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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.

Introduction The COVID-19 pandemic resulted in the suspension of nonemergent surgeries throughout New York. Our tertiary care children’s hospital pivoted towards a brief trial of intravenous (IV) antibiotic therapy in all patients in order to limit operating room (OR) utilization and avoid prolonged hospital stays. We describe our pandemic-based strategy for non-operative management (NOM) of appendicitis but with a limited duration of IV antibiotics. Methods We performed a retrospective study of children treated for acute appendicitis at our center from 3/31/2020 to 5/3/2020 during the peak of the New York pandemic. We compared appendicitis volume to similar months in prior years. We evaluated failure of NOM, length of stay, and compared characteristics of children we successfully treated with our expanded NOM protocol to previously published inclusion criteria for NOM. Results 45.5% of children (25/55) with acute appendicitis underwent NOM. Of the 30 who underwent surgery, 13 had complicated appendicitis while 17 had simple appendicitis. Three patients were COVID-positive, although none had respiratory symptoms. The majority of patients presenting with acute appendicitis (78.2%) did not meet previously published criteria for NOM. Conclusions We treated a similar volume of children with acute appendicitis during the pandemic compared to prior years. We applied non-operative management to nearly half our patients, even as we expanded inclusion criteria for NOM to reduce OR utilization, but limited the duration of the antibiotic trial to avoid prolonged hospital stays. Type of study Retrospective study. Level of evidence IV.

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.

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.

The factors affecting outpatient follow-up (OFU) after pediatric surgery have not been well studied. We evaluate factors impacting OFU and the effect of OFU in pediatric surgical patients.A retrospective review of all pediatric patients operated on by the Division of Pediatric Surgery from February 1st to September 30th, 2017, and subsequently discharged was performed.1242 patients were identified. Overall OFU was 69.6%. Language and distance between patient residence and the hospital had no impact on OFU. Inpatient surgical patients followed-up at a higher rate than ambulatory surgical patients (72.7% vs 64.8%, p 0.01). Out-of-system transfers had the lowest OFU rate at 52.8% (p 0.001). Insurance type and patient age had a significant impact on OFU rates. Thirty-day ED visit and readmission rates were significantly lower in those patients with OFU than in those without (8.8% vs 12.7%, p = 0.04 and 3.7% to 11.0%, p 0.001, respectively). OFU was more beneficial in patients with inpatient procedures or longer hospitalization lengths of stay than in the cohort of ambulatory patients.Socioeconomic status, hospital presentation, and procedural complexity influenced rates of OFU. OFU was associated with significant reductions in 30-day ED visits and readmissions, and this benefit was more pronounced for complex procedures or patients.Retrospective review.Level III.

Purpose Malpractice litigation among pediatric surgeons is a subject of concern and interest, but minimal factual data are known. Our goal was to investigate national litigation trends regarding pediatric surgical conditions. Methods We queried WestlawNext database for malpractice cases involving pediatric (age ≤ 18) surgical conditions. Cases were included if they named a care provider or health center. We gathered data on diagnoses, procedures, care providers, allegations, location, and outcomes. Results Our search revealed 4754 cases, and 170 met inclusion criteria. These ranged from 1965 to 2017 and represented 40 states. 110 cases involved a surgeon (41% pediatric surgeons). Appendicitis was the most common diagnosis identified. Cases frequently involved delayed/missed diagnoses or interventions (45.9%), technical concerns (35.9%), mortalities (26.5%), negligent perioperative care (23.6%), and informed consent concerns (4.7%). Technical complication was the most common allegation against surgeons (49.1%), and nonsurgeon cases typically involved a delayed/missed diagnosis (78.3%). 39% of cases resulted in favor of the defendant, 35% plaintiff, and 14% had a split verdict. Conclusion Litigation involving pediatric surgical conditions is diverse, but appendicitis and circumcision comprise almost a third of cases. A greater understanding of these trends can help steer efforts in quality and safety as well as guide improved communication with families. Level of evidence N/A

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/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.

Introduction The role of advanced care practitioners (ACPs) in pediatric surgery is increasingly important and not well described. Methods Electronic surveys were sent to pediatric surgery division chiefs within the Children's Hospital Association. Results We received 77/163 survey responses (47%). The median number of ACPs per service was 3.0 (range 0–35). ACP number correlated with inpatient census, surgeon number, case volume, trauma centers, intensive care unit status, and fellowship programs but not with presence of residents/hospitalists, hospital setting, or practice type. Nearly all programs incorporated nurse practitioners while almost half utilized physician assistants. Approximately one-third of ACPs were designated for subspecialties (35%) such as trauma and colorectal. Only 9% of centers had surgeon-specific ACPs. ACP responsibilities included both inpatient and outpatient tasks. Nearly all ACPs participated in procedures (89%), mostly bedside (80%). All ACPs worked daytime shifts, with less nights and weekends. Most ACPs billed for services (80%). Satisfaction with ACP coverage was widespread and did not correlate with ACP number. Most respondents felt that ACPs enhance, and not hinder, resident/fellow training (85%). Conclusion ACPs are useful adjuncts in pediatric surgery. A better understanding of practice patterns may help optimize utilization to enhance patient care and can be used to advocate for appropriate resources.

The Children's Oncology Group (COG) renal tumor study (AREN03B2) requires real-time central review of radiology, pathology, and the surgical procedure to determine appropriate risk-based therapy. The purpose of this study was to determine the inter-rater reliability of the surgical reviews.Of the first 3200 enrolled AREN03B2 patients, a sample of 100 enriched for blood vessel involvement, spill, rupture, and lymph node involvement was selected for analysis. The surgical assessment was then performed independently by two blinded surgical reviewers and compared to the original assessment, which had been completed by another of the committee surgeons. Variables assessed included surgeon-determined local tumor stage, overall disease stage, type of renal procedure performed, presence of tumor rupture, occurrence of intraoperative tumor spill, blood vessel involvement, presence of peritoneal implants, and interpretation of residual disease. Inter-rater reliability was measured using the Fleiss' Kappa statistic two-sided hypothesis tests (Kappa, p-value).Local tumor stage correlated in all 3 reviews except in one case (Kappa=0.9775, p0.001). Similarly, overall disease stage had excellent correlation (0.9422, p0.001). There was strong correlation for type of renal procedure (0.8357, p0.001), presence of tumor rupture (0.6858, p0.001), intraoperative tumor spill (0.6493, p0.001), and blood vessel involvement (0.6470, p0.001). Variables that had lower correlation were determination of the presence of peritoneal implants (0.2753, p0.001) and interpretation of residual disease status (0.5310, p0.001).The inter-rater reliability of the surgical review is high based on the great consistency in the 3 independent review results. This analysis provides validation and establishes precedent for real-time central surgical review to determine treatment assignment in a risk-based stratagem for multimodal cancer therapy.