPN present with a malignant phenotype. Pediatric cases may be less aggressive. Resection may improve survival for malignant SPN, which does not appear chemosensitive., (© 2023. Society of Surgical Oncology.)

The appropriate management of pediatric liver malignancies, primarily hepatoblastoma and hepatocellular carcinoma, requires an in depth understanding of contemporary preoperative risk stratification, experience with advanced hepatobiliary surgery, and a good relationship with one's local or regional liver transplant center. While chemotherapy regimens have become more effective, operative indications more well-defined, and overall survival improved, the complexity of liver surgery in small children provides ample opportunity for protocol violation, inadequate resection, and iatrogenic morbidity. These guidelines represent the distillation of contemporary literature and expert opinion as a means to provide a framework for preoperative planning and intraoperative decision-making for the pediatric surgeon., (Copyright © 2023. Published by Elsevier Inc.)

Background: Survival for children with metastatic hepatoblastoma (HB) remains suboptimal. We report the response rate and outcome of two courses of vincristine/irinotecan/temsirolimus (VIT) in children with high-risk (HR)/metastatic HB., Procedures: Patients with newly diagnosed HB received HR window chemotherapy if they had metastatic disease or a serum alpha-fetoprotein (AFP) level less than 100 ng/mL. Patients received vincristine (days 1 and 8), irinotecan (days 1-5), and temsirolimus (days 1 and 8). Cycles were repeated every 21 days. Responders had either a 30% decrease using RECIST (Response Evaluation Criteria in Solid Tumors) criteria OR a 90% (>1 log 10 decline) AFP decline after two cycles. Responders received two additional cycles of VIT intermixed with six cycles of cisplatin/doxorubicin/5-fluorouracil/vincristine (C5VD). Nonresponders received six cycles of C5VD alone., Results: Thirty-six eligible patients enrolled on study. The median age at enrollment was 27 months (range: 7-170). Seventeen of 36 patients were responders (RECIST and AFP = 3, RECIST only = 4, AFP only = 10). The median AFP at diagnosis was 222,648 ng/mL and the median AFP following two VIT cycles was 19,262 ng/mL. Three-year event-free survival was 47% (95% confidence interval [CI]: 30%-62%), while overall survival was 67% (95% CI: 49%-80%)., Conclusion: VIT did not achieve the study efficacy endpoint. Temsirolimus does not improve the response rate seen in patients treated with vincristine and irinotecan (VI) alone as part of the initial treatment regimen explored in this study. Additionally, AFP response may be a more sensitive predictor of disease response than RECIST in HB., (© 2023 Wiley Periodicals LLC.)

Background: The utility of repeated surgical interventions in hepatoblastoma to achieve no evidence of disease (NED) is not well-defined. We examined the effect of aggressive pursuit of NED status on event-free (EFS) and overall survival (OS) in hepatoblastoma with subgroup analysis of high-risk patients., Methods: Hospital records were queried for patients with hepatoblastoma from 2005 to 2021. Primary outcomes were OS and EFS stratified by risk and NED status. Group comparisons were performed using univariate analysis and simple logistic regression. Survival differences were compared with log-rank tests., Results: Fifty consecutive patients with hepatoblastoma were treated. Forty-one (82%) were rendered NED. NED was inversely correlated with 5-year mortality (OR 0.006; CI 0.001-0.056; P < .01). Ten-year OS (P < .01) and EFS (P < .01) were improved by achieving NED. Ten-year OS was similar between 24 high-risk and 26 not high-risk patients when NED was attained (P = .83). Fourteen high-risk patients underwent a median of 2.5 pulmonary metastasectomies, 7 for unilateral disease, and 7 for bilateral, with a median of 4.5 nodules resected. Five high-risk patients relapsed, and three were salvaged., Conclusions: NED status is necessary for survival in hepatoblastoma. Repeated pulmonary metastasectomy and/or complex local control strategies to obtain NED can achieve long-term survival in high-risk patients., Level of Evidence: Level III - Treatment Study - Retrospective Comparative Study., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: Hepatoblastoma (HB) requires surgical resection for cure, but only 20-30% of patients have resectable disease at diagnosis. Patients who undergo partial hepatectomy at diagnosis have historically received 4-6 cycles of adjuvant chemotherapy; however, those with 100% well-differentiated fetal histology (WDF) have been observed to have excellent outcomes when treated with surgery alone., Patients and Methods: Patients on the Children's Oncology Group non randomized, multicenter phase III study, AHEP0731, were stratified based on Evan's stage, tumor histology, and serum alpha-fetoprotein level at diagnosis. Patients were eligible for the very low risk stratum of surgery and observation if they had a complete resection at diagnosis and rapid central histologic review demonstrated HB with 100% WDF histology., Results: A total of 8 eligible patients were enrolled on study between September 14, 2009 and May 28, 2014. Outcome current to 06/30/2020 was used in this analysis. The median age at enrollment was 22.5 months (range: 8-84 months) and the median AFP at enrollment was 714 ng/ml (range: 18-77,747 ng/mL). With a median follow-up of 6.6 years (range: 3.6-9.8 years), the 5-year event-free (EFS) and overall survival (OS) were both 100%., Conclusion: This report supports that HB with 100% WDF histology completely resected at diagnosis is curable with surgery only. The development of evidence-based surgical guidelines utilizing criteria based on PRETEXT group, vascular involvement (annotation factors), tumor-specific histology and corresponding biology will be crucial for optimizing which patients are candidates for resection at diagnosis followed by observation., Level of Evidence: Prognosis study, Level I evidence., (Copyright © 2022. 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.)

