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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.

Survivors of pediatric cancer are at increased risk for infertility and premature hormonal failure. Surgeons caring for children with cancer have an important role to play in understanding this risk, as well as advocating for and performing appropriate fertility preservation procedures. Fertility preservation options in males and females vary by pubertal status and include nonexperimental (oocyte harvest, ovarian tissue cryopreservation, sperm cryopreservation) and experimental (testicular tissue cryopreservation) options. This review summarizes the basics of risk assessment and fertility preservation options and explores unique considerations in pediatric fertility preservation.

RMS most commonly presents in children and adolescents, however a subset of tumors are diagnosed in infants under one year of age. Due to the rarity of infant RMS, utilization of different treatment approaches and goals, and small sample sizes, the published studies of infants with RMS have yielded heterogeneous results. In this review, we discuss the outcomes of infants with RMS treated in various clinical trials and the strategies that various international cooperative groups have employed to reduce the morbidity and mortality related to treatment without compromising the overall survival of this population. This review discusses the unique scenarios of diagnosing and managing congenitals or neonatal RMS, spindle cell RMS and relapsed RMS. This review concludes by exploring novel approaches to diagnosis and management of infants with RMS that are currently being studied by various international cooperative groups.

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.

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.

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/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.

To describe variations in timing of gastrostomy tube (GT) placement for neonates undergoing tracheostomy. Database study of neonates undergoing tracheostomy and GT placement using the Pediatric Health Information System (2012–2015). The primary outcome was timing of GT relative to tracheostomy. Logistic regression evaluated associations of patient- and hospital-level characteristics with GT timing. Of 1156 patients undergoing GT and tracheostomy placement, 42.4% had concurrent GT placement, 23.3% GT placement prior to tracheostomy, and 34.3% GT placement after tracheostomy. The proportion of patients undergoing concurrent placement ranged from 0 to 80% among 47 hospitals. Neonates born at 31–35 weeks, having cardiovascular comorbidities, history of diaphragmatic hernia repair, or gastroesophageal reflux disorder were more likely to receive GT placement prior to tracheostomy. Significant variability exists in the timing of neonatal tracheostomy and GT placement. Opportunities may exist to optimize coordination of care for neonates and reduce anesthetic exposure and hospital resource utilization.

To assess the utility of preoperative venography in evaluating and managing patients with congenital portosystemic shunts (CPSSs).A retrospective study was performed of 42 patients (62% female; median age, 4.1 years) diagnosed with a CPSS from 2005 to 2018. Preoperative venography (n = 39) and balloon occlusive pressure measurements (n = 33) within the mesenteric venous system guided treatment. Primary outcome was serum ammonia levels at 1 month after shunt closure. Management strategies included single (n = 12) or staged (n = 18) operative ligation, endovascular occlusion (n = 8), combined surgical and endovascular closure (n = 2), and observation (n = 2).At 1 month, serum ammonia levels decreased from 82.5 ± 10.3 μmol/L to 38.4 ± 4.6 μmol/L (P.001). No difference was observed in the decrease between patients treated surgically or endovascularly (P = .91). Mean occluded to non-occluded pressure gradients were significantly lower for endovascular closure (5.3 ± 1.8 mmHg) than for surgical closure (12.3 ± 3.3 mmHg, P = .02). Shunts were classified as extrahepatic in 29 patients and as intrahepatic in 13 patients; all shunts demonstrated filling of the portal system with occlusive venography. Broad and short shunts were closed surgically; narrow and long shunts were closed endovascularly. Shunts were closed in a single session (n = 20) if the pressure gradient was less than 10 mmHg and the occluded mesenteric pressure was less than 25 mmHg.Preoperative venography delineates shunt morphology, and balloon occlusion simulates closure hemodynamics. This information is necessary to determine whether definitive closure should be performed through endovascular or surgical methods and whether closure should be performed in a single or staged setting.

