Pediatric Surgical Research Research Articles

Survival outcomes in pediatric patients with metastatic Ewing sarcoma who achieve a rapid complete response of pulmonary metastases.

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

Management of undifferentiated embryonal sarcoma of the liver: A Pediatric Surgical Oncology Research Collaborative study.

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. 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. Seventy-eight patients were identified with a median age of 9.9years [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 1.6cm [IQR: 0.0-4.4] and greater than 90% tumor necrosis in 79% of the patients undergoing delayed resection. R0 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 R0 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 4years [IQR: 2-8], the EFS was 70.0% [95% CI: 60%-82%] and OS was 83% [95% CI: 75%-93%]. Complete resection is associated with improved survival for patients with UESL. Neoadjuvant chemotherapy causes minimal radiographic response, but significant tumor necrosis.

Management and Outcomes of Wilms Tumor With Suprarenal Intravascular Extension: A Pediatric Surgical Oncology Research Collaborative Study.

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

Survival outcomes in pediatric patients with metastatic Ewing sarcoma who achieve a rapid complete response of pulmonary metastases.

11535 Background: Twenty-five percent of patients with Ewing Sarcoma (EWS) have metastatic disease at diagnosis, most commonly in the lungs. Our objectives were to compare overall (OS) and pulmonary disease-free survival (PDFS) between patients with metastatic EWS at diagnosis who achieve rapid complete response (RCR), defined as radiographic resolution of all pulmonary nodules at the end of induction chemotherapy, and those with residual pulmonary nodules after induction chemotherapy (non-RCR). Methods: This multi-center, retrospective cohort study included children under 20 years of age with metastatic EWS treated from 2007-2020 at 19 institutions participating in the Pediatric Surgical Oncology Research Collaborative. Chi-square tests were conducted for differences among groups, and p < 0.05 was considered significant. Kaplan-Meier curves were generated for OS and PDFS. Cox regression was performed for OS, controlling for whole lung irradiation (WLI), extrapulmonary metastases, and age. Results: Among 153 patients with metastatic EWS at diagnosis, 61 (40%) achieved RCR, and 87 (57%) did not (5 unknown). The median number of pulmonary nodules was 3.5 in the RCR vs 5 in the non-RCR groups (p = 0.02), while bilateral disease was present in 44 (72%) RCR vs 75 (86%) non-RCR patients (p = 0.03). Pulmonary relapse occurred in 58 (38%) patients, including 18 (29%) in the RCR and 36 (41%) in the non-RCR groups (p = 0.14). Factors associated with pulmonary relapse in the univariate analysis include median age [15 years (IQR: 12-17) with pulmonary relapse vs 12 years (IQR: 9-16) without (p = 0.03)] and extrapulmonary metastases at diagnosis [24 (41%) patients with pulmonary relapse vs 26 (27%) patients without pulmonary relapse (p = 0.05)]. WLI was performed in 100 patients and was not associated with a reduction in pulmonary relapse (p = 0.93). Complete surgical clearance of pulmonary disease was attempted in 29 (19%) patients, including 17 of the patients who met the criteria for RCR. On Kaplan-Meier analyses, 5-year PDFS did not significantly differ based on RCR (67%) vs. non-RCR (53%, p = 0.13), or WLI (61%) vs. no WLI (54%, p = 0.32). However, 5-year OS did vary significantly between patients with RCR and WLI (85%), RCR without WLI (55%), non-RCR with WLI (60%), and non-RCR without WLI (25%; p < 0.01). In Cox regression analysis, RCR was associated with improved OS after controlling for WLI, extrapulmonary metastases, and patient age (HR 0.51, p = 0.03). Conclusions: Patients with EWS who had pulmonary metastases at diagnosis had improved OS if they achieved RCR and received WLI, despite having no significant differences in rates of pulmonary relapse. Further research is needed to determine whether clearance of pulmonary disease achieved through surgical metastasectomy provides a comparable survival benefit and if any subsets of patients have a reduced risk of pulmonary relapse after resection.