Objective: To understand the temporal relationships of postoperative complications in children and determine if they are related to each other in a predictable manner., Summary of Background Data: Children with multiple postoperative complications have increased suffering and higher risk for mortality. Rigorous analysis of the temporal relations between complications, how complications might cluster, and the implications of such clusters for children have not been published. Herein, we analyze the relationships between postoperative complications in children., Methods: Data source: Surgical operations included in the National Surgical Quality Improvement Program Pediatric Participant Use Data File from 2013 to 2017. The main outcomes measure was presence of 1 or more postoperative complications within 30 days of surgery. Operations followed by multiple complications were analyzed using network analysis to study prevalence, timing, and co-occurrences of clusters of complications., Results: This study cohort consisted of 432,090 operations; 388,738 (89.97%) had no postoperative complications identified, 36,105 (8.35%) operations resulted in 1 postoperative complication and 7247 (1.68%) operations resulted in 2 or more complications. Patients with multiple complications were more likely to be younger, male, African American, with a higher American Society of Anesthesiologists score, and to undergo nonelective operations (P < 0.001). More patients died with 2 complication versus 1 complication vs no complication (5.3% vs 1.5% vs 0.14%, P < 0.001). Network analysis identified 4 Louvain clusters of complications with dense intracluster relationships., Conclusions: Children with multiple postoperative complications are at higher risk of death, than patients with no complication, or a single complication. Multiple complications are grouped into defined clusters and are not independent., Competing Interests: None of the authors have financial, consultant, institutional, or other relationships that might lead to bias or a conflict of interest related to this work., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Background: The Children's Oncology Group (COG) adopted cisplatin, 5-flourouracil, and vincristine (C5V) as standard therapy after the INT-0098 legacy study showed statistically equivalent survival but less toxicity in comparison with cisplatin and doxorubicin. Subsequent experience demonstrated doxorubicin to be effective in patients with recurrent disease after C5V, and this suggested that it could be incorporated to intensify therapy for patients with advanced disease., Methods: In this nonrandomized, phase 3 COG trial, the primary aim was to explore the feasibility and toxicity of a novel therapeutic cisplatin, 5-flourouracil, vincristine, and doxorubicin (C5VD) regimen with the addition of doxorubicin to C5V for patients considered to be at intermediate risk. Patients were eligible if they had unresectable, nonmetastatic disease. Patients with a complete resection at diagnosis and local pathologic evidence of small cell undifferentiated histology were also eligible for an assessment of feasibility., Results: One hundred two evaluable patients enrolled between September 14, 2009, and March 12, 2012. Delivery of C5VD was feasible and tolerable: the mean percentages of the target doses delivered were 96% (95% CI, 94%-97%) for cisplatin, 96% (95% CI, 94%-97%) for 5-fluorouracil, 95% (95% CI, 93%-97%) for doxorubicin, and 90% (95% CI, 87%-93%) for vincristine. Toxicity was within expectations, with death as a first event in 1 patient. The most common adverse events were febrile neutropenia (n = 55 [54%]), infection (n = 48 [47%]), mucositis (n = 31 [30%]), hypokalemia (n = 39 [38%]), and elevated aspartate aminotransferase (n = 28 [27%]). The 5-year event-free and overall survival rates for the 93 patients who did not have complete resection at diagnosis were 88% (95% CI, 79%-93%) and 95% (95% CI, 87%-98%), respectively., Conclusions: The addition of doxorubicin to the previous standard regimen of C5V is feasible, tolerable, and efficacious, and this suggests that C5VD is a good regimen for future clinical trials., (© 2021 American Cancer Society.)

Purpose: Small cell undifferentiated (SCU) histology in hepatoblastoma (HB) tumors has historically been associated with a poor prognosis. Tumors from patients enrolled on Children's Oncology Group (COG) study AHEP0731 underwent institutional and central pathologic review for identification of SCU histology., Patients and Methods: Patients with SCU histology identified at the local treating institution who had otherwise low-risk tumors were upstaged to the intermediate-risk treatment stratum, whereas those only identified by retrospective central review were treated per the local institution as low-risk. Patients with otherwise intermediate- or high-risk tumors remained in that treatment stratum, respectively. Central review was to be performed for all tissue samples obtained at any time point. Treatment was per local review, whereas analysis of outcome was based on central review., Results: Thirty-five patients had some elements (1%-25%) of SCU identified on central review of diagnostic specimens. All but two patient tissue sample retained nuclear INI1 expression. The presence of SCU histology did not correlate with age, alpha-fetoprotein level at diagnosis, or sex. The presence of SCU did not affect event-free survival (EFS). EFS at 5 years for patients with low-risk, intermediate-risk, and high-risk with SCU HB was 86% (95% CI, 33 to 98), 81% (95% CI, 57 to 92), and 29% (95% CI, 4 to 61), respectively, compared with EFS at 5 years for patients without SCU enrolled with low-risk, intermediate-risk, and high-risk of 87% (95% CI, 72 to 95), 88% (95% CI, 79 to 94), and 55% (95% CI, 32 to 74; P = .17), respectively., Conclusion: The presence of SCU histology in HB does not appear to adversely affect outcome. Future studies should be able to treat patients with SCU HB according to risk stratification without regard to the presence of SCU histology., Competing Interests: Howard M. KatzensteinEmployment: EMD Serono Mark D. KrailoConsulting or Advisory Role: Merck Sharp & DohmeTravel, Accommodations, Expenses: Merck Sharp & Dohme Alexander J. TowbinConsulting or Advisory Role: Applied RadiologyResearch Funding: GuerbetPatents, Royalties, Other Intellectual Property: ElsevierOpen Payments Link: https://openpaymentsdata.cms.gov/physician/148955 Allison F. O'NeillConsulting or Advisory Role: CorMedixNo other potential conflicts of interest were reported.

