Importance Despite evidence that enhanced recovery protocols (ERPs) improve outcomes in adults undergoing surgery, adoption for pediatric populations has lagged. Objective To assess the implementation and clinical effectiveness of a consensus-based ERP for pediatric patients undergoing elective gastrointestinal (GI) surgery. Design, Setting, and Participants A prospective type 2 hybrid implementation-effectiveness, stepped-wedge, cluster-randomized by entry date into implementation phase, trial of pediatrics patients, 10 to 18 years of age, undergoing elective GI surgery at 18 US sites from September 2019 to June 2024. Interventions Sites were randomized into 3 groups, each spending at least 9 months in a control phase, with usual care, followed by an implementation phase at 6-month intervals that included a 21-element ERP supported by a structured Implementation Toolkit, based on 5 Active Implementation Frameworks (5AIFs), and a sustainment phase (12-24 months). Implementation was facilitated by a 1-year, group-based Learning Collaborative curriculum, a repository of tools, ERP adherence feedback, and implementation report cards. Main Outcomes and Measures Site-level scores were created based on 5AIFs domains. ERP adherence was assessed by ERP elements delivered at patient and site level. The primary effectiveness outcome, postoperative length of stay (LOS), and secondary effectiveness outcomes (including opioid use, time to regular diet, complications, readmission, and patient-reported health-related quality of life [HRQOL]) were evaluated across study phases (baseline, implementation, and sustainability). Correlations between site-level implementation scores and fidelity were estimated. Results Of the 597 enrolled pediatric patients (median [IQR] age, 15 [13-17] years; 274 [45.9%] female; 323 [54.1%] male), 433 (72.5%) had inflammatory bowel disease. No significant differences were found by study phase in LOS or secondary outcomes, except shorter time to regular diet and decreased opioid use during hospitalization. Patients who received at least 13 ERP elements had shorter median LOS (−1.14 days [95% CI −2.01 to −0.27]) and fewer complications (adjusted odds ratio, 0.48 [95% CI, 0.28-0.82]). Patient-level adherence increased by study phase (number of ERPs: 11 [10-13], 14 [12-15], and 14 [13-15], [ P < .001]). ERP integration into order sets and site culture were moderately correlated with fidelity. Conclusions and Relevance This stepped-wedge cluster-randomized trial found that despite multifaceted implementation strategies, a pediatric GI surgery ERP did not significantly reduce LOS. However, when accounting for implementation fidelity at the patient level, it resulted in significantly lower LOS and complications. Trial Registration ClinicalTrials.gov Identifier: NCT04060303

Importance:Despite evidence that enhanced recovery protocols (ERPs) improve outcomes in adults undergoing surgery, adoption for pediatric populations has lagged. Objective:To assess the implementation and clinical effectiveness of a consensus-based ERP for pediatric patients undergoing elective gastrointestinal (GI) surgery. Design, Setting, and Participants:A prospective type 2 hybrid implementation-effectiveness, stepped-wedge, cluster-randomized by entry date into implementation phase, trial of pediatrics patients, 10 to 18 years of age, undergoing elective GI surgery at 18 US sites from September 2019 to June 2024. Interventions:Sites were randomized into 3 groups, each spending at least 9 months in a control phase, with usual care, followed by an implementation phase at 6-month intervals that included a 21-element ERP supported by a structured Implementation Toolkit, based on 5 Active Implementation Frameworks (5AIFs), and a sustainment phase (12-24 months). Implementation was facilitated by a 1-year, group-based Learning Collaborative curriculum, a repository of tools, ERP adherence feedback, and implementation report cards. Main Outcomes and Measures:Site-level scores were created based on 5AIFs domains. ERP adherence was assessed by ERP elements delivered at patient and site level. The primary effectiveness outcome, postoperative length of stay (LOS), and secondary effectiveness outcomes (including opioid use, time to regular diet, complications, readmission, and patient-reported health-related quality of life [HRQOL]) were evaluated across study phases (baseline, implementation, and sustainability). Correlations between site-level implementation scores and fidelity were estimated. Results:Of the 597 enrolled pediatric patients (median [IQR] age, 15 [13-17] years; 274 [45.9%] female; 323 [54.1%] male), 433 (72.5%) had inflammatory bowel disease. No significant differences were found by study phase in LOS or secondary outcomes, except shorter time to regular diet and decreased opioid use during hospitalization. Patients who received at least 13 ERP elements had shorter median LOS (-1.14 days [95% CI -2.01 to -0.27]) and fewer complications (adjusted odds ratio, 0.48 [95% CI, 0.28-0.82]). Patient-level adherence increased by study phase (number of ERPs: 11 [10-13], 14 [12-15], and 14 [13-15], [P < .001]). ERP integration into order sets and site culture were moderately correlated with fidelity. Conclusions and Relevance:This stepped-wedge cluster-randomized trial found that despite multifaceted implementation strategies, a pediatric GI surgery ERP did not significantly reduce LOS. However, when accounting for implementation fidelity at the patient level, it resulted in significantly lower LOS and complications. Trial Registration:ClinicalTrials.gov Identifier: NCT04060303.