Rise of pediatric surgery collaboratives to facilitate quality improvement

Broad changes in pediatric surgical care delivery are limited by the rarity of pediatric surgical diseases and the geographic dispersion of pediatric surgical care across different hospital types. Pediatric surgical collaboratives and consortiums can provide the patient sample size, research resources, and infrastructure to advance clinical care for children with who require surgery. Additionally, collaboratives can bring together experts and exemplar institutions to overcome barriers to pediatric surgical research to advance quality surgical care. Despite challenges to collaboration, many successful pediatric surgical collaboratives emerged in the last decade and continue to push the field forward towards high-quality, evidence-based care and improved outcomes. This review will discuss the need for continued research and quality improvement collaboratives in pediatric surgery, identify challenges faced when building collaboratives, and introduce future directions to expand impact.

A successful centre for translational paediatric surgical research

The National Children's Research Centre (NCRC), the single largest paediatric research centre in Ireland, has been in existence for over 50years and is located on the grounds of the largest children's hospital in Ireland; Children's Health Ireland at Crumlin. Professor Puri was appointed as the Director of the Research in 1989 and became President of the NCRC in 2009, a position he held until 2016. Professor Puri is one of the most cited paediatric surgical researchers in the world. His research work has been cited over 23,500 times in peer-reviewed articles with an h-index of 76 and i10-index of 494. The aim of this review is to analyse the most relevant areas of academic research at the NCRC, Dublin, during the years when Prof. Puri was Director/President of the NCRC. In addition, the relevant factors essential to create a successful paediatric surgical research centre will be discussed. A literature search using PubMed/Medline was carried out using the search terms "Prem Puri" over a 40-year period (1980-2020). Articles were analysed to identify the most significant research areas in the field of paediatric surgical research and the relevant laboratory and clinical findings. In addition, a separate analysis of successful funding and human factors, such as research fellows working at the NCRC, was also carried out. During the period under review, Professor Puri's team published 750 articles in peer-reviewed journals. Three main areas of research were reviewed with a total number of 391 articles: congenital diaphragmatic hernia (CDH) was the topic with the largest number of publications (153), followed by Hirschsprung's disease (HSCR) (144) and Vesicoureteral reflux (VUR) (94). Eighty research fellows, all paediatric surgeons, from 18 different countries were trained in basic science under the supervision of Professor Puri at the NCRC. Over the last three decades, the NCRC has been one of the most successful centres for paediatric surgical research in the world. The three areas of research with the largest number of publications were identified as CDH, HSCR and VUR. Various factors can explain the success of the NCRC: (a) the constant presence, for over 30years, of a very successful paediatric surgeon leading the centre, (b) a multicultural laboratory with research fellows from all over the world and (c) grants of more than 15million raised over the years, which guaranteed a constant flow of resources for laboratory research.

The 35th International Symposium on Pediatric Surgical Research

The 35th International Symposium on Pediatric Surgical Research

The Peds Duct Score: A Highly Specific Predictive Model for Pediatric Choledocholithiasis

Introduction: To determine whether adult preoperative factors predicting choledocholithiasis (CD) are reliable in children and to create a validated pediatric-specific risk model. Methods: A multicenter retrospective cohort study was performed including children (<18 yo) who underwent cholecystectomy for gallstone disease at 10 Western Pediatric Surgical Research Consortium children’s hospitals between 2016-2019. Multivariable regression analyses and ROC curves were used to determine predictive factors and optimal cutoff values for CD. The predictive model was tested with a 20% hold-out sample. Results: The cohort comprised 979 children after cholecystectomy with an average age of 14.1+3.0 years, 75.1% female, 59.2% Hispanic, BMI was 27.7+8.3. Choledocholithiasis was confirmed in 222 patients (22.7%) by MRCP, ERCP, and/or IOC. Multivariable analyses with ROC curves identified three predictive factors for CD 1) a Dilated CBD>6mm, 2) Ultrasound (US) with visualized CBD stone, and 3) Total bilirubin>1.8 mg/dL (Peds DUCT criteria). These criteria demonstrated appropriate accuracies (>76%), specificities (>78%), and negative predictive values (>79%). Other factors commonly used in adults (elevated AST/ALT, pancreatitis, BMI, and age) were not independently predictive of CD in children. When tested on a hold-out sample, higher stratified risk groups demonstrated high accuracy, specificity, and negative predictive values similar to the adult CD literature. Conclusion: Unique to children, dilated CBD>6mm, US findings of CBD stone, and total bilirubin>1.8 mg/dL are three highly-specific clinical variables predictive of choledocholithiasis. The Peds DUCT score is simple, practical, and validated on a large, multi-institutional pediatric data set and should become standard for choledocholithiasis risk assessment in children.