Objective: To measure the survival among comparable neonates with CDH supported with and without ECLS., Summary of Background Data: Despite widespread use in the management of newborns with CDH, ECLS has not been consistently associated with improved survival., Methods: A retrospective cohort study was performed using ECLS-eligible CDH Study Group registry patients born between 2007 and 2019. The primary outcome was in-hospital mortality. Neonates who did and did not receive ECLS were matched based on variables affecting risk for the primary outcome. Iterative propensity score-matched, survival (Cox regression and Kaplan-Meier), and center effects analyses were performed to examine the association of ECLS use and mortality., Results: Of 5855 ECLS-eligible CDH patients, 1701 (29.1%) received ECLS. "High-risk" patients were best defined as those with a lowest achievable first-day arterial partial pressure of CO2 of ≥60 mm Hg. After propensity score matching, mortality was higher with ECLS (47.8% vs 21.8%, odds ratio 3.3, 95% confidence interval 2.7-4.0, hazard ratio 2.3, P < 0.0001). For the subgroup of high-risk patients, there was lower mortality observed with ECLS (64.2% vs 84.4%, odds ratio 0.33, 95% confidence interval 0.17-0.65, hazard ratio 0.33, P = 0.001). This survival advantage was persistent using multiple matching approaches. However, this ECLS survival advantage was found to occur primarily at high CDH volume centers that offer frequent ECLS for the high-risk subgroup., Conclusions: Use of ECLS is associated with excess mortality for low- and intermediate-risk neonates with CDH. It is associated with a significant survival advantage among high-risk infants, and this advantage is strongly influenced by center CDH volume and ECLS experience., Competing Interests: The authors report no conflicts of interest., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

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.)

This is a commentary on the manuscript titled "Supporting Recovery after Adverse Events: An Essential Component of Surgeon Well-Being" by Berman L, Rialon K, Mueller C, et al., (Copyright © 2021. Published by Elsevier Inc.)

This is a commentary on the manuscript by Mpody C, Arends J, Aldrink J, et al., titled "Prognostic Profiling of Children with Serious Post-Operative Complications: A Novel Probability Model for Failure to Rescue"., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: To better characterize short-term and long-term outcomes in children with pancreatic tumors treated with pancreaticoduodenectomy (PD)., Methods: Patients 21 years of age or younger who underwent PD at Pediatric Surgical Oncology Collaborative (PSORC) hospitals between 1990 and 2017 were identified. Demographic, clinical information, and outcomes (operative complications, long-term pancreatic function, recurrence, and survival) were collected., Results: Sixty-five patients from 18 institutions with a median age of 13 years (4 months-22 years) and a median (IQR) follow-up of 2.8 (4.3) years were analyzed. Solid pseudopapillary tumor of the pancreas (SPN) was the most common histology. Postoperative complications included pancreatic leak in 14% (n = 9), delayed gastric emptying in 9% (n = 6), marginal ulcer in one patient, and perioperative (30-day) death due to hepatic failure in one patient. Pancreatic insufficiency was observed in 32% (n = 21) of patients, with 23%, 3%, and 6% with exocrine, or endocrine insufficiencies, or both, respectively. Children with SPN and benign neoplasms all survived. Overall, there were 14 (22%) recurrences and 11 deaths (17%). Univariate analysis revealed non-SPN malignant tumor diagnosis, preoperative vascular involvement, intraoperative transfusion requirement, pathologic vascular invasion, positive margins, and need for neoadjuvant chemotherapy as risk factors for recurrence and poor survival. Multivariate analysis only revealed pathologic vascular invasion as a risk factor for recurrence and poor survival., Conclusion: This is the largest series of pediatric PD patients. PD is curative for SPN and benign neoplasms. Pancreatic insufficiency is the most common postoperative complication. Outcome is primarily associated with histology., (© 2020 Wiley Periodicals, Inc.)

