Patient Characteristics, Treatment, and Survival in Pleuropulmonary Blastoma: An Analysis from the National Cancer Database.

Pleuropulmonary blastoma (PPB) is a rare childhood tumor originating from the lung or pleura, typically treated with surgery, chemotherapy (CTx), and/or radiation therapy (RTx). This study aimed to assess patient and tumor features, treatment methods, and survival rates in PPB. We retrospectively analyzed PPB patients under 18 from 2004 to 2019, using the National Cancer Database (NCDB). We assessed 5-year overall survival (OS) rates based on disease presentation and treatment regimens, using Kaplan-Meier curves and Cox proportional regression. Among 144 cases identified, 45.9% were female, with a median age of 2 years (interquartile range 1-3). In all, 62.5% of tumors originated from the lung, and 10.4% from the pleura. Moreover, 6.9% were bilateral, and the median tumor size was 8.9 cm, with 4.2% presenting with metastases. The 5-year OS rate was 79.6%, with no significant change over time (2004-2009, 77.6%; 2010-2014, 90.8%; 2015-2019, OS 75.3%; p = 0.08). In all, 5.6% received CTx alone, 31.3% surgery alone, 45.8% surgery/CTx, and 17.4% surgery/CTx/RTx. The OS rates were comparable between the surgery/CTx/RTx (80.0%) and surgery/CTx (76.5%) groups (adjusted Hazard Ratio, 1.72; 95% CI, 0.57-5.19; p = 0.34). Therefore, due to the unchanged survival rates over time, further prospective multicenter studies are needed to determine the optimal multimodal treatment regimens for this rare pediatric tumor.

Operative Volumes and Postoperative Outcomes After Elective Resection of Congenital Lung Malformations in Infants.

OBJECTIVE: To determine whether hospital- and surgeon-level operative volumes are associated with differences in postoperative outcomes among infants undergoing elective lung surgery for a congenital lung malformation (CLM). SUMMARY BACKGROUND DATA: Infant lung surgery is a relatively uncommon procedure performed by pediatric surgeons nationwide. The relationship between surgical volume and postoperative outcomes remains unknown. METHODS: A retrospective cohort study of asymptomatic infants who underwent elective lung resection of a CLM was conducted using the Pediatric Health Information System database (2016-2020). Multivariable linear and poisson regressions were performed based on annual lung resection tertiles. RESULTS: There were 1420 infants managed by 48 hospitals and 309 primary surgeons. Institutions that performed seven or fewer CLM resections per year (56%) were associated with significantly higher postoperative complication rates compared to medium- and high-volume hospitals (low: 134 [34%], medium: 110 [21%], high: 144 [29%]; P<0.001). Surgeons who performed one or fewer CLM resections per year (82%) were associated with significantly higher complication rates compared to medium- and high-volume surgeons (low: 171 [31%], medium: 75 [26%], high: 119 [24%]; P=0.02). Multivariable analyses confirmed that low-volume hospitals were associated with higher complications (OR 1.81, CI 1.38-2.37; P<0.001), and low-volume surgeons had an increased risk of complications (overall: OR 1.37, CI 1.01-1.84; P=0.04). CONCLUSIONS: In this cohort study of infants undergoing elective lung resection for a CLM, lower volume providers were associated with higher postoperative patient morbidity. These findings represent an opportunity to inform quality improvement initiatives on pediatric lung resection and the debate on surgical subspecialization for this unique patient population.

Cryoablation Reduces Opioid Consumption and Length of Stay After Pulmonary Metastasectomy.

INTRODUCTION: Intraoperative cryoablation of intercostal thoracic nerves is gaining popularity as a technique that decreases postoperative pain in thoracic surgery. Our study evaluates the efficacy and safety of cryoablation in pain management of pediatric cancer patients undergoing thoracotomy. METHODS: We reviewed cancer patients undergoing thoracotomies for pulmonary metastasis resection at our children's hospital from 2017 to 2023. Patients who received cryoablation were compared to those who did not. Our primary outcomes were self-reported postoperative pain scores (from 0 to 10) and opioid consumption, measured as oral morphine equivalent per kilogram. RESULTS: Thirty eight procedures were performed in 17 patients, of which 11 (64.7%) were males. Cryoablation was used in 14 (32.4%) procedures, while it was not in 24 (67.6%). Median age (17 y in both groups, P = 0.84) and length of surgery (300 cryoablation versus 282 no cryoablation, P = 0.65) were similar between the groups. Patients treated with cryoablation had a shorter hospital stay compared to those who did not (3.0 versus 4.5 d, respectively, P = 0.04) and received a lower total dose of opioids (2.2 oral morphine equivalent per kilogram versus 14.4, P = 0.004). No significant difference was noted in daily pain scores between the two groups (3.8 cryoablation versus 3.9 no cryoablation, P = 0.93). There was no difference in rates of readmissions between the cryoablation and no-cryoablation groups (14.3% versus 8.3%, P = 0.55). CONCLUSIONS: Our study suggests that cryoablation of the thoracic nerves during a thoracotomy is associated with reduced opiate consumption and shorter hospital stay. Cryoablation appears to be a promising technique for pain management in this patient population.