Predictability and stability testing to assess clinical decision instrument performance for children after blunt torso trauma.

The Pediatric Emergency Care Applied Research Network (PECARN) has developed a clinical-decision instrument (CDI) to identify children at very low risk of intra-abdominal injury. However, the CDI has not been externally validated. We sought to vet the PECARN CDI with the Predictability Computability Stability (PCS) data science framework, potentially increasing its chance of a successful external validation. We performed a secondary analysis of two prospectively collected datasets: PECARN (12,044 children from 20 emergency departments) and an independent external validation dataset from the Pediatric Surgical Research Collaborative (PedSRC; 2,188 children from 14 emergency departments). We used PCS to reanalyze the original PECARN CDI along with new interpretable PCS CDIs developed using the PECARN dataset. External validation was then measured on the PedSRC dataset. Three predictor variables (abdominal wall trauma, Glasgow Coma Scale Score <14, and abdominal tenderness) were found to be stable. A CDI using only these three variables would achieve lower sensitivity than the original PECARN CDI with seven variables on internal PECARN validation but achieve the same performance on external PedSRC validation (sensitivity 96.8% and specificity 44%). Using only these variables, we developed a PCS CDI which had a lower sensitivity than the original PECARN CDI on internal PECARN validation but performed the same on external PedSRC validation (sensitivity 96.8% and specificity 44%). The PCS data science framework vetted the PECARN CDI and its constituent predictor variables prior to external validation. We found that the 3 stable predictor variables represented all of the PECARN CDI's predictive performance on independent external validation. The PCS framework offers a less resource-intensive method than prospective validation to vet CDIs before external validation. We also found that the PECARN CDI will generalize well to new populations and should be prospectively externally validated. The PCS framework offers a potential strategy to increase the chance of a successful (costly) prospective validation.

Incidence and management of pleural effusions in patients with Wilms tumor: A Pediatric Surgical Oncology Research Collaborative study.

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

Pediatric surgical research over the last decade: A bibliometric citation analysis

Objectives: A way of identifying significant works from a plethora of published material is by a bibliometric analysis. The citation count of a particular article indicates its influence within the readership. A survey of the top 10 articles (in terms of citations per year) in pediatric surgery from each of the three leading international journals (Journal of Pediatric Surgery [JPS], European Journal of Pediatric Surgery [EurJPS], and Pediatric Surgery International [PSI]) and the foremost Indian journal (Journal of Indian Association of Pediatric Surgeons [JIAPS]) should give an insight into the research that has been quoted the most in the last decade. Through an extensive literature search, this paper aims to identify, analyse and categorize the characteristics of the most cited papers in pediatric surgery in the last decade, worldwide and in India. Material and Methods: The number of citations of all articles from 4 journals (JPS, EurJPS, PSI, JIAPS) published between January 2013 and May 2022 were sorted using the “Publish or Perish®” software (version 8). The top 10 articles in each journal (by citations per year) published between January 2013 and May 2022 were then tabulated into a Microsoft Excel® sheet and details including the topic studied, citations per year, year of publication and type of study were noted. Results: Out of the 40 articles, there were 13 review articles and 27 original articles – 12 retrospective reviews, 6 consensus papers, 6 surveys and 3 animal research studies. 15 of these articles were from the United States of America (USA), 12 were from Europe and 3 were global, spanning continents. Among the top 10 cited papers from JIAPS, 8 were from India. The most commonly covered topics were esophageal atresia, necrotizing enterocolitis, acute appendicitis and Hirschsprung’s disease. Conclusion: The most influential manuscripts relate to common neonatal surgical conditions and appendicitis. This information should be of value to upcoming pediatric surgeons to choose their prospective research and create a long-lasting impact on the field of pediatric surgery. For the editorial team, it provides an insight into the kind of articles that are of interest to pediatric surgeons.

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 3.9cm. 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.

Histologic type predicts disparate outcomes in pediatric hepatocellular neoplasms: A Pediatric Surgical Oncology Research Collaborative study.

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. 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. 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. 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. 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.