Background: The histopathological assessment of pediatric liver tumors at presentation is critical to establish a diagnosis, guide treatment, and collect appropriate research samples. The purpose of this study was to evaluate complications associated with different approaches to liver biopsy for newly diagnosed hepatoblastoma., Methods: Children with hepatoblastoma were enrolled on Children's Oncology Group study AHEP0731 (September 2009-March 2012). This analysis evaluated the study cohort of initially unresectable patients who therefore underwent a biopsy procedure at diagnosis. The primary endpoint was clinically significant postbiopsy hemorrhage, defined as requiring red blood cell transfusion., Results: We identified 121 children who underwent open (n = 76, 63%), laparoscopic (n = 17, 14%), or percutaneous (n = 28, 23%) liver biopsies. All biopsy procedures yielded adequate tissue for diagnosis. Postbiopsy hemorrhage requiring transfusion occurred after 26% (n = 31) of biopsies. Need for blood product transfusion most frequently occurred following open (n = 27/76, 36%) and laparoscopic (n = 4/17, 24%) biopsies, compared with percutaneous (n = 0/28, 0%) biopsies (p < 0.01)., Conclusions: Pretreatment biopsy of pediatric liver tumors via a percutaneous approach yielded the lowest frequency of clinically significant hemorrhage requiring transfusion, without evidence of sacrificing diagnostic accuracy., Level of Evidence: Level I., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Hepatoblastoma treatment with curative intent requires surgical resection, but only about a third of newly diagnosed patients with hepatoblastoma have resectable disease at diagnosis. Patients who have upfront resection typically receive a total of 4-6 cycles of adjuvant chemotherapy post-surgery, with the combination of cisplatin, fluorouracil, and vincristine. We aimed to investigate whether event-free survival in children with hepatoblastoma who had complete resection at diagnosis could be maintained with two cycles of adjuvant chemotherapy., Methods: In this Children's Oncology Group, multicentre, phase 3 trial, patients were enrolled in four risk groups on the basis of Evans surgical stage, tumour histology, and levels of α-fetoprotein at diagnosis to receive risk-adapted therapy. Here, we report on the low-risk stratum of the trial. Eligible patients were younger than 21 years and had histologically confirmed, stage I or II hepatoblastoma without 100% pure fetal stage I or small-cell undifferentiated histology; elevated serum α-fetoprotein level (>100 ng/mL); a complete resection at diagnosis; at least 50% Karnofsky (patients >16 years) or Lansky (patients ≤16 years) performance status; and had received no previous chemotherapy or other hepatoblastoma-directed therapy. Patients received two 21-day cycles of cisplatin, fluorouracil, and vincristine within 42 days of resection, consisting of cisplatin (100 mg/m 2 per dose or 3·3 mg/kg per dose for children <10 kg) intravenously over 6 h on day 1; fluorouracil (600 mg/m 2 per dose or 20 mg/kg per dose for children <10 kg) intravenous push on day 2; and vincristine (1·5 mg/m 2 per day to a maximum dose of 2 mg, or 0·05 mg/kg per day for children <10 kg) intravenous push on days 2, 9, and 16. The primary outcome was investigator-assessed event-free survival. As prespecified by protocol, we analysed the primary endpoint 6 years after enrolment (cutoff date June 30, 2017). This trial is registered with ClinicalTrials.gov, number NCT00980460, and is now permanently closed to accrual., Findings: Between May 18, 2010, and May 28, 2014, 51 patients in 32 centres in two countries were enrolled into the low-risk stratum of this trial, of whom 49 received c hemotherapy treatment after surgery and were evaluable for activity and safety. Median follow-up time for all evaluable patients was 42 months (IQR 36-62). 4-year event-free survival was 92% (95% CI 79-97) and 5-year event-free survival was 88% (72-95). Two (4%) of 49 patients had surgical complications (bile leaks). The most common grade 3-4 adverse events were febrile neutropenia in seven (14%) patients, decreased neutrophil count in three (6%) patients, infections in four (8%) patients, and diarrhoea in four (8%) patients. Ototoxicity occurred in one (2%) patient. One (2%) patient of the three who relapsed in this cohort died from disease. Two (4%) patients died in clinical remission after therapy discontinuation. One patient died of pneumonia and bacterial sepsis 1 year after therapy discontinuation and another patient died of unrelated causes 57 months after therapy completion. There were no treatment-related deaths., Interpretation: Minimal postoperative chemotherapy with two cycles of cisplatin, fluorouracil, and vincristine can ensure disease control in patients with hepatoblastoma resected at diagnosis. Our results show that dose reduction of ototoxic agents is a safe, effective treatment for these children., Funding: National Institutes of Health., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Background: Management of CDH is highly variable from center to center, as are patient outcomes. The purpose of this study was to examine risk-stratified survival and extracorporeal membrane oxygenation (ECMO) rates at a single center, and to determine whether adverse outcomes are related to patient characteristics or management., Methods: A retrospective single-center review of CDH patients was performed, and outcomes compared to those reported by the CDH Study Group (CDHSG) registry. Patient demographics, disparities, and clinical characteristics were examined to identify unique features of the cohort. A model derived using the registry that estimates probability of ECMO use or death in CDH newborns was used to risk-stratify patients and assess mortality rates. Observed over expected (O/E) ECMO use rates were calculated to measure whether "excess" or "appropriate" ECMO use was occurring., Results: There were 81 CDH patients treated between 2004-2017, and 5034 in the CDHSG registry. Mortality in ECMO-treated patients was higher than the registry. Socioeconomic variables were not significantly associated with outcomes. The strongest predictors of mortality were ECMO use and early blood gas variables. The risk model accurately predicted ECMO use with a c-statistic of 0.79. Compared with the registry, the disparity in mortality rates was greatest for moderate-risk patients. O/E ECMO use was highest in low and moderate-risk patients., Conclusions: ECMO use is a more consistent predictor of mortality than CDH severity at a single center, and there is relative overuse of ECMO in lower-risk patients. Risk stratification allows for more accurate institutional assessment of mortality and ECMO use, and other centers could consider such an adjusted analysis to identify opportunities for outcomes improvement., Level of Evidence: III., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Neuroblastoma is an embryonic cancer arising from neural crest stem cells. This cancer is the most common malignancy in infants and the most common extracranial solid tumor in children. The clinical course may be highly variable with the possibility of spontaneous regression in the youngest patients and increased risk of aggressive disease in older children. Clinical heterogeneity is a consequence of the diverse biologic characteristics that determine patient risk and survival. This review will focus on current progress in neuroblastoma staging, risk stratification, and treatment strategies based on advancing knowledge in tumor biology and genetic characterization. TYPE OF STUDY: Review article. LEVEL OF EVIDENCE: Level II., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: A means for early postnatal stratification of ECMO risk in CDH newborns could be used to comparatively assess the utilization and outcomes of ECMO use between centers. While multiple CDH mortality risk calculators are available, no validated tool exists specifically for prediction of ECMO use. The purpose of this study was to derive and validate an ECMO risk stratification model., Methods: The study population was obtained from CDH Study Group registry for the period between 2007 and 2016. Only centers offering ECMO were included. The cohort was restricted to ECMO candidates and then divided into derivation and validation sets. Using all relevant perinatal predictors in the registry, univariate analysis was performed for the composite outcome of ECMO use or death without ECMO use. The model was derived using the derivation cohort with multivariable logistic regression and automatic stepwise forward selection (P < 0.05 for qualifying variables), and a c-statistic was obtained. The model was then tested on the validation cohort. Sample reuse validation and bootstrap validation were performed. The validated model was then tested for accuracy on CDH subgroups., Results: There were 1992 patients in the derivation cohort. Four significant variables were identified in the final ECMO risk model: 1-min and 5-min Apgar scores and highest and lowest post-ductal partial pressure of CO2 during the first 24 h of life. The model c-statistic was 0.824 which was confirmed with cross-validation and bootstrap optimism correction. The validation cohort c-statistic was 0.823 (N = 993). The model had good discrimination for left and right CDH, inborn and outborn patients, patients born before and after 2011, and high and low volume centers. The model performed significantly better for postnatally diagnosed patients., Conclusions: This study represents proof-of-concept that a risk model can accurately estimate the probability of ECMO use in CDH newborns. This stratification could assist centers as a metric for assessment of ECMO usage and outcomes. Refinement and prospective validation of this model should be carried out prior to clinical application., Level of Evidence: 3., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Neoadjuvant chemotherapy is given to children with unresectable hepatoblastoma to increase the rate and safety of curative complete surgical resection. Elevated levels of serum alpha-fetoprotein (sAFP) decline with tumor shrinkage. In this single-institution retrospective study, we determined early dynamic changes of sAFP levels and tumor volume in children during therapy for unresectable hepatoblastoma., Methods: We correlated early dynamic changes of sAFP levels and tumor volume and the sum of the longest primary tumor and measurable metastatic disease diameters as per RECIST 1.1 criteria with patient outcome., Results: There were 34 patients, 7 of whom died of disease. Patients with ≥ 90% (≥ 1 log 10 ) decrease in sAFP levels after two chemotherapy courses had a better event-free survival (P = 0.039) and overall survival (OS; P = 0.045) than those with < 90% decrease. During this treatment interval, average tumor volume decreased from 481 mL (± 254 mL) to 268 mL (± 258 mL; P < 0.001) which was associated with OS (P = 0.029). Relative change in sAFP levels or tumor volume in between course 2 and pre-surgery or response as per RECIST 1.1 was not associated with OS., Conclusion: Early decline of sAFP levels and tumor volume, but not response as per RECIST 1.1 may predict survival in children with unresectable hepatoblastoma. This finding could be useful to identify therapy non-responders for whom alternative interventions may be required for cure. Confirmation of the finding using larger patient cohorts will be necessary before this strategy is incorporated into prospective trials.

