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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.

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.

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.

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.

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.

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.

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.

Background Atypical teratoid/rhabdoid tumor (AT/RT) of the central nervous system (CNS) with synchronous or metachronous extra-CNS disease is a rare childhood malignancy with a dismal prognosis. Case Description We report a 7-week-old female with metastatic AT/RT and synchronous malignant rhabdoid tumor of the kidney who received an intensive multimodal approach combining surgical resection, intrathecal chemotherapy, and high-dose chemotherapy with autologous peripheral blood stem cell transplant (PBSCT). She is currently 24 months old without any evidence of disease. In addition, we completed an extensive literature review of cases with CNS AT/RT and synchronous or metachronous extra-CNS primary tumors. To date, 31 pediatric cases have been reported, and the median overall-survival was 6 months after diagnosis. The only 3 survivors received autologous PBSCT, and 2 of these patients had complete resection of their CNS tumor. Conclusions The rarity of CNS AT/RT with extra-CNS primary disease and the lack of standard treatment contribute to its reported dismal prognosis. We report a case of a long-term survivor with metastatic AT/RT and synchronous extra-CNS primary tumor. Maximal surgical resection, intrathecal chemotherapy, and consolidative autologous PBSCT may improve prognosis and avoid radiation.

BACKGROUND Osteosarcoma (OS) and Ewing sarcoma (ES) have a high propensity to develop pulmonary metastases. Lung lesions with calcification, peripheral location, and size >5 mm are more likely to represent malignant metastases. We evaluated the incidence of malignancy in nodules 5 mm or less to potentially guide decisions between biopsy and observation. MATERIALS AND METHODS A retrospective review of patients

Background Differentiated thyroid carcinomas (DTCs) are rare in young children but represent almost 10% of all malignancies diagnosed in older adolescents. Methods This article reviews the recent literature describing surgical therapeutic approaches to pediatric DTC, associated complications, and long-term recurrence and survival outcomes. Results Similar to adult thyroid cancers, pediatric DTCs are more common in females and are associated with thyroid nodules, family history of thyroid cancer, radiation exposure, iodine deficiency, autoimmune thyroid disease, and genetic syndromes. Management of thyroid cancers in children involves ultrasound imaging, fine needle aspiration, and surgical resection with treatment decisions based on clinical and radiological features, cytology and risk assessment. Conclusions Total thyroidectomy and compartment based resection of clinically involved lymph node basins form the cornerstone of treatment of DTC. There is an evolving literature regarding the use of molecular genetics to inform treatment strategies and the use of targeted therapies to treat iodine refractory and surgically unresectable progressive disease. Type of study Summary review. Level of evidence This is a review article of previously published Level 1–5 articles that includes expert opinion (Level 5).

Purpose The purpose of this study was to determine the diagnostic accuracy of tumor markers for malignancy in girls with ovarian neoplasms. Methods A retrospective review of girls 2–21 years who presented for surgical management of an ovarian neoplasm across 10 children's hospitals between 2010 and 2016 was performed. Patients who had at least one concerning feature on imaging and had tumor marker testing were included in the study. Sensitivity, specificity, and negative and positive predictive values (PPV) of tumor markers were calculated. Results Our cohort included 401 patients; 22.4% had a malignancy. Testing for tumor markers was inconsistent. AFP had high specificity (98%) and low sensitivity (42%) with a PPV of 86%. The sensitivity, specificity, and PPV of beta-hCG was 44%, 76%, and 32%, respectively. LDH had high sensitivity (95%) and Inhibin A and Inhibin B had high specificity (97% and 92%, respectively). Conclusions Tumor marker testing is helpful in preoperative risk stratification of ovarian neoplasms for malignancy. Given the variety of potential tumor types, no single marker provides enough reliability, and therefore a panel of tumor marker testing is recommended if there is concern for malignancy. Prospective studies may help further elucidate the predictive value of tumor markers in a pediatric ovarian neoplasm population. Type of Study Retrospective Cohort Review. Level of Evidence Level III.

