Failure to Significantly Reduce Radiation Exposure in Children with Suspected Appendicitis in the United States.

BACKGROUND: Ultrasound (US) or magnetic resonance imaging (MRI) is recommended over computed tomography (CT) as the initial imaging modality when considering a diagnosis of appendicitis in children. This reduces unnecessary radiation exposure and has excellent accuracy. We hypothesized a significant increase in US utilization and a reduction in CT utilization in hospitals across the United States. METHODS: We retrospectively reviewed NSQIP-P data from 2015 to 2021 for patients <18 years undergoing appendectomy for acute appendicitis. Rates of US, CT, and MRI usage were compared between NSQIP-P hospitals and referring non-NSQIP-P hospitals. RESULTS: Of the 115,186 children included, 66,303 (57.6%) were imaged in NSQIP-P hospitals, 37,962 (33.0%) in non-NSQIP-P hospitals, and 7947 (6.9%) in both. US alone was used in 53.3%, followed by CT alone in 25.1%, both US + CT in 16.4%, and MRI ± CT/US in 2.6%. Non-NSIQP-P hospitals used less US than NSQIP-P centers (38.6% vs 90.8%, P < 0.0001) and more CT (74.0% vs 25.4%, P < 0.0001). From 2015 to 2021, overall US utilization increased from 68.5% to 72.3% (p < 0.0001) while CT utilization remained unchanged (43.1%-43.2%, P = 0.07). US use increased in non-NSQIP-P centers (18.8%-25.7%, P < 0.0001) but not in NSQIP-P (71.7% vs 70.9%, p = 0.28), while CT alone decreased in both (NSQIP-P: 10.1%-7.7%, P < 0.0001; non-NSQIP-P: 71.0%-59.8%, P < 0.0001). CONCLUSION: US and MRI remain underutilized in diagnosing pediatric appendicitis, especially in non-NSQIP-P hospitals. Trends show modest increase in US utilization; however, CT alone remains a highly used modality in non-NSQIP-P hospitals. Adopting diagnostic strategies from NSQIP-P centers could optimize diagnostic imaging in children. LEVEL OF EVIDENCE: III.

Multi-dimensional immunotyping of human NF1-associated peripheral nerve sheath tumors uncovers tumor-associated macrophages as key drivers of immune evasion in the tumor microenvironment.

PURPOSE: Malignant peripheral nerve sheath tumors (MPNST) are aggressive soft tissue sarcomas and the leading cause of mortality in individuals with neurofibromatosis type 1 (NF1). Despite many clinical trials, outcomes for patients with MPNST have remained stagnant and most succumb to their disease; thus, novel therapeutic approaches are needed. A better understanding of the MPNST immune ecosystem will aid in the development of strategies to activate the immune system against the tumor. Herein, we profile the tumor immune microenvironment (TIME) in NF1-associated peripheral nerve sheath tumors (PNST) to discover insights on the role that tumor-infiltrating immune cells play in malignant transformation. EXPERIMENTAL DESIGN: Utilizing fresh and formalin-fixed, paraffin-embedded tissue from patients diagnosed with NF1-PNST, we dissected the TIME by using immunohistochemistry, multiparameter flow cytometry, and comparative transcriptomic studies. RESULTS: Immunophenotyping confirmed increased immune cells infiltration during malignant progression, with a predominance of infiltrating myeloid cells, particularly CD163+ tumor-associated macrophages (TAM). The T cells within MPNST exhibited signs of tumor activation, characterized by high PD-1 expression. Additionally, MPNST specimens demonstrated elevated levels of immunosuppressive TAM, with heightened PD-L1 expression. The proportion of CD163+ myeloid cells within the TIME correlated with poorer progression free survival. Notably, loss of H3K27 trimethylation correlated with low immune cell infiltration in MPNST. CONCLUSIONS: Malignant transformation of NF1-PNST is characterized by an immunosuppressive microenvironment comprising of TAM with high expression of PD-L1, which are associated with inferior outcomes. These findings suggest a clinical potential of immune modulating therapeutics that can unleash an anti-tumor immune response.

Evaluation of Limited English Proficiency in the Management and Outcomes of Appendicitis in Children.

