Childhood cancer surgery in low- and middle-income countries.

This report summarizes the status of pediatric surgical oncology services in low- and middle-income countries. Factors such as surgical capacity and enablers, and barriers to providing pediatric surgical oncology services are discussed. A review of the literature was conducted to examine the evidence for the capacity of low- and middle-income countries to provide childhood cancer surgery services, focusing on general surgery. Unpublished, ongoing work and initiatives of international organizations are also described.

Management of Chyle Leak in Pediatric Surgical Oncology: A Systematic Review.

INTRODUCTION: Chyle leak, a rare complication, arises from damage to primary lymphatic vessels due to congenital factors or medical interventions, leading to conditions such as chylothorax and chylous ascites. Managing chyle leaks is challenging, especially in pediatric surgical oncology, often arising as postoperative complications. Treatment options range from conservative dietary adjustments to surgical interventions, depending on leak severity and patient condition. This systematic review examines the management of chyle leaks in pediatric surgical oncology, emphasizing both conservative and surgical approaches. METHODS: This systematic review involved extensive database searches (EMBASE, Web of Science, and PubMed) to identify relevant studies on chyle leak management in the pediatric population. The review included studies from 1982 to 2023 and focused on pediatric and adolescent patients, assessing various treatment approaches and outcomes. Nine articles composed of 163 patients (study population size ranging from 2 to 82 patients). Independent reviewers evaluated the selected studies for inclusion. RESULTS: Among 9 articles analyzed, 98.8% of pediatric patients initially received conservative management for chyle leaks, with 11.7% eventually requiring surgical intervention due to persistent leaks (8, 10, and 16 to 22). Neuroblastoma resection is associated with 20% to 40% rate of chyle leak, and the extent of lymphadenectomy has been identified as a risk factor for chyle leak. The study highlighted variability in clinical success rates based on conservative management approaches. DISCUSSION: Chyle leak, while rare, presents a complex challenge, especially in pediatric surgical oncology. Various causes and treatment options exist, with a preference for conservative management initially and surgical intervention in specific circumstances. Factors such as leak severity and patient condition guide the choice between approaches. However, the scarcity of comparative data and randomized trials in the pediatric population necessitates further research to establish optimal management strategies for chyle leaks. CONCLUSIONS: Conservative management of chyle leaks has proven to be the preferred approach in early stages of treatment, whereas surgical management could be the preferred choice in certain situations. Larger prospective studies are needed to further evaluate these results.

A subset of image-defined risk factors predict completeness of resection in children with high-risk neuroblastoma: An international multicenter study.

BACKGROUND: Image-defined risk factors (IDRFs) were promulgated for predicting the feasibility and safety of complete primary tumor resection in children with neuroblastoma (NB). There is limited understanding of the impact of individual IDRFs on resectability of the primary tumor or patient outcomes. A multicenter database of patients with high-risk NB was interrogated to answer this question. DESIGN/METHODS: Patients with high-risk NB (age <20 years) were eligible if cross-sectional imaging was performed at least twice prior to resection. IDRFs and primary tumor measurements were recorded for each imaging study. Extent of resection was determined from operative reports. RESULTS: There were 211 of 229 patients with IDRFs at diagnosis, and 171 patients with IDRFs present pre-surgery. A ≥90% resection was significantly more likely in the absence of tumor invading or encasing the porta hepatis, hepatoduodenal ligament, superior mesenteric artery (SMA), renal pedicles, abdominal aorta/inferior vena cava (IVC), iliac vessels, and/or diaphragm at diagnosis or an overlapping subset of IDRFs (except diaphragm) at pre-surgery. There were no significant differences in event-free survival (EFS) and overall survival (OS) when patients were stratified by the presence versus absence of any IDRF either at diagnosis or pre-surgery. CONCLUSION: Two distinct but overlapping subsets of IDRFs present either at diagnosis or after induction chemotherapy significantly influence the probability of a complete resection in children with high-risk NB. The presence of IDRFs was not associated with significant differences in OS or EFS in this cohort.