Stage I epithelial-predominant favorable-histology Wilms tumors (EFHWTs) have long been suspected to have an excellent outcome. This study investigates the clinical and pathologic features of patients with stage I EFHWTs to better evaluate the potential for a reduction of chemotherapy and its associated toxicity.All patients registered in the Children's Oncology Group (COG) AREN03B2 study between 2006 and 2017 with stage I EFHWTs were identified. EFHWTs were defined as tumors with at least 66% epithelial differentiation, regardless of the degree of differentiation. Clinical information was abstracted from COG records. Event-free survival (EFS) and overall survival (OS) were calculated and compared between groups based on age and therapy.The 4-year EFS rate was 96.2% (95% confidence interval, 92%-100%), and the OS rate was 100%; EFS and OS did not statistically significantly differ with the age at diagnosis (48 vs ≥48 months; P = .37) or treatment (EE4A vs observation only; P = .55). Six events were reported. Three patients developed contralateral tumors and did not otherwise relapse; none of these had nephrogenic rests or a recognized predisposition syndrome. Three patients developed metastatic recurrence; all 3 had received EE4A as their primary therapy after nephrectomy.These findings demonstrate an excellent outcome for stage I EFHWTs with95% EFS and OS. These data support the utility of investigating the treatment of stage I EFHWTs with observation alone after nephrectomy.

Renal masses are most common in children between ages 1 to 3 years, with less known about renal tumors in older children and young adults. The aim of this study was to review the presentation, demographics, histology, and outcomes in patients over 5 years of age with renal tumors compared with younger children. 111 renal tumors were diagnosed in patients 5 years of age and older (median, 7 y; range, 5 to 31 y) between 1950 and 2017 at a single institution. Wilms tumor (WT) was the most common histology in 84 patients (75%), followed by renal cell carcinoma in 12 patients (10.7%). Abdominal pain was the most common presenting symptom (46%) followed by hematuria (28.8%), and a palpable abdominal mass (24.3%). For WT, older children more commonly presented with advanced-stage disease (stages 3 and 4) than younger children (57.7% vs. 11.5%; P

Background/Purpose Melanoma is the most common skin cancer in children and often presents in an atypical fashion when compared to adults. The purpose of this review is to present an update on the epidemiology, surgical and medical management and prevention strategies in pediatric melanoma. Methods A comprehensive review of the current literature on the epidemiology, surgical and medical management and prevention of adult and pediatric melanoma was performed by the authors and the results of this review are summarized in the manuscript. Results Most recently, the incidence of melanoma in children has been declining, possibly owing to increased awareness and sun exposure prevention. The mainstay of therapy is surgical resection, often with sentinel lymph node biopsy. A positive sentinel node has prognostic value; however, completion node dissection is no longer recommended in the absence of clinically or radiographically positive nodes. Those with advanced disease also receive adjuvant systemic therapy using increasingly targeted immunologic therapies. Conclusions Sentinel lymph node positive patients no longer require completion lymph node dissection and instead may be followed by ultrasound. However, it is important to note that children have been excluded from most melanoma clinical trials to date, and therefore, recommendations for management are based on existing pediatric retrospective data and extrapolation from adult studies. Level of evidence IV.