Ethnic disparities in pediatric appendicitis: the impact of hispanic ethnicity on presentation, complications, and postoperative outcomes.

PURPOSE: Our study investigates ethnic disparities in pediatric appendicitis, focusing on the impact of Hispanic ethnicity on presentation, complications, and postoperative outcomes. METHODS: We conducted a retrospective analysis of pediatric patients undergoing appendectomy for acute appendicitis from 2015 to 2020 using the National Surgical Quality Improvement Program-Pediatric database. We compared 30-day postoperative complications, postoperative length of stay, and postoperative interventions between Hispanic and non-Hispanic White patients. RESULTS: 65,976 patients were included, of which 23,462 (35.56%) were Hispanic and 42,514 (64.44%) non-Hispanic White. Hispanic children were more likely to present to the hospital with complicated appendicitis (31.75% vs. 25.15%, P < 0.0001) and sepsis (25.22% vs. 19.02%, P < 0.0001) compared to non-Hispanic White. Hispanics had higher rates of serious complications (4.06% vs. 3.55%, P = 0.001) but not overall complications (5.37% vs. 5.09%, P = 0.12). However, after multivariate analysis, Hispanic ethnicity was not associated with an increased rate of serious postoperative complications (OR 0.93, CI 0.85-1.01, P = 0.088); it was associated with less overall complications (OR 0.88, CI 0.81-0.96, P = 0.003) but a longer postoperative length of stay (OR 1.09, CI 1.04-1.14, P < 0.0001). CONCLUSION: Hispanic children are more likely to present with complicated appendicitis, contributing to increased postoperative complications. Notably, upon adjustment for the impact of complicated appendicitis, our findings suggest potentially favorable outcomes for Hispanic ethnicity. This emphasizes the need to understand delays in presentation to improve outcomes in the Hispanic population.

Access to Laparoscopic Pediatric Surgery: Do Ethnic and Racial Disparities Exist?

INTRODUCTION: Disparate access to laparoscopic surgery may contribute to poorer health outcomes among racial and ethnic minorities, especially among children. We investigated whether racial and ethnic disparities in laparoscopic procedures existed among four common surgical operations in the pediatric population in the United States. METHODS: Using the American College of Surgeons National Surgical Quality Improvement Program-Pediatrics, we conducted a retrospective review of pediatric patients, aged less than 18 y old, undergoing appendectomy, fundoplication, cholecystectomy, and colectomy from 2012 to 2021. To compare the surgical approach (laparoscopy or open), a propensity score matching algorithm was used to compare laparoscopic and open procedures between non-Hispanic Black with non-Hispanic White children and Hispanic with non-Hispanic White children. RESULTS: 143,205, 9,907, 4,581, and 26,064 children underwent appendectomy, fundoplication, colectomy, and cholecystectomy, respectively. After propensity score matching, non-Hispanic Black children undergoing appendectomy were found to be treated laparoscopically less than non-Hispanic White children (93.5% versus 94.4%, P = 0.007). With fundoplication, Hispanic children were more likely to be treated laparoscopically than White ones (86.7% versus 80.9%, P < 0.0001). There were no statistically significant differences between Black or Hispanic children and White children in rates of laparoscopy for other procedures. CONCLUSIONS: Though some racial and ethnic disparities exist with appendectomies and fundoplications, there is limited evidence to indicate that widespread inequities among common laparoscopic procedures exist in the pediatric population.

Critical elements of pediatric rhabdomyosarcoma surgery.

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.

Critical elements of pediatric neuroblastoma surgery.

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

Prognosis of children and young adults with newly diagnosed rhabdomyosarcoma metastatic to bone marrow treated on Children's Oncology Group studies.

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.