INTRODUCTION: Appendectomies are one of the most common pediatric surgical procedures. Limited English proficiency (LEP) may lead to disparities in health outcomes between English-proficient and LEP patients. This study assesses the association between LEP and postoperative outcomes in pediatric appendectomy. METHODS: We analyzed records from the American College of Surgeons National Surgical Quality Improvement Program-Pediatric database from 2010 to 2023 under 18 y of age undergoing appendectomy at our institution. LEP was defined as the need for an interpreter. Primary outcomes were postoperative complications, length of stay (LOS), and postoperative emergency department (ED) visits within 30 d of discharge. Multivariable analyses were performed. RESULTS: One thousand one hundred forty three children with appendicitis were identified, with 208 (18.2%) LEP and 935 (81.8%) English-proficient patients. LEP children were more likely to present with complicated appendicitis (42.8% versus 25.5%, P < 0.0001) and sepsis (34.1% versus 21.6%, P = 0.0003). LEP patients experience more serious (8.6% versus 3.9%, P = 0.02), overall complications (10.1% versus 5.5%, P = 0.006), and organ/surgical space site infections (8.2% versus 3.3%, P = 0.003). On multivariable analysis controlling for ethnicity and factors associated with complicated presentation, LEP was associated with increased postoperative ED visits (adjusted odds ratio [aOR] 2.64, 95% confidence interval [CI] = 1.40-4.39), but not LOS (aOR 1.86, 95% CI = 0.87-3.97) or complications (aOR 1.76, 95% CI = 0.79-4.00). CONCLUSIONS: LEP is independently associated with increased postoperative ED visits. Higher rates of complications and longer LOS may be related to increased complicated appendicitis at presentation. The role of cultural preferences and other social determinants of health that contribute to these disparities needs more investigation.

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.

Interpersonal Violence-Related Trauma Among South African Children.

INTRODUCTION: Injuries account for a major portion of disability-adjusted life years in children globally, and low-and middle-income countries are disproportionally affected. While injuries due to motor vehicle collisions and self-harm have been well-characterized in pediatric populations in South Africa, injuries related to interpersonal violence (IPV) are less understood. Our study aims to characterize patterns of injury, management, and outcomes for pediatric patients presenting with IPV-related injuries in a South African trauma center. METHODS: We performed a retrospective review of trauma patients ≤18 y of age presenting to the Pietermaritzburg Metropolitan Trauma Service in Gray's Hospital in South Africa from 2012 to 2022, comparing those with injuries resulting from IPV to those with non-IPV injuries. Patients' and injury pattern characteristics and outcomes were descriptively analyzed. RESULTS: Out of 2155 trauma admissions, 500 (23.2%) had IPV-related injuries. Among patients with IPV-related injuries, the median age was 16.0 y. 407 (81.4%) patients were male. 271 (54.2%) patients experienced blunt trauma, 221 (44.2%) had penetrating trauma, and 3 (0.6%) suffered both. The most common weapons were knives (21.6%), stones (11.2%), and firearms (11.0%). The most commonly injured regions were the head (56.4%), abdomen (20.8%), and thorax (19.2%). 19.6% underwent surgical intervention, and 14.4% were referred out for subspecialty care. 1.4% patients died, and 1.2% returned to Pietermaritzburg Metropolitan Trauma Service within 30 d of discharge. CONCLUSIONS: IPV patients are a distinctive subgroup of pediatric trauma patients with different demographics, patterns of injury, and clinical needs. Further research is needed to better understand the unique needs of this neglected population.

A Scoping Review of Limited English Proficiency and Immigration in Pediatric Surgery.

INTRODUCTION: With increasing globalization and diversity, the intersection of immigration and language barriers can impact patient outcomes. This scope review aims to summarize current evidence on immigration and language barriers on pediatric surgical outcomes. METHODS: A systematic review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines. Four databases were searched with Medical Subject Heading terms describing pediatric surgery, immigration, limited English proficiency (LEP), and refugees between 2000-2023. Four independent reviewers screened and analyzed texts for final inclusion. RESULTS: Thirty-three studies were included. Ten studies described disease incidence and severity, finding that LEP, immigrant, and refugee patients were more likely to present with severe disease in appendicitis and traumatic injuries. five studies described pain management, finding patients with LEP received fewer pain assessments, waited longer for analgesia, and had more discrepancies in pain scores. Seventeen studies investigated treatment receipt and delay, finding that immigrants and patients with LEP had longer time to and reduced rates of treatment. Seventeen studies described surgical outcomes, finding that patients with LEP have longer length of stay and more postoperative emergency department visits but fewer follow-up appointments. In kidney transplants, patients with LEP and immigrants had worse outcomes, but these trends are not seen in immigrants from Europe. Overall, immigrants and refugees have higher rates of complications and mortality. CONCLUSIONS: Immigrants and patients with LEP and are more likely to present with advanced disease and severe injuries, receive inadequate pain management, experience delays in surgery, and suffer more complications. There is continued need to assess the impact of LEP and immigration on pediatric surgery outcomes.