OBJECTIVE To describe utilization and long-term outcomes of pneumonectomy in children and adolescents with cancer. SUMMARY BACKGROUND DATA Pneumonectomy in adults is associated with significant morbidity and mortality. Little is known about the indications and outcomes of pneumonectomy for pediatric tumors. METHODS The Pediatric Surgical Oncology Research Collaborative (PSORC) identified pediatric patients

A subset of patients with initially unresected (Clinical Group III) rhabdomyosarcoma achieve less than a complete response (CR) despite multimodal therapy. We assessed outcome based upon tumor response at the completion of all planned therapy. We studied 601 Clinical Group III participants who completed all protocol therapy without developing progressive disease on two Children's Oncology Group studies ARST0531 (n = 285) and D9803 (n = 316). Response was defined by imaging and categorized by response; complete resolution (CR), partial response (PR) or no response (NR). Failure-free survival (FFS) and overall survival (OS) between response groups were compared using the log-rank test. We found that radiographic response was CR in 393 (65.4%) and PR/NR in 208 (34.6%) patients. Achieving CR status was associated with study D9803, nonparameningeal (PM) primary sites, tumors ≤5 cm, noninvasive tumors and alveolar histology/FOXO fusion-positive tumors. The overall 5-year FFS was 75% for those achieving CR and 66.5% in those with PR/NR (adj. p = 0.094). Patients with PM primary site who achieved CR had significantly improved FFS (adj. p = 0.037) while those with non-PM primary sites had similar outcomes (adj. p = 0.47). Radiographic response was not associated with OS (adj. p = 0.21). Resection of the end-of-therapy mass did not improve FFS (p = 0.12) or OS (p = 0.37). In conclusion, CR status at the end of protocol therapy in patients with PM Clinical Group III RMS was associated with improved FFS but not OS. Efforts to understand the biology and treatment response in patients with PM primary site are under investigation.

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.

The purpose of this systematic review by the American Pediatric Surgical Cancer Committee was to summarize evidence from the current medical literature regarding fertility restoration and hormone replacement for female children and adolescents treated with gonadotoxic treatments., info:eu-repo/semantics/published

Background/purpose Testicular tissue cryopreservation (TTC) provides an experimental option for fertility preservation for male children at significant risk for azoospermia owing to high-risk gonadotoxic treatments. Methods A single institution, retrospective review of TTC cases from 2015 to 2017. Children at significant risk for azoospermia were eligible for study inclusion. A unilateral wedge biopsy of the testis was performed for TTC. Results TTC was performed in 23 patients. Average age was 10 years old (5 months to 18 years). Diagnoses included solid tumor (74%, n = 17), hematologic malignancy (17%, n = 4), and benign hematologic disease (13%, n = 3). Six patients had TTC at the time of disease relapse. Nine patients were referred for TTC prior to stem cell transplantation. The majority (70%, n = 16) of patients had an additional procedure at the time of TTC. One patient developed postoperative scrotal cellulitis that was treated with antibiotics. The majority of patients (96%, n = 22) had normal testicular tissue with the presence of germ cells on histopathological analysis. Median time to start of medical therapy was seven days with no unanticipated treatment delays. Conclusions Testicular wedge biopsy for TTC can be performed safely, coordinated with other necessary procedures, and does not delay the start of treatment. TTC remains an experimental option for fertility preservation for children, as no spermatogenic recovery or pregnancies from cryopreserved testicular tissues have been reported to date. Level of evidence IV.