The 34th international symposium on pediatric surgical research

The 34th international symposium on pediatric surgical research

Interhospital variability in localization techniques for small pulmonary nodules in children: A pediatric surgical oncology research collaborative study

BackgroundPulmonary nodules that are deep within lung parenchyma and/or small in size can be challenging to localize for biopsy. This study describes current trends in performance of image-guided localization techniques for pulmonary nodules in pediatric patients. MethodsA retrospective review was performed on patients < 21 years of age undergoing localization of pulmonary nodules at 15 institutions. Localization and resection success, time in interventional radiology (IR), operating room (OR) and total anesthesia time, complications, and technical problems were compared between techniques. Results225 patients were included with an average of 1.3 lesions (range 1–5). Median nodule size and depth were 4 mm (range 0–30) and 5.4 mm (0–61), respectively. The most common localization techniques were: wire + methylene blue dye (MBD) (28%), MBD only (25%), wire only (14%), technetium-99 only (11%), coil + MBD (7%) and coil only (5%). Localization technique was associated with institution (p < 0.01); technique and institution were significantly associated with mean IR, OR, and anesthesia time (all p < 0.05). Comparing techniques, there was no difference in successful IR localization (range 92–100%, p = 0.75), successful resection (94–100%, p = 0.98), IR technical problems (p = 0.22), or operative complications (p = 0.16). ConclusionsMany IR localization techniques for small pulmonary nodules in children can be successful, but there is wide variability in application by institution and in procedure time. Level of evidenceRetrospective review, Level 3.

Inflammatory myofibroblastic tumor: A multi-institutional study from the pediatric surgical oncology research collaborative.

10024 Background: Inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm of intermediate malignancy, predominantly seen in children and young adults. Given its rarity, data are limited. We describe the largest cohort of patients with IMT to date, with an aim to further characterize this poorly understood tumor. Methods: A multi-institutional review was performed at 18 North American hospitals participating in the Pediatric Surgical Oncology Research Collaborative to identify IMT patients ≤39 years, diagnosed from 2000-2018. Descriptive statistics are described as median and interquartile range. Multivariable analysis was used to identify predictors of event free survival (EFS). Results: 182 patients were identified with a median age of 11 years (5-17); 52% were female. 33% of tumors were thoracic, 26% abdominal/pelvic, 20% head/neck, and 14% genitourinary. Common presenting symptoms included pain (29%), respiratory symptoms (24%), weight loss (12%), and a palpable mass (10%). Median tumor size was 3.9 cm (1.9-6.5). Anaplastic lymphoma kinase (ALK) overexpression was identified via immunohistochemistry in 53% of patients tested. One third of patients had abnormal cytogenetics, with 12% of the entire cohort having an ALK mutation. 7% of patients had distant disease at diagnosis. 13% of patients received neoadjuvant therapy including chemotherapy (3%), ALK inhibitor (4%), radiation (0.5%), non-steroidal anti-inflammatories (NSAIDs) (7%), or steroids (2%). Of those who underwent resection with known margin status (n = 158), 66% had complete resection, 20% had microscopic positive margins, and 14% had gross residual disease. Just over 40% of patients had an en bloc resection of involved organs, most commonly lung (26%). 21% of patients received adjuvant therapy, including chemotherapy (3%), ALK inhibitor (9%), radiation (0.5%), NSAIDs (8%), or steroids (5%). 12% of all patients received an ALK inhibitor: 24% neoadjuvant, 62% adjuvant, and 14% without surgery. Median follow-up time was 36 months (14-69). Overall 5-year survival (OS) was 95% and 5-year EFS was 80%. Predictors of recurrence included respiratory symptoms, larger tumor size, or distant disease at diagnosis. Gender, race, age and primary site were not predictive of EFS. Likewise, there was no association of ALK overexpression or ALK mutation with EFS. The presence of gross or microscopic margins following resection was not associated with recurrence. Conclusions: IMT is a rare tumor with favorable OS. Five year recurrence rate was 20%. Presenting with respiratory symptoms, larger tumor size, or metastatic disease was associated with recurrence, while ALK positivity was not. Notably, a positive margin after resection was not associated with increased recurrence, indicating that aggressive attempts at surgical resection that would compromise form or function may not be warranted.

‘Paediatric surgical research in the UK – Challenges and opportunities’

Academic pediatric surgery faces challenges and opportunity. The author provides a brief overview of the landscape of academic surgery from a UK perspective and based on his considerable experience, makes suggestions for present and future directions.

Implications of Tumor Characteristics and Treatment Modality on Local Recurrence and Functional Outcomes in Children With Chest Wall Sarcoma: A Pediatric Surgical Oncology Research Collaborative Study.