Patterns, procedures, and indications for pediatric surgery in a Tanzanian Refugee Camp: a 20-year experience.

Background: There are 103 million displaced people worldwide, 41% of whom are children. Data on the provision of surgery in humanitarian settings are limited. Even scarcer is literature on pediatric surgery performed in humanitarian settings, particularly protracted humanitarian settings. Methods: We reviewed patterns, procedures, and indications for pediatric surgery among children in Nyarugusu Refugee Camp using a 20-year retrospective dataset. Results: A total of 1221 pediatric surgical procedures were performed over the study period. Teenagers between the ages of 12 and 17 years were the most common age group undergoing surgery (n=991; 81%). A quarter of the procedures were performed on local Tanzanian children seeking care in the camp (n=301; 25%). The most common procedures performed were cesarean sections (n=858; 70%), herniorrhaphies (n=197; 16%), and exploratory laparotomies (n=55; 5%). Refugees were more likely to undergo exploratory laparotomy (n=47; 5%) than Tanzanian children (n=7; 2%; p=0.032). The most common indications for exploratory laparotomy were acute abdomen (n=24; 44%), intestinal obstruction (n=10; 18%), and peritonitis (n=9; 16%). Conclusions: There is a significant volume of basic pediatric general surgery performed in the Nyarugusu Camp. Services are used by both refugees and local Tanzanians. We hope this research will inspire further advocacy and research on pediatric surgical services in humanitarian settings worldwide and illuminate the need for including pediatric refugee surgery within the growing global surgery movement.

Racial and Ethnic Disparities in Outcomes Among Newborns with Congenital Diaphragmatic Hernia.

Importance: There is some data to suggest that racial and ethnic minority infants with congenital diaphragmatic hernia (CDH) have poorer clinical outcomes. Objective: To determine what patient- and institutional-level factors are associated with racial and ethnic differences in CDH mortality. Design, Setting, and Participants: Multicenter cohort study of 49 US children's hospitals using the Pediatric Health Information System database from January 1, 2015, to December 31, 2020. Participants were patients with CDH admitted on day of life 0 who underwent surgical repair. Patient race and ethnicity were guardian-reported vs hospital assigned as Black, Hispanic (White or Black), or White. Data were analyzed from August 2021 to March 2022. Exposures: Patient race and ethnicity: (1) White vs Black and (2) White vs Hispanic; and institutional-level diversity (as defined by the percentage of Black and Hispanic patients with CDH at each hospital): (1) 30% or less, (2) 31% to 40%, and (3) more than 40%. Main Outcomes and Measures: The primary outcomes were in-hospital and 60-day mortality. The study hypothesized that hospitals managing a more racially and ethnically diverse population of patients with CDH would be associated with lower mortality among Black and Hispanic infants. Results: Among 1565 infants, 188 (12%), 306 (20%), and 1071 (68%) were Black, Hispanic, and White, respectively. Compared with White infants, Black infants had significantly lower gestational ages (mean [SD], White: 37.6 [2] weeks vs Black: 36.6 [3] weeks; difference, 1 week; 95% CI for difference, 0.6-1.4; P < .001), lower birthweights (White: 3.0 [1.0] kg vs Black: 2.7 [1.0] kg; difference, 0.3 kg; 95% CI for difference, 0.2-0.4; P < .001), and higher extracorporeal life support use (White: 316 patients [30%] vs Black: 69 patients [37%]; χ21 = 3.9; P = .05). Black infants had higher 60-day (White: 99 patients [9%] vs Black: 29 patients [15%]; χ21 = 6.7; P = .01) and in-hospital (White: 133 patients [12%] vs Black: 40 patients [21%]; χ21 = 10.6; P = .001) mortality . There were no mortality differences in Hispanic patients compared with White patients. On regression analyses, institutional diversity of 31% to 40% in Black patients (hazard ratio [HR], 0.17; 95% CI, 0.04-0.78; P = .02) and diversity greater than 40% in Hispanic patients (HR, 0.37; 95% CI, 0.15-0.89; P = .03) were associated with lower mortality without altering outcomes in White patients. Conclusions and Relevance: In this cohort study of 1565 who underwent surgical repair patients with CDH, Black infants had higher 60-day and in-hospital mortality after adjusting for disease severity. Hospitals treating a more racially and ethnically diverse patient population were associated with lower mortality in Black and Hispanic patients.

Delayed primary repair in 100 infants with isolated long-gap esophageal atresia: A nationwide analysis of children's hospitals.