Background Traumatic pancreatic laceration with ductal injury in children can be managed non-operatively (NOM); however, variable management affects outcomes. We hypothesized that a standardized management approach with early feeding and limited resource utilization is safe and improves outcomes. Method Prospective, multicenter study of 13 pediatric trauma centers (2018–2022). Children with blunt pancreatic trauma with ductal injury were managed per a standard NOM pathway. Outcomes were compared to a historical NOM cohort with variable management. Results Of 22 patients, the median age was 7.5 years (range 1–14 years). Low-fat diet was started at median 4 days [IQR 2–7] and median hospital stay was 8 days [IQR 4–10]. One patient failed NOM and underwent distal pancreatectomy. Of the rest, most (17/21, 81%) had early recovery and discharged in median 6 days [IQR 4–8.25] while 4 (19%) had prolonged recovery (median stay 24 days, IQR 19.8–30.5) and higher peri-pancreatic cyst development (early 23.5% vs prolonged 75%,p = 0.05). Pancreatic ascites at presentation correlated with cyst development (p < 0.0001). Endoscopic stent (optional) was placed in 33% and did not prevent cyst development. Delayed exocrine pancreatic insufficiency was noted in 1 patient. Compared to the historic cohort (32 patients), TPN use was lower (pre-protocol 56% vs post 23%, p = 0.02), days to diet was shorter (pre-protocol 7 vs post 4; p = 0.03), and cyst development was lower (pre-protocol 81% vs post 33%, p < 0.001). Conclusion Children with traumatic pancreatic ductal injury can be safely managed per the PTS NOM clinical pathway and most recover rapidly. Pancreatic ascites may predict pseudocyst formation. Levels of Evidence IV. Study type: therapeutic, comparative.

OBJECTIVE:To evaluate for disparities in surgical care among US children with hepatoblastoma (HB) and hepatocellular carcinoma (HCC). STUDY DESIGN:In this retrospective National Cancer Database study (2004-2015), children aged <18 years with HB or HCC were included. Multivariable mixed-effects logistic regression was used to evaluate the association of sociodemographic factors (age, sex, race and ethnicity, insurance status, income, proximity to treating hospital) with the odds of undergoing surgical treatment after adjusting for disease-related factors (tumor size, metastasis, comorbidities) and hospital-level effects. Subgroup analyses by tumor histology were performed. RESULTS:A total of 811 children were included (HB: 80.9%; HCC: 19.1%), of which 610 (75.2%) underwent surgical treatment. Following adjustment, decreased odds of undergoing surgical treatment were associated with Black race (OR: 0.46 vs White, 95% CI [95% CI]: 0.26-0.80, P = .01), and having Medicaid (OR: 0.58 vs private, 95% CI: 0.38-0.88, P = .01) or no insurance (OR: 0.33 vs private, 95% CI: 0.13-0.80, P = .02). In children with HB, Black race was associated with decreased odds of undergoing surgical treatment (OR: 0.47 vs White, 95% CI: 0.25-0.89, P = .02). In children with HCC, Medicaid (OR: 0.10 vs private, 95% CI: 0.03-0.35, P < .001), or no insurance status (OR: 0.10 vs private, 95% CI: 0.01-0.83, P = .03) were associated with decreased odds of undergoing surgical treatment. Other than metastatic disease, no additional factors were associated with likelihood of surgical treatment in any group. CONCLUSIONS:Black race and having Medicaid or no insurance are independently associated with decreased odds of surgical treatment in children with HB and HCC, respectively. These children may be less likely to undergo curative surgery for their liver cancer.