Health care consumers are burdened with rising out-of-pocket medical expenses. Surgical specialists' experience and attitude towards patients' out-of-pocket costs and the influence of these factors on healthcare utilization are unknown. Our aim was to define the pediatric surgeons' experience with the financial concerns of their patients. Members from the American Academy of Pediatrics Sections on Plastic Surgery, Surgery and Urology were surveyed. Analysis of variance was used to investigate practice differences. Two hundred and eighteen out of 973 surgeons representing 38 states completed the survey. Nearly half of the surveyed surgeons did not know if cost was a determinant for their patients' choice in surgical facility, or if parents compared costs prior to the visit. Eighty four per cent of the surgeons would consider patient costs if medically appropriate, to entertain less costly alternatives, and adjust surgical scheduling to decrease economic burden. Most pediatric surgical specialists are unaware if out-of-pocket costs influenced patients' preoperative decisions. Nonetheless, they are sympathetic to the issue. As the financial burden of health care shifts to consumers, our survey indicates that surgeons are open to candid discussion surrounding finances and may alter recommendations accordingly if appropriate.

The operating room (OR) is a special place wherein groups of highly skilled individuals must work in a coordinated and harmonious fashion to deliver optimal patient care. Team dynamics and human factors principles were initially studied by the aviation industry to better understand and prevent airline accidents. As a result, crew resource management (CRM) training was designed for all flight personnel to create a highly reliable industry with a commitment to a culture of safety. CRM has since been adapted to health care, resulting in care improvement and harm reduction across a wide variety of medical specialties. When implemented in the OR, CRM has been shown not only to improve communication and morale for OR staff, but also reduce morbidity and mortality for patients. As increasing focus is placed on quality, safety, and high-reliability, surgeons will be expected to participate and lead efforts to facilitate a team approach in this new era of patient care., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background and Objectives: African American children are more than twice as likely to die after surgery compared with white children. In this study, we evaluated whether risk factors for death after surgery differ for African American and white children, and we also assessed whether race-specific risk stratification models perform better than non-race-specific models., Methods: The National Surgical Quality Improvement Program Pediatric Participant Use Data File contains clinical data on operations performed on children at participating institutions in the United States. Variables predictive of death within 30 days of surgery were analyzed for differences in prevalence and strength of association with death for both African American and white children. Classification tree and network analysis were used., Results: Network analyses revealed that the prevalence of preoperative risk factors associated with death after surgery was significantly higher for African American than for white children. In addition, many of the risk factors associated with death after surgery carried a higher risk when they occurred in African American children. Race-specific risk models provided high accuracy, with a specificity of 94% and a sensitivity of 83% for African American children and a specificity of 96% and a sensitivity of 77% for white children, and yet these 2 models were significantly different from each other., Conclusions: Race-specific models predict outcomes after surgery more accurately compared with non-race-specific models. Identification of race-specific modifiable risk factors may help reduce racial disparities in surgery outcome., Competing Interests: POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2018 by the American Academy of Pediatrics.)

