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Malignant melanoma is rare in the pediatric population and management is largely extrapolated from adult guidelines. Adult data have shown that immediate completion lymph node dissection (CLND) does not improve overall survival in selected patients with clinically node negative, sentinel lymph node-positive disease. Current nodal management in children is unknown.The National Cancer Database (NCDB) was queried for patients with melanoma from 2012-2017 and patients categorized as pediatric (≤18 years, n=962) or adult (n=327,987). Factors associated with CLND in children with positive SLNB were evaluated in multivariable analysis. Kaplan-Meier survival analysis was performed.Compared to adults, children present with thicker primary tumors (T3 or T4 26.5% vs 15.5%, p0.001), resulting in higher rates of nodal assessment with SLN biopsy or LND (60.2% vs 36.6%, p0.001) and higher rates of regional nodal disease (35.1% vs 23.4%, p0.001). Children underwent higher rates of CLND after SLN biopsy (10.4% vs 4.1%) and upfront lymph node dissection (15.2% vs 8.7%). A decreased rate of CLND was noted in 2017 compared to 2012 (odds ratio (OR) 0.16 (p=0.005). CLND was performed more often on multivariable analysis for older pediatric age (12 years, OR=1.6, p=0.037) and lower extremity primary (OR=0.29, p0.001). Children with regional nodal disease have improved 3-year overall survival compared to adults (96.5% vs 71.0%, p0.001).Children with melanoma have higher rates of nodal disease but better survival than adults. As in adults, there has been a recent increase in close nodal observation rather than CLND for patients with positive SLN. Further study of nodal surveillance for pediatric patients is warranted.

To assess the preoperative imaging impression and surgeon diagnostic accuracy for pediatric ovarian mature cystic teratomas (MCTs) DESIGN: Retrospective review SETTING: Eleven pediatric hospitals PARTICIPANTS: Patients ages 2 to 21 who underwent surgical management of an ovarian neoplasm or adnexal torsion with an associated ovarian lesion INTERVENTION: None MAIN OUTCOME MEASURES: Preoperative imaging impression, surgeon diagnosis, tumor markers, and pathology RESULTS: Our cohort included 946 females. Final pathology identified 422 (45%) MCTs, 405 (43%) other benign pathologies, and 119 (12%) malignancies. Preoperative imaging impression for MCTs had a 70% sensitivity, 92% specificity, 88% positive predictive value (PPV), and 79% negative predictive value (NPV). For the preoperative surgeon diagnosis, sensitivity was 59%, specificity 96%, PPV 92%, and NPV 74%. Some measures of diagnostic accuracy were affected by the presence of torsion, size of the lesion on imaging, imaging modality, and surgeon specialty. Of the 352 masses preoperatively thought to be MCTs, 14 were malignancies (4%). Eleven patients with inaccurately diagnosed malignancies had tumor markers evaluated and 82% had at least 1 elevated tumor marker, compared with 49% of those with MCTs.Diagnostic accuracy for the preoperative imaging impression and surgeon diagnosis is lower than expected for pediatric ovarian MCTs. For all ovarian neoplasms, preoperative risk assessment including a panel of tumor markers and a multidisciplinary review is recommended. This process could minimize the risk of misdiagnosis and improve operative planning to maximize the use of ovarian-sparing surgery for benign lesions and allow for appropriate resection and staging for lesions suspected to be malignant.

Background Testicular germ cell tumors are uncommon tumors that are encountered by pediatric surgeons and urologists and require a knowledge of appropriate contemporary evaluation and surgical and medical management. Method A review of the recommended diagnostic evaluation and current surgical and medical management of children and adolescents with testicular germ cell tumors based upon recently completed clinical trials was performed and summarized in this article. Results In this summary of childhood and adolescent testicular germ cell tumors, we review the initial clinical evaluation, surgical and medical management, risk stratification, results from recent prospective cooperative group studies, and clinical outcomes. A summary of recently completed clinical trials by pediatric oncology cooperative groups is provided, and best surgical practices are discussed. Conclusions Testicular germ cell tumors in children are rare tumors. International collaborations, data-sharing, and enrollment of patients at all stages and risk classifications into active clinical trials will enhance our knowledge of these rare tumors and most importantly improve outcomes of patients with testicular germ cell tumors. Level of evidence This is a review article of previously published and referenced level 1 and 2 studies, but also includes expert opinion level 5, represented by the American Pediatric Surgical Association Cancer Committee.