Malrotation with midgut volvulus is a time-sensitive pediatric surgical disease that requires emergent operative exploration to avoid bowel loss; however, it also requires specialized pediatric care. The purpose of this study was to identify disparities in bowel resection in children who underwent emergency surgery for malrotation; particularly the role of transfer status.The Pediatric Health Information System was used to identify a multicenter cohort of patients who underwent emergency surgical intervention for malrotation. Univariate and multivariable analyses were used to determine factors associated with the primary outcome of bowel resection; secondary outcomes included surgical complications, prolonged length of stay, TPN dependence, and death.Of 3373 patients with malrotation included, 44.8% were transferred in. Younger age, prematurity and other comorbidity, nonwhite race, and public insurance were associated with transfer. Transferred patients were more likely to undergo bowel resection on univariate (30.7 vs 16.4%, p .001) and multivariable analysis (RR =1.38, p .010). After adjusting for bowel resection, only patient factors including age and comorbidity were associated with surgical complications, TPN dependence, and death.Patients who require hospital-to-hospital transfer for emergent surgical management of malrotation are more likely to require bowel resection which is in turn associated with greater morbidity. Further work is needed to optimize access to prompt surgical care for this condition especially given race and insurance disparities in transfer status.III, prognostic study.

Three large national data sets are commonly used to assess operative outcomes in pediatric surgery; National Surgical Quality Improvement Program Pediatric (NSQIP-P), Pediatric Health Information System (PHIS), and Kids' Inpatient Data set (KID). Hepatectomy and nephrectomy are rare pediatric surgical procedures, which may benefit from large administrative data sets for the assessment of short-term complications.A retrospective review of NSQIP-P (2012-2015), KID (2012), and PHIS (2012-2015) was performed for hepatectomy or nephrectomy cases for children aged 0 to 18 y. Thirty-day perioperative outcomes were collected, analyzed, and compared across data sets and surgical cohorts.Rates of surgical site infection, wound dehiscence, central line infection, sepsis, and venous thromboembolism were similar across NSQIP-P, PHIS, and KID in both cohorts. Rates of pneumonia and renal insufficiency were higher in PHIS and KID versus NSQIP-P in both cohorts. Blood transfusions in NSQIP-P were higher than PHIS and KID in the hepatectomy group (50.9% versus 43.0% versus 32.4%, P 0.001), but similar across data sets in the nephrectomy cohorts (12.0% versus 14.0% versus 13.0%, P = 0.15). PHIS reported higher readmission rates than NSQIP-P for both the hepatectomy (56.5% versus 17.9%, P 0.001) and nephrectomy (32.6% versus 7.6%,P 0.001) cohorts. Thirty-day mortality rates were similar between NSQIP-P and PHIS, but higher in KID as compared with NSQIP-P for hepatectomy (6.4% versus 0.4%, P 0.001) and nephrectomy (2.0% versus 0.3%, P 0.001) cases.Administrative data sets provide large sample sizes for the study of low-volume procedures in children, but there are significant variations in the reported rates of perioperative outcomes between NSQIP-P, PHIS, and KID. Therefore, surgical outcomes should be interpreted within the context of the strengths and limitations of each data set.

Background/Purpose To assess the additive value of magnetic resonance imaging (MRI) in the setting of an equivocal US (Eq-US) with or without an elevated absolute neutrophil count (ANC). Methods Single-institution, retrospective review of children ages 5–18 years who presented to the ER with suspected appendicitis from 9/2015 to 8/2016. US, ANC, and MRI results were reviewed. Imaging was identified as positive/suspicious, normal, or equivocal and ANC Results 738 patients with a median age of 11 years (IQR 8–14) met inclusion criteria. US was equivocal in 61.4%. Among 304 (67.1%) patients with an Eq-US and normal ANC, only 5 (1.6%) had acute appendicitis. In contrast, 28 of 149 patients (18.8%) with Eq-US and elevated ANC had appendicitis. MRI was performed in 125 patients with Eq-US and was positive/suspicious in 2.9% (2/69) with normal ANC and 25.0% (14/56) with elevated ANC. MRI had 94.7% sensitivity and 100% specificity for acute appendicitis in patients with an Eq-US. Conclusions MRI has high sensitivity and specificity for diagnosing acute appendicitis in children. Patients with Eq-US plus a normal ANC have a very low likelihood of appendicitis and do not typically require further imaging. MRI may have utility for children with Eq-US and elevated ANC. Level of Evidence Level III.