A simple, objective and accurate way of grouping children undergoing surgery into clinically relevant risk groups is needed. The purpose of this study, is to develop and validate a preoperative risk classification system for postsurgical 30-day mortality for children undergoing a wide variety of operations. The National Surgical Quality Improvement Project-Pediatric participant use file data for calendar years 2012-2014 was analyzed to determine preoperative variables most associated with death within 30 days of operation (D30). Risk groups were created using classification tree analysis based on these preoperative variables. The resulting risk groups were validated using 2015 data, and applied to neonates and higher risk CPT codes to determine validity in high-risk subpopulations. A five-level risk classification was found to be most accurate. The preoperative need for ventilation, oxygen support, inotropic support, sepsis, the need for emergent surgery and a do not resuscitate order defined non-overlapping groups with observed rates of D30 that vary from 0.075% (Very Low Risk) to 38.6% (Very High Risk). When CPT codes where death was never observed are eliminated or when the system is applied to neonates, the groupings remained predictive of death in an ordinal manner.

African American children are more than twice as likely as white American children to die after surgery, and have increased risk for longer hospital stays, post-surgical complications, and higher hospital costs. Prior research into disparities in pediatric surgery outcomes has not considered interactions between patient-level Clinical Risk Factors (CRFs) and population-level Social, Economic, and Environmental Factors (SEEFs) primarily due to the lack of integrated data sets. In this study, we analyze correlations between SEEFs and CRFs and correlations between CRFs and surgery outcomes. We used a dataset from a cohort of 460 surgical cases who underwent surgery at a children's hospital in Memphis, Tennessee in the United States. The analysis was conducted on 23 CRFs, 9 surgery outcomes, and 10 SEEFs and demographic variables. Our results show that population-level SEEFs are significantly associated with both patient-level CRFs and surgery outcomes. These findings may be important in the improved understanding of health disparities in pediatric surgery outcomes.

Purpose To determine whether the pattern of lung nodules in children with metastatic hepatoblastoma (HB) correlates with outcome. Methods Thirty-two patients with metastatic HB were enrolled on Children's Oncology Group Protocol AHEP0731 and treated with vincristine and irinotecan (VI). Responders to VI received two additional cycles of VI intermixed with six cycles of cisplatin/fluorouracil/vincristine/doxorubicin (C5VD), and nonresponders received six cycles of C5VD alone. Patients were imaged after every two cycles and at the conclusion of therapy. All computed tomography scans and pathology reports were centrally reviewed, and information was collected regarding lung nodule number, size, laterality, timing of resolution, and pulmonary surgery. Results Among the 29 evaluable patients, only 31% met Response Evaluation Criteria in Solid Tumors (RECIST) for measurable metastatic disease. The presence of measurable disease by RECIST, the sum of nodule diameters greater than or equal to the cumulative cohort median size, bilateral disease, and ≥ 10 nodules were each associated with an increased risk for an event-free survival event ( P = .48, P = .08, P = .065, P = .03, respectively), with nodule number meeting statistical significance. Ten patients underwent pulmonary resection/metastasectomy at various time points, the benefit of which could not be determined because of small patient numbers. Conclusion Children with metastatic HB have a poor prognosis. Overall tumor burden may be an important prognostic factor for these patients. Lesions that fail to meet RECIST size criteria (ie, those < 10 mm) at diagnosis may contain viable tumor, whereas residual lesions at the end of therapy may constitute eradicated tumor/scar tissue. Patients may benefit from risk stratification on the basis of the burden of lung metastatic disease at diagnosis.

Background: The identification of new therapies for high-risk (HR) hepatoblastoma is challenging. Children's Oncology Group study AHEP0731 included a HR stratum to explore the efficacy of novel agents. Herein, the authors report the response rate to the combination of vincristine (V) and irinotecan (I) and the outcome of patients with high-risk hepatoblastoma., Methods: Patients with newly diagnosed metastatic hepatoblastoma or those with a serum α-fetoprotein (AFP) level <100 ng/mL were eligible. Patients received 2 cycles of V at a dose of 1.5 mg/m 2 /day intravenously on days 1 and 8 and I at a dose of 50 mg/m 2 /day intravenously on days 1 to 5. Patients were defined as responders if they had either a 30% decrease in tumor burden according to Response Evaluation Criteria In Solid Tumors (RECIST) or a 90% (>1 log 10 ) decline in their AFP level. Responders were to receive 2 additional cycles of VI intermixed with 6 cycles of the combination of cisplatin, doxorubicin, 5-fluorouracil, and vincristine (C5VD). Nonresponders were to receive 6 cycles of C5VD alone., Results: A total of 32 patients with a median age at diagnosis of 26 months (range, 11-159 months) were enrolled between September 2009 and February 2012. Fourteen of 30 evaluable patients were responders (RECIST and AFP in 6 patients, RECIST only in 3 patients, and AFP only in 5 patients). The median AFP decline after 2 cycles of VI for the entire group was 345,565 ng/mL (85% of the initial AFP). The 3-year event-free and overall survival rates were 49% (95% confidence interval, 30%-65%) and 62% (95% confidence interval, 42%-77%), respectively., Conclusions: The VI combination appears to have substantial activity against HR hepatoblastoma. The ultimate impact of this regimen in improving the outcomes of children with HR hepatoblastoma remains to be determined. Cancer 2017;123:2360-2367. © 2017 American Cancer Society., (© 2017 American Cancer Society.)