ImportanceInguinal hernia repair in preterm infants is common and is associated with considerable morbidity. Whether the inguinal hernia should be repaired prior to or after discharge from the neonatal intensive care unit is controversial.ObjectiveTo evaluate the safety of early vs late surgical repair for preterm infants with an inguinal hernia.Design, Setting, and ParticipantsA multicenter randomized clinical trial including preterm infants with inguinal hernia diagnosed during initial hospitalization was conducted between September 2013 and April 2021 at 39 US hospitals. Follow-up was completed on January 3, 2023.InterventionsIn the early repair strategy, infants underwent inguinal hernia repair before neonatal intensive care unit discharge. In the late repair strategy, hernia repair was planned after discharge from the neonatal intensive care unit and when the infants were older than 55 weeks’ postmenstrual age.Main Outcomes and MeasuresThe primary outcome was occurrence of any prespecified serious adverse event during the 10-month observation period (determined by a blinded adjudication committee). The secondary outcomes included the total number of days in the hospital during the 10-month observation period.ResultsAmong the 338 randomized infants (172 in the early repair group and 166 in the late repair group), 320 underwent operative repair (86% were male; 2% were Asian, 30% were Black, 16% were Hispanic, 59% were White, and race and ethnicity were unknown in 9% and 4%, respectively; the mean gestational age at birth was 26.6 weeks [SD, 2.8 weeks]; the mean postnatal age at enrollment was 12 weeks [SD, 5 weeks]). Among 308 infants (91%) with complete data (159 in the early repair group and 149 in the late repair group), 44 (28%) in the early repair group vs 27 (18%) in the late repair group had at least 1 serious adverse event (risk difference, −7.9% [95% credible interval, −16.9% to 0%]; 97% bayesian posterior probability of benefit with late repair). The median number of days in the hospital during the 10-month observation period was 19.0 days (IQR, 9.8 to 35.0 days) in the early repair group vs 16.0 days (IQR, 7.0 to 38.0 days) in the late repair group (82% posterior probability of benefit with late repair). In the prespecified subgroup analyses, the probability that late repair reduced the number of infants with at least 1 serious adverse event was higher in infants with a gestational age younger than 28 weeks and in those with bronchopulmonary dysplasia (99% probability of benefit in each subgroup).Conclusions and RelevanceAmong preterm infants with inguinal hernia, the late repair strategy resulted in fewer infants having at least 1 serious adverse event. These findings support delaying inguinal hernia repair until after initial discharge from the neonatal intensive care unit.Trial RegistrationClinicalTrials.gov Identifier: NCT01678638

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

Hospital length of stay (LOS) following admission for appendicitis is difficult to predict. Shock index, pediatric age adjusted (SIPA) accurately identifies severely injured trauma patients and predicts mortality among children admitted to the ICU. Our aim was to determine if elevated SIPA at presentation, and time to normalization of SIPA, can identify children with perforated appendicitis and predict hospital LOS. This was a retrospective cohort study of children 1–17 years admitted to a quaternary care referral center with appendicitis after appendectomy in 2021. The primary outcomes were presence of perforated appendicitis and hospital LOS. Generalized linear regressions were performed. Covariates included in all models were age, sex, fecalith, initial temperature, and time from diagnosis to OR. We included 169 patients; 53 (31.4