Many benign and malignant conditions require gonadotoxic treatment regimens that may threaten a child or adolescent's future fertility. Both males and females who have undergone gonadotoxic regimens are more likely to be infertile or have difficulty getting pregnant when compared to their siblings, however the desire to parent in the future is a priority. All children who are at risk of infertility should receive a fertility preservation (FP) consultation before treatment begins. We present a case report of a prepubertal boy who was diagnosed with osteosarcoma. His initial chemotherapeutic regimen did not qualify him for a FP procedure, but when he relapsed, his subsequent therapy did. Due to his peripubertal status he attempted sperm banking, a non-experimental FP method, but was azoospermic. He ultimately achieved success with a combination of testicular tissue cryopreservation and testicular sperm extraction, experimental FP procedures. What follows is a discussion of available FP options for peripubertal males and the current state of research for male fertility restoration. Keywords: Fertility preservation, Testicular tissue cryopreservation, Testicular sperm extraction

There is a lack of pediatric-specific guidelines for the workup and management of primary spontaneous pneumothorax (PSP) in children. The aim of this study was to describe current practices among North American pediatric surgeons.An online survey comprising 18 questions was sent out through the American Pediatric Surgical Association Outcomes and Clinical Trials Committee to all members. Bivariate analysis was performed using Chi-square analysis.A total of 287 surveys were completed (33% response rate). For a first episode of PSP, 57% of surgeons opt for chest tube drainage, 4% for upfront video-assisted thoracoscopic surgery (VATS), 3% for needle aspiration, and 29% for only oxygen administration. Eighty-one percent of surgeons report that the size of the pneumothorax influences management. However, neither practice setting (P = .87) nor years in practice (P = .11) correlated with initial management strategy. For patients with a persistent air leak after chest tube placement, there is wide variation in duration of observation before performing VATS, with 40% operating after 3 days, but 21% waiting at least 5 days. The use of chest computed tomography (CT) is also highly variable. Eighty-two percent of respondents perform surgery only after the second episode of PSP. Most perform a stapled apical blebectomy and mechanical pleurodesis for both initial and recurrent PSP.There is significant variation among pediatric surgeons in the management of spontaneous pneumothorax, including the use of CT, timing of operation, and duration of observation for air leak before performing surgery. Prospective data are needed to better inform guidelines and standardize practice.

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.

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.

Purpose While central venous port placement is common, there remains variation in placement technique and rates of technical complications. The aim of this study was to assess variability in techniques and identify predictors of complications for children undergoing port placement. Methods We retrospectively reviewed all 331 patients who underwent venous port placement at a single tertiary children's hospital from May 2018 to June 2020. The primary outcome was early revision or replacement (R/R) for complications occurring within 30 days. Secondary outcomes included radiation exposure and rate of intraoperative conversion to a secondary site. Results The median age was 7 years (Interquartile Range 3-13 years) and the most common diagnoses were leukemia (30.2%), solid tumors (27.8%), and brain tumors (16.9%). Initial approach for port placement was ultrasound-guided internal jugular (IJV) in 255 (147 by surgery and 108 by interventional radiology [IR]) and landmark subclavian vein (SCV) in 76 (all by surgery). Early R/R occurred in 5.1%, including 9.0% of patients with leukemia but 1.1% with solid tumors. Individual proceduralist volume ranged from 2 to 98 cases and was inversely correlated with early R/R (r = -0.12, p = 0.30). In univariate analysis, ports placed by IR had an increased rate of early R/R (9.3%, n = 10) compared to those placed by surgery (3.2%, n = 7, p = 0.036) but this was not significant in multivariable regression controlling for diagnosis and age (Hazard Ratio 2.04; p = 0.19). Mean fluoroscopy time was significantly longer for ports placed by IR (59.9 s) compared to those placed by surgery (15.1 s, p 0.001). Initial SCV access was associated with an increased (14.5 vs 0.4%) rate of conversion to a secondary site. Conclusions Though venous port placement is a largely safe procedure in children, a substantial minority of patients, particularly those with leukemia, require early R/R. Proceduralist volume and training may influence early R/R, fluoroscopy exposure, and anatomic site preferences.