Background: Intussusception is uncommon in children older than 3 years, and use of enema reduction in older children is controversial. We sought to determine whether older children are at greater risk of requiring operative intervention and/or having pathology causing lead points, such that enema reduction should not be attempted., Methods: The Pediatric Health Information System database was reviewed from January 1, 2009-June 30, 2014. Patients were followed for 6 months from initial presentation or until bowel resection occurred. Successful enema reduction was defined as having radiologic reduction without additional procedures., Results: A total of 7,412 patients were identified: 6,681 were <3 years old, 731 patients were >3 years old. In those >3 years old, 450 (62%) were treated successfully with enema reduction; the rate of patients with a tumor diagnosis was similar in patients <3 years old and patients >3 years old (5% vs 6%, P = .07). The rate of a Meckel's diagnosis was greater in patients >3 years old (2% vs 14%, P < .0001). In patients >3 years old, duration of stay between patients who underwent primary operative therapy versus those who underwent operative therapy after enema reduction was similar (4 days vs 4 days, P = .06). Older age was not associated with increased risk of recurrent admission for intussusception (P = .45)., Conclusion: Pediatric Health Information System data suggest that enema reduction may be safe and effective for a majority of children even if older than 3 years., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Informed consent for operative procedures performed on children relies on the ability of the surgeon to estimate and describe accurately the risks and benefits of the planned operation to the parents. Understanding patient-specific risks is also an important prerequisite for surgeons and hospital administrators who wish to change hospital processes and improve patient safety. This study tests the feasibility of estimating the risk of death within 30 days of surgery using National Surgical Quality Improvement Program (NSQIP)-Pediatric data from a single children's hospital., Study Design: Patient data submitted to NSQIP-Pediatric from our hospital were analyzed to identify variables predictive of death within 30 days of operation. A multiple logistic regression model was constructed using 3 years of data and validated using data submitted the following year. The model was then tested using the participant use file provided by NSQIP-Pediatric for 2012., Results: The model identified 7 variables predictive of death: neonatal status, respiratory support, inotropic support, having a blood disorder, cerebrovascular injury, previous cardiac intervention, and the work relative value unit for the procedure. The resulting final model had a c statistic = 0.97., Conclusion: It is possible for a participating children's hospital to use NSQIP-Pediatric data to develop risk models for patient mortality occurring within 30 days of operation at their institution. The model presented may be generalizable to other institutions, but needs further testing and refining., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Purpose: Increasing numbers of programs participating in the pediatric surgery match has resulted in economic and logistical issues for candidates, General Surgery residencies, and Pediatric Surgery training programs (PSTP). We sought to determine the ideal number of interviews conducted by programs based on resultant rank order lists (ROL) of matched candidates., Methods: PSTPs received 4 online surveys regarding interview practices (2011-2012, 2014), and matched candidate ROL (2008-2010, 2012, 2014). Program directors (PD) also provided estimates regarding minimum candidate interview numbers necessary for an effective match (2011-2012, 2014). Kruskal-Wallis equality-of-populations rank tests compared ROL and interview numbers conducted. Quartile regression predicted ROL based on the interview numbers. Wilcoxon signed rank-sum tests compared the interview numbers to the minimal interview number using a matched pair. p Values<0.05 were significant., Results: Survey response rates ranged from 85-100%. Median ROL of matched candidates (2-3.5) did not differ between programs (p=0.09) and the lowest matched ROL for any year was 10-12. Interview numbers did not affect the final candidate ROL (p=0.22). While PDs thought the minimum median interview number should be 20, the number actually conducted was significantly higher (p<0.001)., Conclusion: These data suggest that PSTPs interview excessive numbers of candidates. Programs and applicants should evaluate mechanisms to reduce interviews to limit costs and effort associated with the match., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Background: Our group recently published a clinical pathway (Le Bonheur Clinical Pathway [LeB-P]) that used the Samuel Pediatric Appendicitis Score with selective use of ultrasonography (USG) for diagnosis of children at risk for appendicitis. The objective of this study was to model the cost-effectiveness of implementing the LeB-P compared with usual care., Study Design: We constructed a decision analytic model comparing hospital costs for the following diagnostic strategies for suspected appendicitis: emergency department clinician judgment alone, USG on all patients, CT on all patients, overnight observation with surgical evaluation without studies, and the LeB-P. Prevalence of disease, outcomes probabilities, and hospital and professional costs for each option were derived from published literature, national cost data, and our previous study results. Cost-effectiveness was calculated using these 3 sets of parameters., Results: In the base case model, USG was the preferred strategy over LeB-P and overnight observation with surgical evaluation without studies. Emergency department clinician judgment alone and CT were dominated by the other pathways, based on either lower diagnostic accuracy or increased costs. Compared with LeB-P, USG costs $337 less per patient evaluated, but increased the diagnostic error rate by 2%. Using LeB-P rather than USG would cost an institution an additional $17,206 to eliminate one misdiagnosis, which is known as the incremental cost-effectiveness ratio., Conclusions: Although performing USG on all children with suspected appendicitis was determined to be the most cost-effective strategy, using the Pediatric Appendicitis Score with selective use of USG (LeB-P) improved diagnostic accuracy at a moderate increase in cost and decreased CT use., (Copyright © 2015 American College of Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The long-term physiologic and oncologic outcomes of treatment for inferior vena cava (IVC) thrombosis in children with malignant abdominal tumors are unclear., Methods: We conducted a retrospective review of children with malignant IVC tumor thrombosis treated at our institution between January 1996 and December 2011. Extent of tumor thrombus was classified using the Hinman system. Disease stage, management, and oncologic and physiologic outcomes and complications were evaluated., Results: We identified 15 patients (median age, 4.7 years): 12 with Wilms tumor, 2 with hepatoblastoma, and 1 with adrenocortical carcinoma. Neoadjuvant chemotherapy changed Hinman levels in 2 (13%) patients. IVC thrombus resection was complete in 6 (40%) patients, partial in 7 (47%) patients, and not performed in 1 (6.7%) patient. On follow-up imaging, 8 (53%) patients' IVCs were patent, 6 (40%) had residual thrombus, and 1 (6.7%) was surgically interrupted. Three (20%) patients had perioperative complications, and 2 (13%) experienced transient effects related to IVC occlusion., Conclusions: Surgical management of tumor thrombus in the vena cava of children with solid abdominal tumors is challenging. Evidence on which to base strong treatment recommendations is lacking. Few long-term physiologic complications were observed., (Copyright © 2015 Elsevier Inc. All rights reserved.)