Background Children with total colonic Hirschsprung disease (TCHD) are a unique group of patients with pre- and postoperative management challenges. This review provides a rational, expert-based approach to diagnosing and managing TCHD. Methods The guidelines were developed by the Hirschsprung Disease Interest Group members established by the American Pediatric Surgical Association (APSA) Board of Governors. Group discussions, literature review, and expert consensus were used to summarize the current knowledge regarding diagnosis, staged approach, the timing of pull-through, and pre-and postoperative management in children with TCHD. Results This paper presents recommendations for managing TCHD before and after reconstruction, including diagnostic criteria, surgical approaches, bowel management, diet, antibiotic prophylaxis, colonic irrigations, and post-surgical considerations. Conclusions A clear understanding of the unique challenges posed by TCHD and consensus on its treatment are lacking in the literature. This review standardizes this patient group's pre- and postoperative management. Level of Evidence V

OBJECTIVE:To describe the impact of lack of workplace support (LOWS) for obstetric health on surgeon distress and career satisfaction. BACKGROUND:Although most pregnant surgeons desire clinical duty reductions to mitigate obstetric risk, few modify their schedules due to low workplace support. METHODS:US surgeons with at least 1 live birth completed an electronic survey. LOWS during pregnancy was defined as (1) disagreeing that colleagues/leadership were supportive of obstetric-mandated bedrest; (2) feeling unable to reduce clinical duties despite health concerns due to risk of financial penalties, the requirement to make up missed call shifts, being perceived as "weak," burdening colleagues, or accommodations being denied by the workplace. Multivariate logistic regression determined the association between LOWS and burnout, low quality of life, plans to leave clinical practice or to reduce work hours, and the likelihood of recommending a surgical career to one's child. RESULTS:Of 557 surgeons, the 360 (64.6%) who reported LOWS during pregnancy were more likely to report burnout (OR:2.57; 95% CI: 1.60-4.13), low quality of life (OR: 1.57; 95% CI: 1.02-2.41), a desire to leave their practice (OR: 2.74; 95% CI: 1.36-5.49), plans to reduce clinical hours in the next year (OR: 4.25; 95% CI: 1.82-9.90), and were less likely to recommend their career to their child (OR: 0.44; 95% CI: 0.28-0.70). CONCLUSIONS:LOWS for maternal-fetal health concerns is associated with burnout, low quality of life, and career dissatisfaction. The work environment is a modifiable factor requiring system-level interventions to limit clinical work during pregnancy and provide fair compensation for covering surgeons. Supporting surgeons during pregnancy is a short-term investment with long-term implications for improving the longevity and diversity of the workforce.

Hirschsprung-associated enterocolitis (HAEC) is an important cause of morbidity and the leading cause of mortality in patients with Hirschsprung disease. The pathophysiology of disease includes dysmotility of the enteric nervous system, dysbiosis of the microbiota, failure of the intestinal barrier, and impaired immunity. Common manifestations include fever, abdominal distension, lethargy, vomiting, and diarrhea. Given the non-specific signs and symptoms of HAEC, high clinical suspicion is warranted, especially in patients with risk factors. Diagnosis and management of HAEC depend on the severity of disease presentation. Several preoperative and postoperative modalities have been explored to prevent HAEC. The current review elaborates on the risk factors, pathogenesis, diagnosis, treatment, and prevention of HAEC.

IntroductionThere is wide variation in the cost of disposable operating room supplies between surgeons performing the same operation at the same institution. The general relationship between variation in disposable supply cost and patient outcomes is unknown. We aimed to evaluate the relationship between disposable supply cost and patient outcomes for sixteen common operations.MethodsCost data were reviewed for the most common procedures performed by five surgical divisions at a single children’s hospital over a six-month period in 2021. For procedure, the median disposable OR costs were calculated. Each operation performed was categorized as low cost (below the group median) or high cost (above the group median. We compared the rates of adverse events (clinic visit within 5 days, 30-day emergency department visit, unplanned reoperation, unplanned readmission, anesthesia complications, prolonged hospital length of stay, need for blood product transfusion, or death) between procedures with low and high disposable supply costs.Results1139 operations performed by 48 unique surgeons from five specialties were included; 596 (52%) were low-cost and 543 (48%) high-cost. The low and high-cost groups did not differ regarding most demographic characteristics. Overall, 21.9% of children suffered any adverse outcome; this rate did not differ between the low and high-cost groups when evaluated individually or in aggregate (20.5% vs 23.6%, p=0.23).ConclusionOur data demonstrate that across a wide range of pediatric surgical procedures, the cost of disposable operating room supplies was not associated with the risk of adverse outcomes.Level of Evidencelevel 3