BACKGROUND: Available evidence supports ovary-sparing surgery for benign ovarian neoplasms; however, preoperative risk stratification of pediatric ovarian masses can be difficult. Our objective of this study was to characterize the surgical management of pediatric ovarian neoplasms across 10 children’s hospitals and to identify factors that could potentially aid in the preoperative risk stratification of these lesions. METHODS: A retrospective review of girls and women aged 2 to 21 years who underwent surgery for an ovarian neoplasm between 2010 and 2016 at 10 children’s hospitals was performed. Multivariable logistic regression was used to examine the relationships between the preoperative cohort characteristics, procedure performed, and risk of malignancy. RESULTS: Among 819 girls and women undergoing surgery for an ovarian neoplasm, malignant lesions were identified in 11%. The overall oophorectomy rate for benign disease was 33% (range: 15%–49%) across institutions. Oophorectomy for benign lesions was independently associated with provider specialty (P = .002: adult gynecologist, 45%; pediatric surgeon, 32%; pediatric gynecologist, 18%), premenarchal status (P = .02), preoperative suspicion for malignancy (P < .0001), larger lesion size (P < .0001), and presence of solid components (P < .0001). Preoperative findings independently associated with malignancy included increasing size (P < .0001), solid components (P = .003), and age (P < .0001). CONCLUSIONS: The rate of oophorectomy for benign ovarian disease remains high within the pediatric population. Identification of factors associated with the choice of procedure and the risk of malignancy may allow for improved preoperative risk stratification and fewer unnecessary oophorectomies. These results have been used to develop and validate a multidisciplinary preoperative risk stratification algorithm that is currently being studied prospectively across 10 institutions.

Although most children with cancer can be cured of their disease, a subset of patients with adverse tumor types or biological features, and those with relapsed or refractory disease have significantly worse prognosis. Furthermore, current cytotoxic therapy is associated with significant late effects. Precision oncology, using molecular therapeutics targeted against unique genetic features of the patient's tumor, offers the potential to transform the multimodal therapy for these patients. Potentiated by advances in sequencing technology and molecular therapeutic development, and accelerated by large-scale multi-institutional basket trials, the field of pediatric precision oncology has entered the mainstream. These novel therapeutics have important implications for surgical decision making, as well as pre- and postoperative care. This review summarizes the current state of precision medicine in pediatric oncology including the active North American and European precision oncology clinical trials. LEVEL OF EVIDENCE: Treatment study Level V.

Adequate nutritional support for critically ill pediatric patients is an essential component of optimal care in the intensive care unit. For select patient populations in this setting, the enteral delivery of nutrients can bring unique challenges and potential risks. The focus of this paper is to provide a review of existing literature concerning the safety, efficacy and benefits of enteral nutrition in select surgical patient populations for whom these discussions are most controversial: patients with traumatic brain injury, patients receiving extracorporeal life support (ECLS), and patients receiving vasopressor therapy, such as in sepsis. Recommendations in the context of consensus or a call to investigate research gaps are provided based on a review of the evidence.

This chapter describes the surgical procedure for wide local excision of malignant melanoma with or without sentinel lymph node biopsy. The text includes an introduction that outlines the indications, risks, alternatives, essential steps, needed equipment, and variations in technique for the procedure in question. This is followed by a template operative dictation, which provides the reader with an operative report, such as is found in a patient chart or electronic medical record. Because melanoma can arise in multiple anatomic locations, the surgical approach and technique will vary based on the primary tumor location, thickness, and other characteristics.

This chapter describes the surgical procedure for right/left thyroid lobectomy/total thyroidectomy. The text includes an introduction that outlines the indications, risks, alternatives, essential steps needed, equipment, and variations in technique for the procedure in question. This is followed by a template operative dictation that provides the reader with an operative report, such as is found in a patient chart or electronic medical record. A description is provided of the following critical concepts for this procedure: developing subplatysmal flaps, exposing the thyroid gland, ligation of superior pole vessels, medial mobilization of each lobe, preservation of the recurrent laryngeal nerve, preservation of the parathyroid glands, and dissecting the thyroid free from the trachea.

This chapter describes the surgical procedure for adrenalectomy as performed by the following approaches: open and minimally invasive. The text includes an introduction that outlines the indications, risks, alternatives, essential steps, needed equipment, and variations in technique for the procedure in question. This is followed by a template operative dictation, which provides the reader with an operative report, such as is found in a patient chart or electronic medical record. A description is provided of the following critical concepts for this procedure: preoperative optimization based on hypersecretory/functional status, access to the adrenal gland by mobilizing overlying structures, vascular control (especially venous for pheochromocytoma), and complete dissection of adrenal gland and surrounding adipose tissue.