Despite increased utilization of robotic-assisted surgery in the pediatric population during the past decade, reports of comparative analysis between robotic surgery and laparoscopic surgery are lacking. Our aim was to evaluate outcomes between pediatric robotic-assisted cholecystectomy (RC) and laparoscopic cholecystectomy (LC).A single institution retrospective analysis of 299 patients undergoing either RC or LC, between January 2015 and December 2018 was performed. Demographic data as well as clinical characteristics and related outcomes were abstracted and compared using univariate analysis. Related hospital costs were estimated using a charge to cost methodology.The median age of the cohort was 15.5 years (IQR 14.0-17.0); 76% females and 70% white, with 74% (n = 220) undergoing LC and 26% (n = 79) undergoing RC. The majority of RC were performed using single-site technique and RC proportion increased with time (10% in 2015 vs. 41% in 2018, p0.001). The majority of RC were more commonly attributed to patients with nonacute indications for cholecystectomy compared to acute clinical indications (87% vs. 13%). Median operative time was 98 min vs. 79 min for RC and LC respectively (p0.001). Median postoperative LOS was similar between groups (22 h). There were no significant differences in postoperative complication, in-hospital opioid utilization and 30-day readmissions. Average total hospital costs for RC were $15,519 compared to $11,197 for LC.Pediatric robotic-assisted cholecystectomy is feasible with similar outcomes compared to laparoscopic cholecystectomy. However, it is associated with longer operative times and higher costs. The single-site RC technique may provide a potential cosmetic benefit.

Background Management of ovarian torsion has evolved toward ovarian preservation regardless of ovarian appearance during surgery. However, patients with torsion and an ovarian neoplasm undergo a disproportionately high rate of oophorectomy. Our objectives were to identify factors associated with ovarian torsion among females with an ovarian mass and to determine if torsion is associated with malignancy. Methods A retrospective review of females aged 2-21 y who underwent an operation for an ovarian cyst or neoplasm between 2010 and 2016 at 10 children's hospitals was performed. Multivariate logistic regression was used to assess factors associated with torsion. Imaging data were assessed for sensitivity, specificity, and predictive value in identifying ovarian torsion. Results Of 814 girls with an ovarian neoplasm, 180 (22%) had torsion. In risk-adjusted analyses, patients with a younger age, mass size >5 cm, abdominal pain, and vomiting had an increased likelihood of torsion (P 5 cm had two times the odds of torsion (odds ratio: 2.1; confidence interval: 1.2, 3.6). Imaging was not reliable at identifying torsion (sensitivity 34%, positive predictive value 49%) or excluding torsion (specificity 72%, negative predictive value 87%). The rates of malignancy were lower in those with an ovarian mass and torsion than those without torsion (10% versus 17%, P = 0.01). Among the 180 girls with torsion and a mass, 48% underwent oophorectomy of which 14% (n = 12) had a malignancy. Conclusions In females with an ovarian neoplasm, torsion is not associated with an increased risk of malignancy and ovarian preservation should be considered.

Describe the current practice patterns and diagnostic accuracy of frozen section (FS) pathology for children and adolescents with ovarian masses DESIGN: Prospective cohort study from 2018 to 2021 SETTING: Eleven children's hospitals PARTICIPANTS: Females age 6-21 years undergoing surgical management of an ovarian mass INTERVENTIONS: Obtaining intraoperative FS pathology MAIN OUTCOME MEASURE: Diagnostic accuracy of FS pathology RESULTS: Of 691 patients who underwent surgical management of an ovarian mass, FS was performed in 27 (3.9%), of which 9 (33.3%) had a final malignant pathology. Among FS patients, 12 of 27 (44.4%) underwent ovary-sparing surgery, and 15 of 27 (55.5%) underwent oophorectomy with or without other procedures. FS results were disparate from final pathology in 7 of 27 (25.9%) cases. FS had a sensitivity of 44.4% and specificity of 94.4% for identifying malignancy, with a c-statistic of 0.69. Malignant diagnoses missed on FS included serous borderline tumor (n = 1), mucinous borderline tumor (n = 2), mucinous carcinoma (n = 1), and immature teratoma (n = 1). FS did not guide intervention in 10 of 27 (37.0%) patients: 9 with benign FS underwent oophorectomy, and 1 with malignant FS did not undergo oophorectomy. Of the 9 patients who underwent oophorectomy with benign FS, 5 (55.6%) had benign and 4 (44.4%) had malignant final pathology.FSs are infrequently utilized for pediatric and adolescent ovarian masses and could be inaccurate for predicting malignancy and guiding operative decision-making. We recommend continued assessment and refinement of guidance before any standardization of use of FS to assist with intraoperative decision-making for surgical resection and staging in children and adolescents with ovarian masses.