INTRODUCTION:Dr. Asa G. Yancey published a surgical technique describing a pull - through of normal colon through a cuff of aganglionic colon to treat Hirschsprung disease in 1952, 12 y before Dr. Franco Soave whose name is attached to the procedure. Yancey and his pioneering operation went unrecognized for over half a century because of discriminatory segregation in the publishing practices of academic medicine dating back to the 1950s. MATERIALS AND METHODS:We performed a literature review on the surgical therapies for Hirschsprung disease. This history was supplemented with first-hand accounts provided by Yancey's children. Further information by leaders of the American Pediatric Surgical Association Hirschsprung interest group regarding the future of surgical nomenclature for the endorectal pull-through procedure was acquired through interviews. RESULTS:A review of the literature revealed that Yancey's description of the pull-through technique for Hirschsprung disease was published 12 y prior to Soave's publication and yet, Yancey received little to no recognition for his work. Yancey's children describe a surgeon who was persistent in his endeavor to create a more inclusive field of academic surgery as well as a man who prioritized his family and the education of future surgeons. Conversations with the American Pediatric Surgical Association Hirschsprung interest group suggest active changes to give posthumous credit to Yancey, including renaming the procedure to the Yancey - Soave pull-through technique. CONCLUSIONS:Here, we provide a historical review of Yancey's career as well as insights on the man behind the surgery and how he persevered in academic surgery despite experiencing discrimination during the civil rights movement.

INTRODUCTION:Predicted 1-year survival of children with trisomy 18 (T18) has increased to 59.3%. We aimed to systematically review the characteristics, management, and outcomes of children with T18 and hepatoblastoma. METHODS:A systematic literature review of the PubMed, Embase, Scopus, Web of Science, and Cochrane Library databases was performed according to the PRISMA 2020 statement (end-of-search date: 03/03/2024). RESULTS:Fifty studies reporting on 70 patients were included. The median age at diagnosis was 11.5 months, 85.9% were female (n = 55/64), and 15.0% had mosaic T18 (n = 6/40). Diagnosis was made during symptom evaluation (most commonly hepatomegaly or abdominal mass) in 45.5% (n = 15/33), incidentally in 24.2% (n = 8/33), during surveillance with abdominal ultrasound in 18.2% (n = 6/33), and at autopsy in 12.1% (n = 4/33). The median tumor size was 6.4 cm, 33.3% had multiple tumors (n = 14/42), and metastasis was present in one patient (3.8%; n = 1/26). Neoadjuvant chemotherapy was administered in 42.6% (n = 26/61) and adjuvant chemotherapy in 31.6% (n = 18/57). Surgical treatment was performed in 64.2% (n = 43/67). Of the patients not diagnosed on autopsy, overall mortality was 35.5% (n = 22/62) over a median follow-up of 11.0 months. Among the 26 deceased patients (including those diagnosed on autopsy), the most common causes of death were cardiopulmonary disease (38.5%, n = 10/26) and tumor progression (30.8%, n = 8/26). CONCLUSIONS:T18 does not preclude resection with curative intent for hepatoblastoma. Combination of surgery and chemotherapy should be considered in children on an individualized basis depending on tumor characteristics and underlying cardiopulmonary comorbidities. Locoregional modalities may have a role in the setting of severe comorbidities. LEVEL OF EVIDENCE:Level IV evidence.