Care for pediatric patients with serious or potentially life-limiting illness involves the interplay of multiple medical and surgical teams within the hospital. Pediatric surgeons are capable of performing procedures that can improve the quality of life for children facing serious illness, but which also carry the potential for significant risk and burden. Patients and families are often faced with decisions about invasive surgical procedures and interventions, stressing the need for seamless collaboration between palliative care and surgical providers. Equally important is the need for clear and open-ended communication with patients and families by all medical teams to determine if potential surgical procedures and interventions align with their goals and to ensure that the perceived benefits of interventions outweigh any risks. Over the last two decades, pediatric palliative care has grown into a thriving medical subspecialty Despite the importance of collaborative care, there is lack of literature on the interaction of pediatric surgery and palliative care and the role of pediatric surgeons in providing primary palliative care. This review defines surgical pediatric palliative care, and provides an in-depth discussion of the unique complexities involved in caring for children with serious and potentially life-limiting illness, while highlighting specific challenges through detailed case presentations.

BACKGROUND Most children with intermediate-risk rhabdomyosarcoma (RMS) have gross disease (group III) at the initiation of chemotherapy. Delayed primary excision (DPE) after induction chemotherapy allows for a reduction in adjuvant radiation dose, but with the risk of potential surgical morbidity. The objectives of this study were to compare outcomes in children with group III RMS who did and did not undergo DPE and to assess surgical morbidity. METHODS The study included 369 patients who had clinical group III RMS at sites amenable to DPE from intermediate-risk Children's Oncology Group studies D9803 (encouraged DPE) and ARST0531 (discouraged DPE). RESULTS The primary tumor site was bladder/prostate (136 patients; 37%), extremity (97 patients; 26%), trunk (24 patients; 7%), retroperitoneum (91 patients; 25%), or intrathoracic/perineum/perianal (21 patients; 6%). In total, 112 patients (53.9%) underwent DPE in D9803, and 26 patients (16.2%) underwent DPE in ARST0531 (P < .001), with loss of vital organ or function in 30 of 138 patients (22%). DPE allowed for a reduced radiation dose in 110 of 135 patients (81%; 51% were reduced to 36 Gy, and 30% were reduced to 42 Gy). Patients who underwent DPE had improved unadjusted overall survival (P = .013). In adjusted regression analysis, the risk of death (hazard ratio, 0.71; 95% CI 0.43-1.16) was similar for patients who did and did not undergo DPE and was improved for the subset of patients who had tumors of the trunk and retroperitoneum (hazard ratio, 0.44; 95% CI, 0.20-0.97). CONCLUSIONS Children with group III RMS have equivalent or improved outcomes with DPE and can receive a decreased radiation dose for definitive local control. The choice of local control modality should weigh the potential morbidity of surgery versus that of higher dose irradiation.

BACKGROUND Most children with cancer utilize a central venous line (CVL) for treatment. Complications often necessitate early replacement, revision, or addition (RRA), but the rate of these procedures is not known. This study sought to determine rates of RRA in pediatric oncology patients, and associated risk factors. MATERIALS AND METHODS Data queried from the Pediatric Health Information System including patients ≤18 years old with malignancy and CVL placement. Analysis included: first CVL placement of the calendar year and subsequent procedures for 6 months thereafter. RESULTS A total of 6553 children met inclusion criteria (55.9% male, median age 6 years, interquartile range: 2 to 12). RRA within 6 months was required in 25.6% of patients, with 1.7% requiring 5 or more lines. Patients with Central Line-Associated Bloodstream Infection (CLABSI) were 2.78 times more likely to require RRA within 6 months of initial CVL placement, but accounted for only 16% of RRA patients. Factors associated with RRA were age below 1 year, CLABSI, hematologic malignancy, malnutrition, clotting disorder, deep vessel thromboembolism, and obesity. Patients with implantable ports as initial CVL (42%) were less likely to need RRA. CONCLUSION Twenty-five percent require at least 1 RRA within 6 months, with associated morbidity and costs. Though strongly associated, most revisions were not related to CLABSI episodes.