BACKGROUND: This study aimed to evaluate the contemporary surgical management of long-gap esophageal atresia, a rare and challenging problem managed by pediatric general surgeons. METHODS: A retrospective review of the Pediatric Health Information System database for infants who underwent neonatal gastrostomy, followed by surgical reconstruction for long-gap esophageal atresia (2014-2021). Patients with birthweight less than 1.5 kg and those who received neonatal cardiac surgery were excluded. Outcomes were analyzed, including the need for further procedures, length of stay, and mortality. RESULTS: Of 1,346 infants who underwent repair across 47 major children's hospitals, 100 (7%) met the inclusion criteria for long-gap esophageal atresia. Cardiac anomalies were identified in 43% of patients. The median age at repair was 87 days (interquartile range, 62-133). Ten percent of patients had a planned or unplanned reoperation ≤30 days after index surgery, and 4% underwent reoperation at >30 days. The median time to reoperation was 9 days (interquartile range, 7-60). Mortality during index admission was 5%, and the median hospital length of stay was 143 days (interquartile range, 101-192). Length of stay was significantly longer in patients with cardiac anomalies (cardiac: 179 days, non-cardiac: 125 days; P < .001), and 52% of patients required at least 1 postoperative dilation. The median time to the first dilation was 70 days (interquartile range, 42-173). CONCLUSION: This large multicenter study highlights the challenges of infants with long-gap esophageal atresia but suggests a high rate of successful delayed primary reconstruction. Hospitalizations are prolonged, and anastomotic stricture rates remain high. These data are useful for pediatric surgeons in counseling families on surgical repair strategy, timing, and postoperative outcomes.

Rhabdomyosarcoma with isolated lung metastases: A report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group.

BACKGROUND: To determine outcomes of children with rhabdomyosarcoma (RMS) with isolated lung metastases. METHODS: Data were analyzed for 428 patients with metastatic RMS treated on COG protocols. Categorical variables were compared using Chi-square or Fisher's exact tests. Event-free survival (EFS) and overall survival (OS) were estimated using Kaplan-Meier method and compared using the log-rank test. RESULTS: Compared with patients with other metastatic sites (n = 373), patients with lung-only metastases (n = 55) were more likely to be <10 years of age, have embryonal histology (embryonal rhabdomyosarcoma), have N0 disease, and less likely to have primary extremity tumors. Lung-only patients had significantly better survival outcomes than patients with all other sites of metastatic disease (p < .0001) with 5-year EFS of 48.1 versus 18.8% and 5-year OS of 64.1 versus 26.9%. Patients with lung-only metastases, and those with a single extrapulmonary site of metastasis, had better survival compared with patients with two or more sites of metastatic disease (p < .0001). In patients with ERMS and lung-only metastases, there was no significant difference in survival between patients ≥10 years and 1-9 years (5-year EFS: 58.3 vs. 68.2%, 5-year OS: 66.7 vs. 67.7%). CONCLUSIONS: With aggressive treatment, patients with ERMS and lung-only metastatic disease have superior EFS and OS compared with patients with other sites of metastatic disease, even when older than 10 years of age. Consideration should be given to including patients ≥10 years with ERMS and lung-only metastases in the same group as those <10 years in future risk stratification algorithms.

Morbidity and Mortality in Neonates with Symptomatic Congenital Lung Malformation.

BACKGROUND: Neonatal resection is the mainstay treatment of children presenting with symptomatic congenital lung malformation (CLM) at birth. The objective of this study was to evaluate risk factors for increased morbidity and mortality after neonatal CLM resection using a large multicenter database. STUDY DESIGN: Retrospective review of the Pediatric Health Information System database was performed. Children with a symptomatic CLM managed by lung resection before 30 days of age were included (2016 to 2021). Primary outcomes measures were postoperative respiratory complication and any complication, including death. RESULTS: Of 1,791 CLM patients identified, 256 (14%) underwent neonatal resection for symptomatic disease. Pathology included 123 (48%) congenital pulmonary airway malformation, 24 (10%) bronchopulmonary sequestration, 5 (2%) congenital lobar emphysema, 16 (6%) hybrid, and 88 (34%) unclassified lesion. Preoperative mechanical ventilation and extracorporeal membrane oxygenation (ECMO) were employed in 149 (58.2%) and 17 (6.7%) of cases, respectively. The median age at resection was 6.5 days (interquartile range 2 to 23). Postoperatively, 25 (10%) required mechanical ventilation for 48 hours or more, 3 (1%) continued ECMO, and 3 (1%) required ECMO rescue. The overall respiratory complication rate was 34% (87), rate of any complication was 51% (130), median postoperative length of stay was 20 days (interquartile range 9 to 52), and mortality rate was 14.5% (37). Birthweight was inversely correlated with complication risk (incidence rate ratio 0.55, 95% CI 0.36 to 0.83, p = 0.006). Cardiac structural anomaly was associated with a 21-day longer postoperative length of stay (95% CI 6 to 35, p = 0.006) and 2.2 times increased risk of any complication (95% CI 1.18 to 4.02, p = 0.014). CONCLUSIONS: In this large multicenter study, ECMO use and mortality are relatively uncommon among neonates undergoing lung resection for a symptomatic CLM. However, postoperative morbidity remains high, particularly in those with cardiac structural disease.