Introduction Laparoscopic splenectomy (LS) is the standard of care for hematologic disorders requiring splenectomy. Less is known about the outcomes following robotic-assisted splenectomy (RS) for this indication. Our aim was to describe outcomes of RS to LS in pediatric patients with hematologic disorders in our institution. Methods A single institution retrospective review was performed of pediatric patients undergoing LS vs. RS from 2014 to 2019. Patient demographics, diagnosis, spleen size, hospital length of stay (LOS), operative time, post-operative opioid use, and hospital charges were evaluated. Standard univariate analyses were performed. Results Twenty-four patients were included in the study (14 LS, 10 RS). The mean spleen size at the time of surgery was larger in the RS group compared to LS (14.5 cm vs. 12.2 cm, p = 0.03). Operative time between the two cohorts was comparable (RS 140.5 vs LS 154.9 min). Median LOS for RS was shorter than LS (2.1 vs. 3.2 days, p = 0.02). Cumulative postoperative opioid analgesic requirements were not significantly different between the groups (17.4 mg vs. 30.5 mg). The median hospital charges, including the surgical procedure and hospital stay were higher in the RS group ($44,724 RS vs $30,255 LS, p = 0.01). Conclusion Robotic splenectomy is a safe and feasible option for pediatric patients with hematologic disorders, and was associated with decreased LOS but higher charges compared to laparoscopic splenectomy. Further studies are required to delineate the optimal use and potential benefits of robot-assisted surgical techniques in children. Level of Evidence II.

Melanoma is the most common skin cancer in children, often presenting in an atypical fashion. The incidence of melanoma in children has been declining. The mainstay of therapy is surgical resection. Sentinel lymph node biopsy often is indicated to guide therapy and determine prognosis. Completion lymph node dissection is recommended in selective cases after positive sentinel lymph node biopsy. Those with advanced disease receive adjuvant systemic treatment. Because children are excluded from melanoma clinical trials, management is based on pediatric retrospective data and adult clinical trials. This review focuses on epidemiology, presentation, surgical management, adjuvant therapy, and outcomes of pediatric melanoma.

Objective 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. Summary background data 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. Methods 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 2008-2017 was performed. Patient-reported quality of life was assessed prospectively using PROMIS surveys. Results 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 (IQR = 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 posterior rib 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 following chest wall tumor resection. Conclusions 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.

Minimally invasive nephrectomy is performed routinely for adult renal tumors and for many benign pediatric conditions. Although open radical nephroureterectomy remains the standard of care for Wilms tumor and most pediatric renal malignancies, there are an increasing number of reports of minimally invasive surgery (MIS) for those operations as well. The APSA Cancer Committee performed a systematic review to better understand the risks and benefits of MIS in pediatric patients with renal tumors. Methods The search focused on MIS for renal tumors in children and followed the Preferred Reporting Items for Systematic Review and Meta-Analysis (PRISMA) checklist. The initial database search identified 491 published articles, and after progressive review of abstracts and full-length articles, 19 were included in this review. Results There were two direct comparison studies where open surgery and MIS were compared. The remaining studies reported only on minimally invasive nephrectomy. Across all studies, there were a total of 151 patients, 126 of which had Wilms tumor and 10 patients had RCC. 104 patients had MIS, with 47 patients having open surgery. In the two studies in which open surgery and MIS were directly compared, more lymph nodes were harvested during open surgery (median = 2 (MIS) vs 5 (open); mean = 2.47 (MIS) vs 3.8 (open)). Many noncomparison studies reported the harvest of 2 of fewer lymph nodes for Wilms tumor. Several MIS patients were also noted to have intraoperative spill or positive margins. Survival between groups was similar. Conclusions There is a lack of evidence to support MIS for pediatric renal tumors. This review demonstrates that lymph node harvest has been inadequate for MIS pediatric nephrectomy and there appears to be an increased risk for intraoperative spill. Survival data are similar between groups, but follow-up times were inconsistent and patient selection was clearly biased, with only small tumors being selected for MIS. Type of study Review article. Level of evidence III.

Background Minimally invasive surgery has broad applicability to pediatric diseases, including pediatric cancer resection. Neuroblastic tumors of childhood are highly variable in presentation, and so careful selection of appropriate candidates for minimally invasive resection is paramount to achieving safe and durable surgical and oncological outcomes. Methods The American Pediatric Surgical Association Cancer Committee developed questions seeking to better define the role of minimally invasive surgery for neuroblastic tumors. A search using PubMed, Medline, Embase, Web of Science, ProQuest Dissertations, and Clinical Trials was performed for articles published from 1998 to 2018 in accordance with the Preferred Reporting Items for Systematic Review and Meta-Analysis Protocols (PRISMA-P) guidelines. Results The evidence identified is all retrospective in nature. Minimally invasive surgical resection of neuroblastic tumors is safe for carefully selected smaller (4-6 cm) image defined risk factor (IDRF)-negative abdominal tumors when oncologic principles are followed. Size is a less-well defined criterion for thoracic neuroblastic tumors. Open approaches for both abdominal and thoracic tumors may be preferable in the presence of IDRF's. Conclusion Small tumors without IDRF's are reasonable candidates for minimally invasive resection. Surgical oncologic guidelines should be closely followed. The quality of data supporting this systematic review is poor and highlights the need for refinement in the study of such surgical techniques to improve knowledge and outcomes for patients with neuroblastic tumors. Type of Study Systematic Review. Level of Evidence Level III and Level IV.