To determine the impact of tumor characteristics and treatment approach on (1) local recurrence, (2) scoliosis development, and (3) patient-reported quality of life in children with sarcoma of the chest wall. Children with chest wall sarcoma require multimodal therapy including chemotherapy, surgery, and/or radiation. Despite aggressive therapy which places them at risk for functional impairment and scoliosis, these patients are also at significant risk for local recurrence. A multi-institutional review of 175 children (median age 13 years) with chest wall sarcoma treated at seventeen Pediatric Surgical Oncology Research Collaborative institutions between 2008 and 2017 was performed. Patient-reported quality of life was assessed prospectively using PROMIS surveys. The most common diagnoses were Ewing sarcoma (67%) and osteosarcoma (9%). Surgical resection was performed in 85% and radiation in 55%. A median of 2 ribs were resected (interquartile range = 1-3), and number of ribs resected did not correlate with margin status ( P = 0.36). Local recurrence occurred in 23% and margin status was the only predictive factor(HR 2.24, P = 0.039). With a median follow-up of 5 years, 13% developed scoliosis (median Cobb angle 26) and 5% required corrective spine surgery. Scoliosis was associated with posteriorrib resection (HR 8.43; P= 0.003) and increased number of ribs resected (HR 1.78; P = 0.02). Overall, patient-reported quality of life is not impaired after chest wall tumor resection. Local recurrence occurs in one-quarter of children with chest wall sarcoma and is independent of tumor type. Scoliosis occurs in 13% of patients, but patient-reported quality of life is excellent.

Pediatric Surgical Research Output in Germany in the Last 30 Years - An Assessment and International Comparison of Three Dedicated Paediatric Surgical Journals.

Purpose: Research output of once-leading countries in surgical journals is decreasing despite an overall increase of scientific publications by 8% per year. We aimed to assess research outputs of German, Dutch, and Israeli pediatric surgeons in dedicated pediatric surgical journals in order to get insight into trends in pediatric surgical research.Methods: We collected bibliographic information on all original articles in the Journal of Pediatric Surgery, European Journal of Pediatric Surgery, and Pediatric Surgery International in 1985–1988, 2000–2003, and 2015–2018 that had a German, Dutch or Israeli last author from a department of pediatric surgery. Citation counts were obtained from the Web of Science.Results: Research output of German pediatric surgery decreased from 19 manuscripts in 1988 (0.1/surgeon/year) to eight manuscripts in 2017 (0.02/surgeon/year), whereas those of the Netherlands increased from two manuscripts in 1985 (0.08/surgeon/year) to 12 manuscripts in 2016 (0.3/surgeon/year). The declining German research output negatively correlated with increasing numbers of specialist pediatric surgeons for total (τ = −0.54; P = 0.0156) and manuscripts per surgeon (τ = −0.79; P = 0.0001), resulting in a negative trend over time (χ2 = 11.845, P = 0.0006). Analyses of citation patterns revealed that manuscripts by Dutch pediatric surgeons and those published in the Journal of Pediatric Surgery had higher absolute citation counts than the reference category of a German manuscript in the European Journal of Pediatric Surgery. Age-corrected citation rates resembled this result by increasing from 2000 to 2003 ( = 0.799, range: 0–3.368) to 2015–2018 ( = 2, range: 0–5) (P = 0.035) for the Netherlands. Assessment of manuscript types revealed that the proportion of prospective studies increased in the German sample (χ2 = 5.05, P = 0.0246), but remained the lowest among the comparators. Surprisingly, the proportion of non-clinical manuscripts from Germany also increased over time (χ2 = 4.001, P = 0.0455), whereas it remained constant in both the Netherlands and Israel.Conclusion: German pediatric surgical research output decreased in the last thirty years based on the sample of dedicated pediatric surgical journals, while Dutch productivity increased. Citation rates—as a measure of scientific impact—were associated and increased with Dutch manuscripts. The involved factors remain to be determined and whether this represents a shift toward other journals or mirrors a general development.

Pneumonectomy for Pediatric Tumors-a Pediatric Surgical Oncology Research Collaborative Study.

To describe utilization and long-term outcomes of pneumonectomy in children and adolescents with cancer. Pneumonectomy in adults is associated with significant morbidity and mortality. Little is known about the indications and outcomes of pneumonectomy for pediatric tumors. 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. 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). 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.