This review is focused on the special issues and challenges confronting physicians and surgeons caring for an unborn child, or a newborn with a liver tumor. Liver tumors at this age are very rare and they make it difficult for pediatric surgeons to gain experience necessary to obtain good results. On the other hand, adult hepatobiliary surgeons faced with a fetus or infant with a liver mass are ill equipped to care for the patient even if they have done a high volume of adult liver surgery and are expert in the field. Often a team approach is the best solution.

Background: We previously conducted a randomized, clinical trial comparing early appendectomy with interval appendectomy for perforated appendicitis. The purpose of the present study was to evaluate the effect this clinical trial had on subsequent practice patterns and outcomes for patients with perforated appendicitis at the free-standing children's hospital conducting the trial., Methods: A retrospective study was conducted comparing children with perforated appendicitis treated before the trial (2005-2006) and after the trial (2009-2011). Early appendectomy was performed within 24 hours of diagnosis; interval appendectomy occurred 4-6 weeks after initial treatment with antibiotics. Patient characteristics, treatment variables, and outcomes were collected and compared., Results: The pretrial group consisted of 92 patients-62 (67%) underwent early appendectomy, and 30 (33%) patients had interval appendectomy. The posttrial group was composed of 103 patients, with 87 (84%) undergoing early appendectomy and 16 (16%) interval appendectomy (P = .005). The groups were similar in patient and admission characteristics, although the posttrial group had a lower percentage of self-pay patients and fewer computed tomography scans; health care use was similar between groups. Overall, the posttrial group had fewer adverse events (18% vs 34%, P = .02), specifically fewer wound infections (2% vs 14%, P = .001) and fewer unplanned readmissions (7% vs 16%, P = .04) than the pretrial group. In the posttrial group, those patients selected for interval appendectomy were more likely to complete the planned course of therapy than in the pretrial group., Conclusion: A clinical trial conducted at our institution to evaluate currently available treatment options for perforated appendicitis did change practice patterns at our hospital. After the trial, there was an increase in the use of early appendectomy, a decrease in the number of computed tomography scans performed per patient, and a reduction in the overall adverse event rate., (Copyright © 2014 Mosby, Inc. All rights reserved.)

Background/purpose: Congenital diaphragmatic hernia (CDH) remains a significant cause of neonatal death. A wide spectrum of disease severity and treatment strategies makes comparisons challenging. The objective of this study was to create a standardized reporting system for CDH., Methods: Data were prospectively collected on all live born infants with CDH from 51 centers in 9 countries. Patients who underwent surgical correction had the diaphragmatic defect size graded (A-D) using a standardized system. Other data known to affect outcome were combined to create a usable staging system. The primary outcome was death or hospital discharge., Results: A total of 1,975 infants were evaluated. A total of 326 infants were not repaired, and all died. Of the remaining 1,649, the defect was scored in 1,638 patients. A small defect (A) had a high survival, while a large defect was much worse. Cardiac defects significantly worsened outcome. We grouped patients into 6 categories based on defect size with an isolated A defect as stage I. A major cardiac anomaly (+) placed the patient in the next higher stage. Applying this, patient survival is 99% for stage I, 96% stage II, 78% stage III, 58% stage IV, 39% stage V, and 0% for non-repair., Conclusions: The size of the diaphragmatic defect and a severe cardiac anomaly are strongly associated with outcome. Standardizing reporting is imperative in determining optimal outcomes and effective therapies for CDH and could serve as a benchmark for prospective trials., (© 2013.)

Objective: This study investigates how the epidemiology, hospital utilization, and surgical management of Hirschsprung disease (HD) have changed over the last decade in the United States, using a statistically valid national sample., Background: HD is a congenital gastrointestinal disorder that requires surgical resection for correction. Some patients experience long-term bowel dysfunction requiring hospital care. Historically, patients had multiple staged operations, whereas more recently, single-stage laparoscopic resection and pull-through operations are more common. Assessment of possible changes over time in HD-associated complications requiring hospitalization and length of hospital stay has not been quantified epidemiologically., Methods: The Kids' Inpatient Database was queried for all discharges with an International Classification of Disease, Ninth Revision, Clinical Modification code for HD in the years 1997, 2000, 2003, and 2006. The HD cohorts from these 4 time points were compared, specifically analyzing differences in demographic data, associated diagnoses, in-hospital mortality and length of stay, procedures performed during hospitalization, and frequency of hospitalizations for HD-associated complications. Results reported included estimated frequencies and means with 95% confidence intervals., Results: The estimated numbers of HD discharges, associated demographic data, and numbers of pull-through procedures have remained stable over the decade. The mean age (years) at the time of pull-through has decreased from 1.45 to 1.16 to 1.18 to 0.97 (P = 0.01). The mean length of stay (days) for these procedures has increased from 8.40 to 8.46 to 9.25 to 10.55 (P = 0.002). The estimated numbers of hospital admissions for HD-related constipation increased in recent years from 395 to 340 to 536 to 566 (P = 0.001). The estimated numbers of admissions for enterocolitis suggest an increasing trend from 466 to 402 to 584 to 556 (P = 0.11)., Conclusions: HD pull-through procedures are being performed at younger ages over time, and post-pull-through lengths of stay have increased. Admissions for some HD-related complications have increased over the decade. Prospective cohort studies are needed to determine whether causal relationships exist among these trends.