We describe a rare pediatric case of a phalangeal giant cell tumor of bone with extensive bilateral lung metastases following curettage, wide resection, and amputation. Concurrent peripheral blood eosinophilia and pleural effusion with marked eosinophilia (47%) were present. To discover genetic changes driving tumor metastasis, genomic and transcriptome profiling of the metastatic lung mass as well as germline analysis were performed. Whole exome sequencing detected a histone H3F3A p.G35V missense mutation in tumor cells. RNA sequencing revealed overexpression of receptor activator of nuclear factor kappa-B ligand (RANKL). The patient is alive with no residual disease and uncompromised respiratory function 29 months after amputation of primary tumor and 19 months after surgical resection of his metastatic lung disease.

Background/Purpose Rhabdomyosarcoma is the most common soft tissue sarcoma in children and young adults and requires multimodality treatment. The purpose of this review is to present an update on risk stratification as well as surgical and medical management strategies in pediatric rhabdomyosarcoma. Methods A comprehensive review of the current literature on pediatric rhabdomyosarcoma, including the most recent Children's Oncology Group studies and several international collaboratives, was performed by the authors and key findings were summarized in the manuscript. Results FOXO1 fusion status is a stronger prognostic factor than histology and is now used for risk stratification in treatment protocols. For assessment of regional nodal involvement, FDG-PET-CT shows poor sensitivity and specificity to detect histologically confirmed nodal metastasis. Thus, surgical assessment of regional lymph nodes is required for rhabdomyosarcoma of the extremities or trunk as well as paratesticular rhabdomyosarcoma in patients ≥ 10 years of age, although adherence to surgical guidelines remains poor. Hemiscrotectomy performed for scrotal violation in paratesticular rhabdomyosarcoma has not shown an improvement in event free survival and is not recommended. Conclusions Surgical and medical treatment strategies for rhabdomyosarcoma in children continue to evolve. This review provides current evidence-based treatment standards with an emphasis on surgical care. Type of Study Review. Level of Evidence Level IV.

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.

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.

Pulmonary 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.A 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.225 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).Many 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.Retrospective review, Level 3.

Background: Video-assisted thoracoscopic surgery (VATS) with pulmonary apical wedge resection is the mainstay procedure performed for spontaneous pneumothorax (sPTX). However, there is variability ...

Neonates with omphaloceles routinely have a transthoracic echocardiogram (TTE) performed due to a high association with congenital heart defects (CHD). The utility of a TTE in these patients with a normal fetal echocardiogram is unknown. The primary objective of this study was to determine whether a critical CHD diagnosis was missed in patients with an omphalocele who had a normal fetal echocardiogram. The secondary objective of the study was to determine whether any CHD diagnosis was missed postnatally when a fetal echocardiogram was read as normal. A retrospective chart review was performed of patients with omphaloceles born between January 1, 2008, and June 30, 2020. Patients were included if they had a fetal echocardiogram that was read as normal and had a postnatal echocardiogram performed. Baseline demographics, postnatal data echocardiographic findings, and hospital course were collected. Critical CHD was defined as CHD requiring neonatal cardiac intervention. Fifty-six fetal echocardiograms on patients with omphaloceles were performed, of which 24 patients met the inclusion criteria. No patient was diagnosed with a critical CHD postnatally (negative predictive value [NPV] = 100%). Two patients were diagnosed with ventricular septal defects (VSD) postnatally (NPV = 91.7%). One of the VSDs required closure with a patch at 4 months of life, while the other, a small muscular VSD, closed spontaneously within the first year of life. Both patients had a murmur on exam during their initial hospital stay. The patient that required surgery also had an abnormal electrocardiogram and chest X-ray. There were no mortalities due to cardiac causes in these patients. Critical CHD was not missed on any patient with an omphalocele who had a normal fetal echocardiogram. All other patients with omphaloceles who had CHD diagnosed postnatally had an abnormal clinical finding on postnatal evaluation. The routine performance of a postnatal TTE in patients with an omphalocele who had a normal fetal echocardiogram may not be needed in those with a normal clinical workup. Further studies evaluating echocardiographic imaging recommendations are needed to maximize care and optimize resource allocation in this complex patient population.