Pancreaticoduodenectomy with vascular reconstruction is rarely performed in children. We present a 3-year-old male with stage IV hepatoblastoma and pre-treatment extent of disease (PRETEXT) stage III with tumor into the portal vein and superior mesenteric vein (SMV), and with brain and lung metastases status post chemotherapy and stereotactic radiosurgery to left frontal brain lesion. He then underwent deceased donor liver transplant with Roux-en-Y hepaticojejunostomy complicated by two recurrences to bilateral lungs treated with wedge resections. His course lastly involved a third hepatoblastoma recurrence to the SMV that was managed with pylorus-preserving pancreaticoduodenectomy with SMV resection and reconstruction.

BackgroundAt present, parents lack objective methods to evaluate their child’s postoperative recovery following discharge from the hospital. As a result, clinicians are dependent upon a parent’s subjective assessment of the child’s health status and the child’s ability to communicate their symptoms. This subjective nature of home monitoring contributes to unnecessary emergency department (ED) use as well as delays in treatment. However, the integration of data remotely collected using a consumer wearable device has the potential to provide clinicians with objective metrics for postoperative patients to facilitate informed longitudinal, remote assessment. ObjectiveThis multi-institutional study aimed to evaluate the impact of adding actual and simulated objective recovery data that were collected remotely using a consumer wearable device to simulated postoperative telephone encounters on clinicians’ management. MethodsIn total, 3 simulated telephone scenarios of patients after an appendectomy were presented to clinicians at 5 children’s hospitals. Each scenario was then supplemented with wearable data concerning or reassuring against a postoperative complication. Clinicians rated their likelihood of ED referral before and after the addition of wearable data to evaluate if it changed their recommendation. Clinicians reported confidence in their decision-making. ResultsIn total, 34 clinicians participated. Compared with the scenario alone, the addition of reassuring wearable data resulted in a decreased likelihood of ED referral for all 3 scenarios (P<.01). When presented with concerning wearable data, there was an increased likelihood of ED referral for 1 of 3 scenarios (P=.72, P=.17, and P<.001). At the institutional level, there was no difference between the 5 institutions in how the wearable data changed the likelihood of ED referral for all 3 scenarios. With the addition of wearable data, 76% (19/25) to 88% (21/24 and 22/25) of clinicians reported increased confidence in their recommendations. ConclusionsThe addition of wearable data to simulated telephone scenarios for postdischarge patients who underwent pediatric surgery impacted clinicians’ remote patient management at 5 pediatric institutions and increased clinician confidence. Wearable devices are capable of providing real-time measures of recovery, which can be used as a postoperative monitoring tool to reduce delays in care and avoidable health care use.

INTRODUCTION:Adrenocortical carcinoma (ACC) is a rare but aggressive pediatric endocrine tumor. However, there is no recent US national report on the management or outcomes of pediatric ACC. We aimed to examine the clinical characteristics, current management strategies, and outcomes of pediatric ACC. METHODS:In this retrospective National Cancer Database study between 2004 and 2019, children (<18 y) with ACC were included. Overall survival was examined by means of Kaplan-Meier method, log-rank tests, and Cox regression modeling. RESULTS:Seventy-eight children with ACC were included. The median age was 10 y, the median tumor size was 10.2 cm, and 35.9% had metastasis at diagnosis. Most patients underwent surgical treatment (84.6%), 56.4% received chemotherapy, and 7.7% received radiation. The 1-, 3-, and 5-y overall survival rates were 87.0%, 62.0%, and 60.1%, respectively. In unadjusted analysis, surgical treatment was associated with improved overall survival (log-rank test, P < 0.001). In multivariable Cox regression, metastasis at diagnosis was associated with inferior overall survival (hazard ratio: 2.72, 95% confidence interval: 1.15-6.40, P = 0.02), when adjusting for age, tumor size, receipt of surgical treatment, and chemotherapy. In patients with nonmetastatic ACC, increasing age was associated with inferior overall survival (hazard ratio: 1.12, 95% confidence interval: 1.00-1.24, P = 0.04), when adjusting for tumor size, receipt of surgical treatment, and chemotherapy. CONCLUSIONS:Most children with ACC in the USA undergo surgical treatment with about half of these also receiving chemotherapy. Metastasis at diagnosis was independently associated with inferior overall survival; in patients with nonmetastatic ACC, increasing age was independently associated with inferior overall survival.