This chapter describes the surgical procedure for hepatic resection (right/left hepatectomy; extended right/left hepatectomy; wedge resection). The text includes an introduction that outlines the indications, risks, alternatives, essential steps, needed equipment, and variations in technique for the procedure in question. This is followed by a template operative dictation, which provides the reader with an operative report, such as is found in a patient chart or electronic medical record. Because liver tumors can arise in multiple anatomic locations, the surgical approach and technique will vary based on the primary tumor.

This chapter describes the surgical procedure: exploratory laparotomy, right/left nephrectomy, paraaortic/paracaval lymph node dissection. The text includes an introduction that outlines the indications, risks, alternatives, essential steps, needed equipment, and variations in technique for the procedure in question. This is followed by a template operative dictation, which provides the reader with an operative report, such as is found in a patient chart or electronic medical record. A description is provided of the following critical concepts for this procedure: mobilization of the colon, dissection of the kidney, vascular control and ligation, ureteral division, and lymph node sampling.

This chapter describes the surgical procedure for abdominal resection of neuroblastoma. The text includes an introduction that outlines the indications, risks, alternatives, essential steps, needed equipment, and variations in technique for the procedure in question. This is followed by a template operative dictation, which provides the reader with an operative report, such as is found in a patient chart or electronic medical record. Because neuroblastomas can arise in multiple anatomic locations, the surgical approach and technique will vary based on the primary tumor.

BACKGROUND: Optimal management of neutropenic appendicitis (NA) in children undergoing cancer therapy remains undefined. Management strategies include upfront appendectomy or initial nonoperative management. We aimed to characterize the effect of management strategy on complications and length of stay (LOS) and describe implications for chemotherapy delay or alteration. METHODS: Sites from the Pediatric Surgery Oncology Research Collaborative performed a retrospective review of children with NA over a 6-year period. RESULTS: Sixty-six children, with a median age of 11 years (range 1–17), were identified with NA while undergoing cancer treatment. The most common cancer diagnoses were leukemia (62%) and brain tumor (12%). Upfront appendectomy was performed in 41% of patients; the remainder had initial nonoperative management. Rates of abscess or perforation at diagnosis were equivalent in the groups (30% vs 24%; P = .23). Of patients who had initial nonoperative management, 46% (17 of 37) underwent delayed appendectomy during the same hospitalization. Delayed appendectomy was due to failure of initial nonoperative management in 65% (n = 11) and count recovery in 35% (n = 6). Cancer therapy was delayed in 35% (n = 23). Initial nonoperative management was associated with a delay in cancer treatment (46% vs. 22%, P = .05) and longer LOS (29 vs 12 days; P = .01). Patients who had initial nonoperative management and delayed appendectomy had a higher rate of postoperative complications (P CONCLUSIONS: In pediatric patients with NA from oncologic treatment, upfront appendectomy resulted in lower complication rates, reduced LOS, and fewer alterations in chemotherapy regimens compared to initial nonoperative management.

Identification of indeterminate melanocytic skin lesions capable of neoplastic progression is suboptimal and may potentially result in unnecessary morbidity from surgery. MicroRNAs (miRs) may be useful in classifying indeterminate Spitz tumors as having high or low risk for malignant behavior.RNA was extracted from paraffin-embedded tissues of benign nevi, benign Spitz tumors, indeterminate Spitz tumors, and Spitzoid melanomas in adults (n = 62) and children (n = 28). The expression profile of 12 miRs in adults (6 miRs in children) was analyzed by real-time polymerase chain reaction.Benign Spitz lesions were characterized by decreased expression of miR-125b and miR-211, and upregulation of miR-22, compared with benign nevi (p 0.05). A comparison of Spitzoid melanomas to benign nevi revealed overexpression of miR-21, miR-150, and miR-155 in the malignant primaries (p 0.05). In adults, Spitzoid melanomas exhibited upregulation of miR-21, miR-150, and miR-155 compared with indeterminate Spitz lesions. Indeterminate Spitz lesions with low-risk pathologic features had lower miR-21 and miR-155 expression compared with Spitzoid melanoma tumors in adults (p 0.05), while pathologic high-risk indeterminate Spitz lesions had increased levels of miR-200c expression compared with low-risk indeterminate lesions (p 0.05). Pediatric Spitzoid melanomas exhibited increased miR-21 expression compared with indeterminate Spitz lesions (p 0.05). Moreover, miR-155 expression was increased in indeterminate lesions with mitotic counts1 and depth of invasion1 mm, suggesting miR-155 expression is associated with histological characteristics.miR expression profiles can be measured in indeterminate Spitz tumors and correlate with markers of malignant potential.