Purpose Current investigational priorities in the treatment of favorable histology Wilms tumor (FHWT) center on accurate staging and risk-stratification. The extent of lymph node (LN) sampling has not been clearly defined; its importance cannot be overstated as it guides adjuvant therapy. The identification of a minimum LN yield to minimize the risk of harboring occult metastatic disease could help development of surgical guidelines. This study focuses on using the beta-binomial distribution to estimate the risk of occult metastatic disease in patients with FHWT. Materials & methods The National Cancer Database was queried for patients with unilateral FHWT from 2004 to 2013. Data were used to characterize nodal positivity for patients who underwent surgery and had ≥ 1 positive LN and ≥ 2 LNs examined. The probability of missing a positive LN (i.e., false negative) for a given LN yield was calculated using an empirical estimation and the beta-binomial model. Patients were then stratified by tumor size. Results 422 patients met study criteria. To limit the chance of missing a positive LN to ≤ 10%, the empirical estimation and beta-binomial model estimated that 6 and 10 LNs needed to be sampled, respectively. Tumor size did not influence the result. Internal validation showed little variation to maintain a false negative rate ≤ 10%. Conclusions Using mathematical modeling, it appears that the desired LN yield in FHWT to reduce the risk of false-negative LN sampling to ≤ 10% is between 6 and 10. The current analysis represents an objective attempt to determine the desired surgical approach to LN sampling to accurately stage patients with FHWT. Level of evidence II

Background Despite variability at presentation, presacral masses in patients with and without anorectal malformations (ARM) appear histologically similar. The purpose of this study was to identify differences in oncologic outcomes between these two groups. Methods A retrospective review was performed utilizing our institutional cancer and colorectal and pelvic reconstruction databases for patients with presacral masses and sacrococcygeal teratomas between 1990 and 2017. Data captured included age at surgical resection, type of ARM, tumor location within the pelvis, tumor histopathology, tumor size, adjuvant chemotherapy, recurrence, and follow-up. Results Forty-six patients comprised our cohort, of whom 12 had an ARM. The median age was older at resection for those with an ARM (1.4 years; range 1 day to 29.4 years) compared to those without an ARM (9 days; range 0 days to 6.9 years) (p = 0.01). The mean tumor size was 2.5 cm in patients with an ARM compared to 6.0 cm in patients without an ARM (p = 0.036). All patients with ARM had exclusively intrapelvic tumors, and histopathology included mature teratoma (8), yolk sac tumor (1), lipoma (1), and unknown (2). Tumor location for patients with sacral and presacral masses without ARM included exclusively extrapelvic (10), primarily extrapelvic with large intrapelvic component (7), primarily intrapelvic with extrapelvic component (1), exclusively intrapelvic (8), and unknown (8). Histopathology for patients with presacral masses without ARM included mature teratoma (20), immature teratoma (7), yolk sac tumor (3), ganglioneuroma (1), neuroblastoma (1), benign epithelial cyst (1), and unknown (1). Tumor recurrence rate was similar between patients with ARM (n = 3, 25%) and those without an ARM (n = 5, 15%) (p = 0.41). The 5-year event free survival was 65% (95% CI: 25%–87%) in the group with ARM and 81% (95% CI: 60%–92%) in the group without ARM (p = 0.44). Conclusion Sacral and presacral masses in patients with ARM are resected at a later age and are more likely to be intrapelvic. They appear histologically similar and have similar rates of recurrence and malignancy when compared to patients without ARM. Level of Evidence III Type of Study Retrospective comparative study.

This article reviews of the current evidence-based treatment standards for children with Wilms tumor. In this article, a summary of recently completed clinical trials by the Children’s Oncology Group are provided, the current diagnostic evaluation and surgical standards are discussed, and the surgical impact on current risk stratification for patients with Wilms tumor is highlighted. LEVEL OF EVIDENCE: This is a review article of previously published and referenced LEVEL 1 studies, but also includes expert opinion LEVEL V, represented by the American Pediatric Surgical Association Cancer Committee.

Gastroblastomas are rare tumors with a biphasic epithelioid/spindle cell morphology that typically present in early adulthood and have recurrent MALAT1-GLI1 fusions. We describe an adolescent patient with Wiskott-Aldrich syndrome who presented with a large submucosal gastric tumor with biphasic morphology. Despite histologic features consistent with gastroblastoma, a MALAT1-GLI1 fusion was not found in this patient's tumor; instead, comprehensive molecular profiling identified a novel EWSR1-CTBP1 fusion and no other significant genetic alterations. The tumor also overexpressed NOTCH and FGFR by RNA profiling. The novel fusion and expression profile suggest a role for epithelial-mesenchymal transition in this tumor, with potential implications for the pathogenesis of biphasic gastric tumors such as gastroblastoma.