Background/Purpose Thoracoscopic excision of pulmonary nodules is often required for diagnostic or therapeutic purposes, however subpleural and sub-centimeter nodules can be difficult to visualize. Various CT-guided localization techniques have been described, though there is minimal published pediatric data regarding the use of microcoils. We hypothesize that microcoil localization facilitates thoracoscopic resection of pulmonary nodules in children. Methods A multi-institutional retrospective review of children who underwent preoperative CT-guided localization of lung nodules was conducted from 2012 to 2019. A combination of methylene blue dye (MBD), wires, and microcoils were utilized for CT-guided localization. When microcoils were utilized, fluoroscopy assisted in lesion identification and resection. Results Eighteen patients (mean age 13 years, range 2–21 years) underwent thoracoscopic resection of 24 preoperatively localized pulmonary nodules. Mean size and depth of the lesions were 5.5 mm and 10 mm, respectively. Microcoil placement was successful 95% of the time and assisted in lesion localization in 88% of cases. Wire localization was not a durable technique, as 3 of 5 wires became dislodged upon lung isolation. Conclusions Preoperative CT-guided localization with microcoils can assist in fluoroscopic-guided resection of pulmonary nodules in children. This technique avoids the pitfall of wire dislodgement, and provides surgeons an additional technique to localize sub-centimeter, subpleural nodules. Type of Study Retrospective Review. Level of Evidence Level III.

Background Hirschsprung-associated enterocolitis (HAEC) is a serious potential complication after primary pull-through surgery for Hirschsprung's disease (HSCR). Administration of anal botulinum toxin (BT) injection may improve obstructive symptoms at the internal anal sphincter, leading to improved fecal passage. The timing of administration and effects on delay or prevention of HAEC are unknown. We hypothesized that BT administration increased the postoperative time to HAEC and aimed to investigate whether anal BT administration after primary pull-through surgery for HSCR is associated with increased time to inpatient HAEC admission development. Methods We performed a retrospective cohort study examining children with HSCR at US children's hospitals from 2008 to 2018 using the Pediatric Health Information System database with an associated primary pull-through operation performed before 60 d of age. The intervention assessed was the administration of BT concerning the timing of primary pull-through, and two groups were identified: PRO (received BT at or after primary pull-through, before HAEC) and NOT (never received BT, or received BT after HAEC). The primary outcome was time from pull-through to the first HAEC admission. The Cox proportional hazards model was developed to examine the BT administration effect on the primary outcome after controlling for patient-level covariates. Results We examined a total of 1439 children (67 in the PRO and 1372 in the NOT groups). A total of 308 (21.4%) developed at least one episode of HAEC, including 76 (5.3%) who had two or more episodes. Between 2008 and 2018, the frequency of BT administration has increased from three to 20 hospitals with a frequency of administration between 2.2% and 16.2%. Prophylactic BT (PRO) was not associated with increased time to HAEC event on adjusted analysis. Conclusions Among children with HSCR undergoing primary pull-through surgery, prophylactic BT administration did not demonstrate increased time to first HAEC event. A better-powered study with prophylactic BT is required to determine the effect on HAEC occurrence and timing. Level of Evidence Level II (retrospective cohort study).

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.