PURPOSE Children receiving radiotherapy of the head, neck, or chest as treatment of primary malignancies are at increased risk for secondary thyroid malignancy. We hypothesized that current standards (yearly thyroid physical examination) lead to a substantial number of missed thyroid nodules. Our objectives were: (1) use thyroid ultrasound to assess thyroid nodules in childhood cancer survivors; and (2) compare prevalence of thyroid nodules in thyroid radiation-exposed patients as compared with cancer survivors without radiation exposure. METHODS We recruited 60 patients with thyroid radiation and chemotherapy exposure (median age at cancer diagnosis 10.8 y) and 59 patients with chemotherapy exposure alone (median age at diagnosis 4.3 y) from our long-term survivorship clinics. Each patient had a thyroid physical examination and thyroid ultrasound performed. RESULTS Thirty-three patients (27.7%) had nodules >0.3 cm of which 2 were palpated (6.1%). We found 22 radiated patients (36.7%) with nodules versus 11 nonradiated patients (18.6%) (P=0.03). Eleven patients were biopsied and 1 diagnosis of secondary papillary thyroid carcinoma was confirmed. CONCLUSION Our study supports further examination of incorporating thyroid ultrasounds into long-term survivorship follow-up guidelines in radiation-exposed patients for the detection of thyroid nodules and secondary malignancies.

BACKGROUND Rhabdoid predisposition syndrome is characterized by germline alterations in SMARCB1 or SMARCA4, leading to synchronous or metachronous central nervous system (CNS) and extra-CNS rhabdoid tumors. Rare survivors have been reported to date. METHODS We describe the molecular profiling and treatment regimen of three patients with synchronous atypical teratoid/rhabdoid tumor (ATRT) and malignant rhabdoid tumor of the kidney (MRT-K). All patients underwent radical nephrectomy of the kidney, and gross total resection of the primary CNS tumor was achieved for two patients. An intensive chemotherapy regimen was administered; an induction phase based on the modified Third Intergroup Rhabdomyosarcoma Study (IRS-III) for ATRT followed by a consolidation phase with three cycles of high-dose chemotherapy and autologous hematopoietic progenitor cell rescue, without irradiation. All three patients were enrolled on an institutional comprehensive genomic profiling protocol. RESULTS A germline focal 22q deletion, including SMARCB1, was detected in two patients, while the third patient had a maternally-inherited heterozygous frameshift variant in SMARCB1. Somatic loss of heterozygosity of 22q was identified in all patients, resulting in biallelic inactivation of SMARCB1. Divergent tumor subgroups were described using DNA methylation. The three MRT-K samples were classified as MYC subtype. One ATRT was classified as SHH while the other as TYR. One patient is currently three years off-therapy without evidence of disease, while the other two patients have completed the consolidation phase without recurrent disease. CONCLUSION Molecular profiling of CNS and extra-CNS rhabdoid tumors revealed different epigenetic subgroups. An intensive multimodal therapeutic approach without irradiation may achieve prolonged survival.