Current surgical approaches to pediatric renal tumors.

Pediatric renal tumors are among the most common pediatric solid malignancies. Surgical resection is a key component in the multidisciplinary therapy for children with kidney tumors. Therefore, it is imperative that surgeons caring for children with renal tumors fully understand the current standards of care in order to provide appropriate surgical expertise within this multimodal framework. Fortunately, the last 60 years of international, multidisciplinary pediatric cancer cooperative group studies have enabled high rates of cure for these patients. This review will highlight the international surgical approaches to pediatric patients with kidney cancer to help surgeons understand the key differences and similarities between the European (International Society of Pediatric Oncology) and North American (Children's Oncology Group) recommendations.

European Pediatric Surgeons' Association Survey on Timing of Inguinal Hernia Repair in Premature Infants.

AIM: This article evaluates the practice patterns of European Pediatric Surgeons' Association (EUPSA) members regarding the timing of inguinal hernia (IH) repair in premature infants. METHODS: Online survey containing 29 questions distributed to EUPSA members during January 2023. RESULTS: A total of 180 responds were received. Overall, IH repair prior to discharge was favored by 60% of respondents when there was a history of incarceration and 56% when there was not. In the case of very/extremely premature infants (< 32 weeks) with no history of incarceration, fewer (43%) respondents postpone the surgery until after discharge. The majority of respondents cited the risk of incarceration as the reason for advocating surgery prior to discharge, whereas a reduced risk of apnea was the most cited reason for respondents who prefer delayed surgery. Open approach under general anesthesia was favored by 54% of respondents, with 27% of them preferring open approach with spinal anesthesia. Laparoscopic surgery for premature infants is used in 11% while 7% of them preferred in all premature infants including extremely/very premature ones. Contralateral side evaluation was never done by 40% of respondents and 29% only performed it only during laparoscopic repair. The majority of respondents (77%) indicated that they have an overnight stay policy for premature infants < 45 weeks of gestation. CONCLUSION: There is variation in the practice patterns of pediatric surgeons in the treatment of IH in premature infants. Due to the concern for the high risk of incarceration, IH repair before discharge was the most prevalent practice. Lower risk of postoperative apnea was cited as the most common reason for delaying surgery. Randomized studies are required to establish the optimal timing for IH repair in premature infants.

Surgical Management of Wilms Tumors with Intravenous Extension: A Multicenter Analysis of Clinical Management with Technical Insights.

BACKGROUND: About 5% of Wilms tumors present with vascular extension, which sometimes extends to the right atrium. Vascular extension does not affect the prognosis, but impacts the surgical strategy, which is complex and not fully standardized. Our goal is to identify elements of successful surgical management of Wilms tumors with vascular extensions. PATIENTS AND METHODS: A retrospective study of pediatric Wilms tumors treated at three sites (January 1999-June 2019) was conducted. The inclusion criterion was the presence of a renal vein and vena cava thrombus at diagnosis. Tumor stage, pre and postoperative treatment, preoperative imaging, operative report, pathology, operative complications, and follow-up data were reviewed. RESULTS: Of the 696 pediatric patients with Wilms tumors, 69 (9.9%) met the inclusion criterion. In total, 24 patients (37.5%) had a right atrial extension and two presented with Budd-Chiari syndrome at diagnosis. Two died at diagnosis owing to pulmonary embolism. All patients received neoadjuvant chemotherapy and thrombus regressed in 35.6% of cases. Overall, 14 patients had persistent intra-atrial thrombus extension (58%) and underwent cardiopulmonary bypass. Most thrombi (72%) were removed intact with nephrectomy. Massive intraoperative bleeding occurred during three procedures. Postoperative renal insufficiency was identified as a risk factor for patient survival (p = 0.01). With a median follow-up of 9 years (range: 0.5-20 years), overall survival was 89% and event-free survival was 78%. CONCLUSIONS: Neoadjuvant chemotherapy with proper surgical strategy resulted in a survival rate comparable to that of children with Wilms tumors without intravascular extension. Clinicians should be aware that postoperative renal insufficiency is associated with worse survival outcomes.