Purpose/Objective(s) Pediatric patients with metastatic solid tumors historically have a poor overall survival. Some pediatric patients may still be potentially curable with aggressive local therapy to metastatic disease, and adult trials have defined a group of oligometastatic patients, where the use of stereotactic body radiation therapy (SBRT) has an overall survival benefit. The purpose of this study is to report results of the use of SBRT in the treatment of pediatric metastatic disease. Materials/Methods All pediatric and adolescents and young adult (AYA) patients age 3-40 years who received SBRT were included between the years 2000-2020. SBRT included radiation treatments with 3-5 fractions and ablative doses above conventional palliative radiation. Study endpoints included local control (LC), progression free survival (PFS), overall survival (OS), cumulative incidence (CI) of death or local failure and toxicity. The endpoints with respect to survival and LC were calculated using the Kaplan-Meier estimate. The cumulative incidence of local failure was calculated using death as a competing risk. Toxicity was scored using CTCAE v5.0. Results Thirty-six lesions in 16 patients were treated with SBRT and comprised the overall cohort. The most common pediatric solid tumors treated were Ewing's Sarcoma (n = 3), Osteosarcoma (n = 3), and Rhabdomyosarcoma (n = 2). The majority of lesions irradiated were in the spine (n = 11), lung (n = 8), pelvis (n = 7), and brain (n = 5). The median follow up was 12 months. The median OS and PFS for the entire cohort were 17 months and 15.7 months, respectively. Patients were treated to a median dose of 25 Gy in 5 fractions. The 1-year OS for the entire cohort was 75%. The 6- and 12-month local control was 85% and 78%, respectively. There were no local failures in irradiated lesions for patients who received a BED10≥100 Gy. Patients treated with SBRT who had ≤5 metastatic lesions at first recurrence had a superior 1-year OS of 100% versus 50% for those with > 5 lesions. No patients experienced a grade 4 or 5 toxicity in our cohort. One patient (6.3%) experienced a CTCAE grade 3 toxicity (seizure) which resolved after anti-epileptic medications. Conclusion Local control was excellent with SBRT delivered to metastatic disease, particularly for lesions receiving a BED10≥100 Gy. High-grade toxicity was rare in our patient population. Patients with 5 or fewer metastatic sites have a significantly better OS compared to > 5 sites. Future prospective trials with multi-institutional collaboration will be necessary to evaluate appropriate patient selection and the optimal radiation dose regimen.

Background/Purpose Cancer predisposition syndromes (CPS) are a heterogeneous group of inherited disorders that greatly increase the risk of developing malignancies. CPS are particularly relevant to pediatric surgeons since nearly 10% of cancer diagnoses are due to inherited genetic traits, and CPS often contribute to cancer development during childhood. Materials/Methods The English language literature was searched for manuscripts, practice guidelines, and society statements on “cancer predisposition syndromes in children”. Following review of these manuscripts and cross-referencing of their bibliographies, tables were created to summarize findings of the most common CPS associated with surgically treated pediatric solid malignancies. Results Pediatric surgeons should be aware of CPS as the identification of one of these syndromes can completely change the management of certain tumors, such as WT. The most common CPS associated with pediatric solid malignancies are outlined, with an emphasis on those most often encountered by pediatric surgeons: neuroblastoma, Wilms’ tumor, hepatoblastoma, and medullary thyroid cancer. Frequently associated non-tumor manifestations of these CPS are also included as a guide to increase surgeon awareness. Screening and management guidelines are outlined, and published genetic testing and counseling guidelines are included where available. Conclusion Pediatric surgeons play an important role as surgical oncologists and are often the first point of contact for children with solid tumors. In their role of delivering a diagnosis and developing a follow-up and treatment plan as part of a multidisciplinary team, familiarity with common CPS will ensure evidence-based practices are followed, including important principles such as organ preservation and intensified surveillance plans. This review defines and summarizes the CPS associated with common childhood solid tumors encountered by the pediatric surgeon, as well as common non-cancerous disease stigmata that may help guide diagnosis. Type of study Summary paper. Level of evidence 5.

Sacrococcygeal masses (SCM) are uncommon in children. The purpose of this study is to review the functional fecal and urinary outcomes following resection of SCM and to determine the impact of a multidisciplinary clinic (MDC) on these outcomes.A retrospective review was performed of patients who underwent SCM resection between 1979 and 2019. Baylor Social Continence Scale (BCS), Vancouver Symptom Score (VSS) and Cleveland constipation score (CSS) surveys were used to assess fecal and urinary continence at time of most recent follow up. Age, tumor characteristics, histopathology, and type of anorectal malformations (ARM), if present, were also recorded.75 patients were included. 51 (69%) patients were females and 23 (31%) had an associated ARM. The median age at resection was 8.5 months (IQR 0-26.8). 41 (56%) patients were followed in the MDC. 27 (82%) of patients seen in the MDC were clean for stool and 26 (87%) were dry for urine, while only 17 (59%) of patients not seen in the MDC were clean for stool and dry for urine (p0.05). There was improvement in Baylor, Vancouver and Cleveland scores.A multidisciplinary approach to the care of patients following SCM resection may improve bowel and bladder outcomes.