Introduction Atelectasis is a problem in sedated pediatric patients undergoing cross-sectional imaging, impairing the ability to accurately interpret chest computed tomography (CT) imaging for the presence of malignancy, often leading to additional maneuvers and/or repeat imaging with additional radiation exposure. Methods A quality improvement team established a best-practice protocol to improve the quality of thoracic CT imaging in young patients with suspected primary or metastatic pulmonary malignancy. The specific aim was to increase the percentage of chest CT scans obtained for the evaluation of pulmonary nodules with acceptable atelectasis scores (0-1) in patients aged 0-5 years with malignancy, from a baseline of 45% to a goal of 75%. Results A retrospective cohort consisted of 94 patients undergoing chest CT between February 2014 and January 2015 before protocol implementation. The prospective cohort included 195 patients imaged between February 2015 and April 2018. The baseline percentage of CT scans that were scored 0 or 1 on the atelectasis scale was 44.7%, which improved to 75% with protocol implementation. The mean atelectasis score improved from 1.79 (±0.14) to 0.7 (±0.09). Sedation incidence decreased substantially from 73.2% to 26.5% during the study period. Conclusions Using quality improvement methodology including standardization of care, the percentage of children with atelectasis scores of 0-1 undergoing cross-sectional thoracic imaging improved from 45% to 75%. Also, eliminating the need for sedation in these patients has further improved image quality, potentially allowing for optimal detection of smaller nodules, and minimizing morbidity.

Neuroblastoma is an embryonic cancer arising from neural crest stem cells. This cancer is the most common malignancy in infants and the most common extracranial solid tumor in children. The clinical course may be highly variable with the possibility of spontaneous regression in the youngest patients and increased risk of aggressive disease in older children. Clinical heterogeneity is a consequence of the diverse biologic characteristics that determine patient risk and survival. This review will focus on current progress in neuroblastoma staging, risk stratification, and treatment strategies based on advancing knowledge in tumor biology and genetic characterization. TYPE OF STUDY: Review article. LEVEL OF EVIDENCE: Level II.

Background Chemotherapy is used preoperatively for children with bilateral Wilms tumor (BWT) or unilateral high-risk Wilms tumor (UHRWT) to promote tumor regression to facilitate renal preservation with nephron-sparing surgery (NSS). In adults, various surgical techniques have been described to preserve renal tissue. Few studies have examined the use of surgical adjuncts in NSS in children with renal tumors. Methods We performed a multi-institutional retrospective review of patients with BWT or UHRWT. Patient demographics, tumor size at diagnosis, following neoadjuvant chemotherapy, utilization of surgical adjuncts including intraoperative ultrasound (IOUS), margin status, complications, renal function, and follow-up were recorded. Results The cohort comprised 23 patients: 18 BWT, 3 UHRWT, and 2 patients with solitary kidney. Twenty-two of the 23 patients had successful NSS. IOUS was used 19 times, and seven had positive margins after surgery. Cooling/vascular isolation was used six times. At a median follow-up of 18 mo, median estimated glomerular filtration rate Schwartz was 126 mL/min/1.73 m2 and median serum creatinine 0.39 mg/dL in the 22 patients who had successful NSS. There have been no tumor recurrences. Conclusions In patients with BWT and UHRWT, surgical adjuncts such as cooling/vascular isolation are uncommonly performed. IOUS may be helpful but does not guarantee negative microscopic margins. Level of evidence Level 4, Case series with no comparison group.

Wilms tumor (WT) is the most prevalent pediatric renal tumor and most commonly occurs between ages 1 and 5 years. Data are lacking on children younger than 12 months with renal tumors. The cancer registry at the authors' institution was queried to identify patients 12 months and younger with renal masses. Demographics, clinical presentation, histopathology, stage, and survival outcomes were reviewed. The most common presenting symptoms included an asymptomatic abdominal mass (73%) and hematuria (9%). Histopathology revealed WT in 73% of patients, mesoblastic nephroma in 20%. Of those infants younger than 1 month of age, mesoblastic nephroma was the most common histopathology (68%). The 5-year overall survival (OS) was 93%, and 5-year event-free survival (EFS) was 93% for the entire group. For patients with WT, 5-year OS was 88% and 5-year EFS was 83%. Outcomes for congenital mesoblastic nephroma were excellent with 5-year OS and EFS of 100%. Reasons for good prognosis may be multifactorial and may include frequent well child checks in the first year of life and favorable histology. Patients in this age group are more likely to be classified as very low risk and may be treated with surgical resection alone.