Transnational Capacity Building Efforts in Global Neurosurgery: A Review and Analysis of Their Impact and Determinants of Success.

BACKGROUND: Neurosurgical capacity building efforts attempt to address the shortage of neurosurgeons and lack of neurosurgical capacity in low- and middle-income countries. This review sought to characterize neurosurgical capacity building efforts in low- and middle-income countries and the challenges they face, and identify factors that predict higher engagement, better training, and performance of high-impact activities. METHODS: A scoping review using PubMed and Embase databases was performed and relevant articles were identified. Programs were classified into 6 categories and the activities they performed were classified as having a high-, medium-, or mild impact on capacity. Programs were also classified using the Olivieri engagement and training criteria. RESULTS: Fifty-seven articles representing 42 unique efforts were included. The most important determinant of impact was a program's design and intent. Furthermore, 91% of training and twinning programs received high (class 2 or 3) engagement classifications compared to 17% of mission trips and training camps (P < 0.001); 91% of training and twinning programs received high training classifications compared to 64% of mission trips and training camps (P = 0.015); and 91% of training and twinning programs reported performing high-impact activities compared to 29% of mission trips and training camps (P < 0.001). CONCLUSIONS: Training and twinning programs are more engaged, offer better training, and are more likely to perform high-impact activities compared to mission trips and training camps, suggesting that these types of programs offer the greatest chance of producing substantial and sustainable improvements to neurosurgical capacity.

Global Disparities in Burn Outcomes: Does Gender Predict Mortality in the Global Burn Registry?

INTRODUCTION: Global burn injury burden disproportionately impacts low- and middle-income countries. Surgery is a mainstay of burn treatment, yet access to surgical care appears to be inequitably distributed for women. This study sought to identify gender disparities in mortality and access to surgery for burn patients in the World Health Organization Global Burn Registry (GBR). METHODS: We queried the World Health Organization GBR for a retrospective cohort (2016-2021). Patients were stratified by sex. Outcomes of interest were in-hospital mortality and surgical treatment. Patient demographics, injury characteristics, outcomes, and health facility resources were compared between sexes with Wilcoxon rank sum test for nonparametric medians, and chi-squared or Fisher's exact test for nonparametric proportions. Multivariable logistic regressions were performed to assess the relationships between sex and mortality, and sex and surgery. RESULTS: Of 8445 patients in the GBR from 20 countries (10 low resource), 40% of patients were female, with 51% of all patients receiving surgical treatment during their hospitalization. Female patients had a higher incidence of mortality (24% versus 15%, P < 0.001) and a higher median total body surface area (20% versus 15%, P < 0.001), yet a lower incidence of surgery (47% versus 53%, P < 0.001) following burn injury when compared to males. In multivariable analysis, female sex was independently associated with mortality after controlling for age, time to presentation, smoke injury, percent total body surface area, surgery, and country income status. Female sex was independently associated with surgical care (odds ratio 0.86, P = 0.001). CONCLUSIONS: Female burn patients suffer higher mortality compared to males and are less likely to receive surgery. Further study into this gender disparity in burns is warranted.

Inflammatory myofibroblastic tumor: A multi-institutional study from the Pediatric Surgical Oncology Research Collaborative.

Inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm of intermediate malignancy. We describe the largest cohort of IMT patients to date, aiming to further characterize this rare, poorly understood tumor. This is a multi-institutional review of IMT patients ≤39 years, from 2000 to 2018, at 18 hospitals in the Pediatric Surgical Oncology Research Collaborative. One hundred and eighty-two patients were identified with median age of 11 years. Thirty-three percent of tumors were thoracic in origin. Presenting signs/symptoms included pain (29%), respiratory symptoms (25%) and constitutional symptoms (20%). Median tumor size was 3.9 cm. Anaplastic lymphoma kinase (ALK) overexpression was identified in 53% of patients. Seven percent of patients had distant disease at diagnosis. Ninety-one percent of patients underwent resection: 14% received neoadjuvant treatment and 22% adjuvant treatment. Twelve percent of patients received an ALK inhibitor. Sixty-six percent of surgical patients had complete resection, with 20% positive microscopic margins and 14% gross residual disease. Approximately 40% had en bloc resection of involved organs. Median follow-up time was 36 months. Overall 5-year survival was 95% and 5-year event-free survival was 80%. Predictors of recurrence included respiratory symptoms, tumor size and distant disease. Gross or microscopic margins were not associated with recurrence, suggesting that aggressive attempts at resection may not be warranted.

Trends of lymph node sampling and metastasis in pediatric and young adult patients with clear cell, epithelioid, and synovial sarcomas.

BACKGROUND: Clear cell sarcoma of soft tissue (CCS), epithelioid sarcoma, and synovial sarcoma are rare tumors historically identified as high risk for lymph node metastasis. This study investigates incident nodal metastasis and associated survival in children and young adults with these subtypes. PROCEDURE: Using the National Cancer Database (2004-2015), we created a retrospective cohort of 1303 patients (aged ≤25 years) who underwent local control therapy for CCS, epithelioid sarcoma, and synovial sarcoma. Kaplan-Meier curves estimated overall survival (OS) by subtype. Stratifying on subtype, Cox regressions assessed OS by lymph node sampling status and nodal metastasis. RESULTS: There were 103 (7.9%) patients with CCS, 221 (17.0%) with epithelioid sarcoma, and 979 (75.1%) with synovial sarcoma. Lymph node sampling was more frequent in patients with CCS (56.3%) and epithelioid sarcoma (52.5%) versus synovial sarcoma (20.5%, p < .001). Synovial sarcoma metastasized to lymph nodes less frequently than CCS or epithelioid sarcoma (2.1% vs. 14.6% and 14.9%, p < .001). Across all subtypes, lymph node metastasis was associated with inferior OS (HR 2.02, CI 1.38-2.95, p < .001). Lymph node sampling was associated with improved OS in CCS (HR 0.35, CI: 0.15-0.78, p = .010), inferior OS in synovial sarcoma (HR 1.60, CI: 1.13-2.25, p = .007), and no statistical association with OS in epithelioid sarcoma. CONCLUSIONS: Lymph node metastasis is rare in children and young adults with synovial sarcoma. Lymph node sampling procedures were not consistently performed for patients with CCS or epithelioid sarcoma, but improved OS supports routine lymph node sampling in children and young adults with CCS.

Laparoscopic Versus Open Surgical Repair of Duodenal Atresia: A NSQIP-Pediatric Analysis.

INTRODUCTION: Laparoscopic repair of duodenal atresia in neonates has gained popularity among some pediatric surgeons. Single-center studies suggest comparable short-term outcomes to open surgery. The purpose of this study was to utilize a large, multi-institutional pediatric dataset to examine 30-day post-operative outcomes by operative approach for newborns who underwent duodenal atresia repairs. METHODS: We identified neonates aged ≤1 wk in the 2016-2018 National Surgical Quality Improvement Program-Pediatric -database that underwent a laparoscopic or open repair for duodenal atresia. Preoperative characteristics were compared between operative approaches. Postoperative complications, operative time, postoperative length of stay (LOS), and supplemental nutrition at discharge were assessed using multivariate regressions. RESULTS: There were 267 neonates who met inclusion criteria. There were 233 (87%) infants who underwent open repairs and 34 (13%) who underwent laparoscopic repairs. Ten (29%) children who had laparoscopy were converted to open. After adjusting for confounding, laparoscopy was associated with an increase in operative time by 65 min (95% confidence interval 45-87 min, P < 0.001) but a five-day shorter LOS (95% confidence interval -9 to -2, P = 0.006) when compared to laparotomy. There were no significant differences in postoperative complications or supplemental nutrition at discharge. CONCLUSIONS: Our findings suggest that laparoscopic repairs of duodenal atresia are associated with shorter postoperative LOS but longer operative times when compared to open repairs. Although the conversion rate to laparotomy remained relatively high, the laparoscopic approach was associated with comparable 30-day postoperative outcomes.