Thyroid Imaging Reporting and Data System (TI-RADS) is validated in adults but not yet in children. The purpose of this study was to determine the sensitivity, specificity, and accuracy of TI-RADS in predicting thyroid malignancy for pediatric nodules, and to compare the diagnostic accuracy to the current American Thyroid Association (ATA) guidelines.A single institution retrospective review was performed of patients younger than 21 years who underwent thyroid nodule fine needle aspiration biopsy (FNAB). Two radiologists were blinded to the pathology and independently classified all biopsied thyroid nodules based on TI-RADS. ATA and TI-RADS guidelines were analyzed to determine the diagnostic sensitivity and specificity of both scoring systems.115 patients (median age 15.5 years, 90 females) with 138 nodules were scored using TI-RADS. There was moderate inter-rater agreement between radiologists (Kappa = 0.51; p 0.0001). Evaluating several potential TI-RADS criteria, 23.2%-68.1% of nodules were recommended for FNAB, compared to 82.6% of nodules using ATA guidelines. Using TI-RADS ≥ 3 (without size cutoff) as an indication for FNAB had 100% sensitivity with no missed suspicious or malignant nodules on cytology or pathology.Using TI-RADS for diagnostic management of pediatric thyroid nodules improves accuracy in predicting malignancy.

Button batteries (BBs) are found in many households and are a source of esophageal foreign body in the pediatric population. Upon ingestion, significant caustic injury can occur within 2 hours leading to tissue damage and severe, potentially fatal sequelae. Aortoesophageal fistula (AEF) is a rare complication that nearly always results in mortality. We report a rare case of a toddler who developed an AEF after BB ingestion and survived following staged aortic repair. There should be a high index of suspicion for this complication with the history of BB ingestion and presence of hematemesis, hemoptysis, or melena.

Purpose The objective of this quality improvement (QI) initiative was to implement a standardized clinical treatment protocol for patients presenting with primary spontaneous pneumothorax (PSP) in order to decrease hospital length of stay (LOS), diagnostic radiation exposure, and related cost. Methods Baseline data from patients admitted with PSP from January 1, 2016 to July 31, 2018 were compared to data from patients managed using a newly developed evidence-based treatment pathway from August 1, 2018 to December 31, 2019. Standard QI methodology was used to track results. Results Fifty-six episodes of PSP were observed during the baseline period and 40 episodes of PSP following initiation of the PSP protocol. The average LOS decreased from 4.5 days to 2.9 days. Patients underwent an average of 8.8 X-rays per admission preintervention versus 5.9 postintervention. The rate of CT scans decreased from 45% to 15% (p = 0.002). There was no significant difference in the rates of 30-day recurrence between the preintervention (13%) and postintervention (10%) groups (p = 0.7). Average admission costs per patient decreased by $1322 after adoption of the pathway. Conclusions Adoption of a standardized treatment protocol for PSP led to a reduction in LOS, diagnostic imaging utilization, and cost without increasing clinical recurrence. Type of study Quality improvement. Level of evidence Level III.

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

Background/purpose Lymphadenopathy is a common complaint in children. Pediatric surgeons are often called upon to evaluate, treat, and/or biopsy enlarged lymph nodes. With many nonsurgical causes in the differential diagnosis, the surgeon plays the important role of providing reassurance and timely diagnosis while minimizing the pain and morbidity associated with surgical interventions in children. The purpose of this summary paper is to provide a management guide for surgeons working up children with lymphadenopathy. Materials/methods The English language literature was searched for “lymphadenopathy in children”. All manuscript types were considered for review, regardless of medical specialty, with emphasis placed on published guidelines, algorithms, and reviews. After thorough review of these manuscripts and cross-referencing of their bibliographies, the attached algorithm was developed, with emphasis on the role and timing of surgical intervention. Results The APSA Cancer Committee developed the attached algorithm to fill a gap in the surgical literature. It outlines lymphadenopathy workup and treatment with emphasis on the role and timing of surgical intervention. Conclusion This review defines and summarizes the common etiologies and presentations of lymphadenopathy in children, and offers a straightforward algorithm for evaluation of and treatment with an emphasis on malignancy risk and surgical management. Type of study Summary paper. Level of evidence Level V.