Optimal treatment of children who develop appendicitis while undergoing treatment for an oncologic diagnosis has not been defined, in part due to theoretical concerns for an increased risk of postoperative wound complications. We hypothesized that synchronous oncologic diagnosis conferred no increased odds of developing a wound complication in pediatric patients undergoing appendectomy. Retrospective cohort study using the National Surgical Quality Improvement Program, Pediatric (2012–2017) of patients

Fluorescence-guided surgery (FGS) is an increasingly available and popular method of visual field augmentation. The basic premise of FGS entails injection of fluorescent indocyanine green (ICG) and subsequent detection with a near-infrared (NIR) camera. For pediatric surgical oncologists, FGS remains experimental but is a promising modality for identifying tumor margins, locating metastases, performing sentinel lymph node biopsies, protecting peritumoral structures of interest, and facilitating reconstruction. Familiarity with basic ICG pharmacokinetics and NIR detection optics is critical for surgeons wishing to judiciously use FGS, as its success is firmly grounded in a thorough understanding of its capabilities and limitations. In this practical guide, we outline several well-described and innovative FGS applications by disease type, including their methods of administration, modes of detection, and typical ICG dosing paradigms. LEVEL OF EVIDENCE: V.

Background Rates of long-term survival for children with pulmonary metastatic osteosarcoma are low, and complete surgical resection of all visible pulmonary metastases is necessary for long term survival. Surgical approaches for metastasectomy include thoracotomy and thoracoscopy, with the approach chosen influenced by training and institutional bias. Thoracotomy with manual palpation of lung surfaces can identify nodules not seen on preoperative imaging, but no clear survival benefit has been demonstrated compared to complete thoracoscopic resection of all visible nodules. Methods All member of the American Pediatric Surgical Association were surveyed, and 204 members responded. Results Thoracoscopy was the preferred approach of 34% of surgeons for patients with 3 unilateral nodules but only 21% for those with 5 unilateral nodules. Hospital volume did not correlate with operative approach. Localization strategies are used by 37% of surgeons who prefer thoracotomy and 64% who prefer thoracoscopy. Importantly, the vast majority of responding surgeons (84%) expressed a willingness to participate in a randomized controlled trial of thoracotomy versus thoracoscopy. Conclusion Findings of this survey of North American pediatric surgeons confirm both the need for, and interest in, a prospective trial to define optimal surgical management of children with osteosarcoma with limited pulmonary metastasis. Level of evidence V.

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: 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

Background Laparoscopic oophorectomy with tissue cryopreservation (OTC) for fertility preservation is usually performed prior to therapy. When fertility preservation is considered after prior open abdominopelvic tumor surgery there may be a perceived barrier to laparoscopic OTC. This study evaluates the feasibility of OTC with a laparoscopic approach after open surgery. Methods This is a single institution retrospective study from 2011 to 2019. Results Planned laparoscopic OTC was performed after open surgery in 17 of 113 patients. Median age was 4.2 years. The most common diagnoses were Wilms Tumor (35%) and neuroblastoma (35%). The most common procedures were nephrectomy (41%) and exploratory laparotomy with biopsy (35%). The median amount of time between open surgery and OTC was 29 days. Sixteen (94%) had a laparoscopic OTC. Regardless of operative technique, patients resumed therapy a median of 3 days after OTC. Conclusions Prior abdominopelvic surgery should not be a barrier to OTC. Laparoscopic OTC is feasible after a variety of open oncologic operations, regardless of time-interval between the procedures and without incurring a significant delay in resuming oncologic therapy.

Survival for children with cancer has improved significantly in recent decades, prompting an increasing emphasis on minimizing late effects of therapy, including infertility and premature gonadal insufficiency. The time interval after diagnosis and before therapy initiation can be stressful and overwhelming for patients and their families coming to terms with the implications of the diagnosis, but is also the optimal time to address oncofertility options. Pediatric surgeons are often an integral part of the care team for these patients during this vulnerable time period and play a key role in advocating for and performing oncofertility procedures. Children with cancer have both non-experimental and experimental fertility preservation options available depending on their pubertal status and a risk assessment performed based on their anticipated therapy. This review provides an oncofertility toolkit for pediatric surgeons to perform a risk assessment, counsel families on fertility preservation options, and establish an oncofertility program tailored to the resources available at their institutions.