Contemporary surgical management of pediatric non-rhabdomyosarcoma soft tissue sarcoma.

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

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

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

Enhanced Recovery After Surgery 2.0: Optimizing Pain Management in Nuss Procedure: Cryoablation and Nerve Block Strategies for Reduced Opioid Use.

INTRODUCTION: Our study assesses the association between cryoablation, with and without nerve block supplementation, post-Nuss procedure pain, and opioid use in pectus excavatum (PE) patients. METHODS: We conducted a retrospective cohort study at a single center for PE patients who underwent the Nuss procedure from 2017 to 2022. Outcomes included postoperative opioid use (measured in oral morphine milligram equivalent per kilogram [OME/kg]), average pain score (scale 0-10), and length of stay (LOS). RESULTS: One hundred sixty-four patients (146 males and 18 females) were included, with 79 (48.2%) receiving neither cryoablation nor nerve block, 60 (36.6%) receiving intraoperative cryoablation alone, and 25 (15.2%) receiving both cryoablation and nerve block. The median age was 16 y. Nerve block recipients consumed fewer opioids during hospitalization than cryoablation alone and nonintervention groups (1.5 versus 2.3 versus 5.8 OME/kg, respectively, P < 0.0001). Average pain scores over the total LOS were lower in nerve block recipients (3.5 versus 3.8 versus 4.2, P = 0.03), particularly on postoperative day 0 (P = 0.002). Nerve block recipients had a shorter LOS than cryoablation alone and nonintervention groups (43.4 versus 54.7 versus 66.2 h, P < 0.0001). On multivariate analysis, cryoablation alone resulted in significantly less opioid use compared to no intervention (3.32 OME/kg reduction, 95% confidence interval -4.16 to -2.47, P < 0.0001). Addition of nerve block further reduced opioid use by 1.10 OME/kg (95% confidence interval -2.07 to -0.14, P = 0.04). CONCLUSIONS: Cryoablation with nerve block supplementation is associated with reduced pain, opioid use, and LOS post-Nuss for PE repair compared to cases without cryoablation or with cryoablation only. Cryoablation with regional nerve blocks should be considered for Nuss repair under the enhanced recovery after surgery pathway.

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.

Liver transplant for primary biliary tract neuroendocrine tumor in a nine-year-old girl.

BACKGROUND: Neuroendocrine tumors (NETs) are rare epithelial neoplasms that arise most commonly from the gastrointestinal tract. In pediatrics, the most common site of origin is in the appendix, with the liver being the most common site of metastasis. Neuroendocrine tumors arising from the biliary tract are extremely rare. METHODS: We describe a case of a nine-year-old girl who presented with obstructive cholestasis and was found to have multiple liver masses identified on biopsy as well-differentiated neuroendocrine tumor with an unknown primary tumor site. RESULT: The patient underwent extensive investigation to identify a primary tumor site, including endoscopy, endoscopic ultrasound, and capsule endoscopy. The patient ultimately underwent definitive management with liver transplant, and on explant was discovered to have multiple well-differentiated neuroendocrine tumors, WHO Grade 1, with extensive infiltration into the submucosa of bile duct, consistent with primary biliary tract neuroendocrine tumor. CONCLUSION: Identifying the site of the primary tumor in NETs found within the liver can be challenging. To determine if an extrahepatic primary tumor exists, workup should include endoscopy, EUS, and capsule endoscopy. Children with well-differentiated hepatic NETs, with no identifiable primary tumor, and an unresectable tumor, are considered favorable candidates for liver transplantation.

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.

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.

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.