Background Failure to rescue (FTR), mortality after a major postoperative complication, is a superior surgical quality metric compared to surgical mortality or complications rates alone. Our objective was to develop and validate a novel pediatric profiling to identify high-risk subjects among the subset of children who develop serious post-operative complications. Methods We performed a retrospective study of children who developed one or more serious postoperative complications following inpatient surgery across NSQIP-Pediatric hospitals (2012–2017). We evaluated the rate of FTR according to pre-operative comorbidity burden. Results We identified 45,504 surgical cases with major post-operative complications (FTR rates: 2.4%). Surgical cases with greater than six pre-operative comorbidities (n = 12,148;28%) accounted for 80% of FTR events. The expected probability of FTR was 0.1%(95%CI:0.1%–0.2%) among low-risk cases, 3.3%(95%CI:3.0%–3.5%) among intermediate-risk cases, and 22.6%(95%CI:20.9%–24.3%) among high-risk cases. About half of surgical cases in the high-risk profile group died within 48 h of surgery. Comparatively, cases in the intermediate-risk group had a much longer time to mortality (10 days). Conclusion We propose a prognostic index to accurately identify children at risk for FTR. The use of such an index may provide surgeons with a window of opportunity to implement aggressive monitoring and therapeutic strategies to reduce mortality. Level of evidence IV

Background: Image-guided percutaneous core needle biopsy (PCNB) is increasingly utilized to diagnose solid tumors. The objective of this study is to determine whether PCNB is adequate for modern biologic characterization of neuroblastoma. Procedure: A multi-institutional retrospective study was performed by the Pediatric Surgical Oncology Research Collaborative on children with neuroblastoma at 12 institutions over a 3-year period. Data collected included demographics, clinical details, biopsy technique, complications, and adequacy of biopsies for cytogenetic markers utilized by the Children's Oncology Group for risk stratification. Results: A total of 243 children were identified with a diagnosis of neuroblastoma: 79 (32.5%) tumor excision at diagnosis, 94 (38.7%) open incisional biopsy (IB), and 70 (28.8%) PCNB. Compared to IB, there was no significant difference in ability to accurately obtain a primary diagnosis by PCNB (95.7% vs 98.9%, P =.314) or determine MYCN copy number (92.4% vs 97.8%, P =.111). The yield for loss of heterozygosity and tumor ploidy was lower with PCNB versus IB (56.1% vs 90.9%, P

Summary Background Sacrococcygeal teratoma (SCT) is the most commonly occurring presacral tumor and can affect the lower urinary tract by direct mass effect, or as a sequela from surgical resection. Despite the potential impact on urologic function, there is no current standard for urological involvement or follow-up for these patients. The purpose of this study was to evaluate the need for urologic involvement for lower urinary tract dysfunction (LUTD) in patients with SCTs at our institution. Methods We performed a retrospective chart review of patients diagnosed with SCT and managed at our institution between 1990 and 2019. Data collected included: patient demographics, surgical and pathology reports, presence of tethered cord or anorectal malformation, and need for urologic involvement for LUTD. LUTD included acute urinary retention, need for chronic intermittent catheterization, and/or urinary incontinence. Acute urinary retention was defined as requiring catheterization to empty the bladder for a limited time (outside the standard post-operative indwelling catheter time period) and the eventual return to spontaneous voiding. Chronic intermittent catheterization was defined as those with urinary retention that has persisted and required continued catheterization at the time of chart review. Urinary incontinence was defined as urine leakage in children older than 3 years of age. Patients with unavailable records were excluded. Comparison between groups was performed with Mann Whitney and chi-squared tests. Results Forty-five patients with SCTs were identified. LUTD was identified in 23 patients (51%). The most common reason for LUTD was urinary retention (n = 16, 70%): 9 patients had acute retention and 7 had chronic retention (Fig. 1). Nine patients (39%) had urinary incontinence: 2 of these patients (4% of all SCT patients) had urinary fistulas (vesicovaginal (n = 1) and urethrovaginal (n = 1)). Only 5 patients (22%) had LUTD recognized preoperatively. Nine patients had concomitant tethered cord, and 7 of these (78%) had LUTD. Of patients with LUTD, Altman type IV was the most common location (n = 10, 43%). There was no significant difference in tumor type between those with and without LUTD. Patients without LUTD had significantly shorter follow-up. Conclusions Greater than 50% of patients with SCTs have known LUTD. Two of these patients were found to have urinary fistulas requiring urinary diversion. A multidisciplinary team including urology should be involved upfront in the management of patients with SCTs, and LUTD should be routinely assessed at follow-up visits. Evaluation for a urinary fistula should occur in the presence of urinary incontinence. Download : Download high-res image (150KB) Download : Download full-size image

Peptic ulcer disease (PUD) is not common in children but is a recognized cause of recurrent abdominal pains. Despite the current effectiveness of medical treatment, diagnosis may not be made until complicated by bleeding, perforation, or gastric outlet obstruction, making surgical intervention necessary. A high index of suspicion is necessary for early recognition and prompt treatment.