Female Fertility Cryopreservation Outcomes in Childhood Cancer: A Systematic Review.

BACKGROUND AND AIMS: As survival rates in childhood cancer progress significantly, health outcomes in adulthood are pivotal to quality of life (QoL). Female patients undergoing chemotherapy and radiation for childhood cancer may experience adverse effects such as gonadotoxicity-related ovarian insufficiency. Ovarian tissue cryopreservation (OTC) is well studied in adults, but has only recently started to be explored in an effort to preserve fertility in young patients with childhood cancer. This systematic review aims to critically highlight contemporary outcomes of cryopreservation in female pediatric cancer patients. METHODS: A systematic search was conducted in PubMed, Embase, and Web of Science databases to identify English-language full text articles and abstracts published between 2004 and 2022 describing cryopreservation among female children (0-21 years old) with cancer. Abstracts and full-text articles were screened for inclusion. Subsequently, data from eligible studies was extracted and analyzed. Descriptive statistics were utilized to estimate overall outcomes of cryopreservation. RESULTS: Of 104 abstracts and 34 full-text articles, 12 studies were included. Data was collected from 7 world countries and involved some 612 pediatric and adolescent patients with malignant disease. Most common cancers included hematological malignant disease (81%), CNS nervous system malignant tumors (56%), and sarcomas (39%). Of the 6 studies with full reporting, OTC was undertaken in 501 patients, and 5.9% (30/501) of these patients underwent ovarian tissue transplantation (OTT). After OTT, 27 patients desired pregnancy and 33% (9/27) became pregnant. Six of these 9 patients (67%) had live births. CONCLUSIONS: Preliminary analysis showed that OTC has been successfully performed but not yet studied thoroughly in pediatric cancer patients in a longitudinal manner. This study has further shown that cryopreservation outcomes are mainly reported among adult patients living in high income countries, demonstrating a crucial need for long-term outcome studies focused on pediatric and prepuberal OTC, subsequent OTT, and potential pregnancy. This work is considered critical to aid standardize recommendations of fertility preservation in childhood cancer patients and to better inform the efficacy of these procedures to benefit patients in world nations of all fiscal income levels. LEVEL OF EVIDENCE: Level III.

Minimally invasive approach of paediatric neuroblastoma with thoracic vascular encasement.

ABSTRACT: Neuroblastoma (NB) is the most frequent paediatric extracranial solid tumour. The surgical management of these tumours in newborns changed recently, performing resections in cases with tumour size increase after birth. Minimally invasive procedures were mostly reported in cases without pre-operative image-defined risk factors (IDRFs), defined by vascular and organ involvement. Thoracoscopic resection represents a minority of the overall surgical procedures for neuroblastic tumour management, as the posterior mediastinum is one of the least frequent locations of NB. A thoracoscopic resection was performed on a 22-month-old child with a NB encasing the aorta and a 6-month-old child with the encasement of the left subclavian and vertebral artery. A step-by-step minimally invasive procedure was described, highlighting anatomical landmarks and dissection techniques. The described technique was performed in 130 min. Thoracoscopic resection provided a macroscopic resection without surgical complications and patient was discharged on the 3 rd post-operative day. The study shows a feasible and safe thoracoscopic approach for paediatric thoracic NB with IDRFs.

Congenital lung malformations: a nationwide survey on management aspects by the Italian Society of Pediatric Surgery.

INTRODUCTION: Over the years, congenital lung malformations (CLM) management remains a controversial topic in pediatric thoracic surgery. The Italian Society of Pediatric Surgery performed a national survey to study the current management variability among centers, trying to define national guidelines and a standardized approach of children with congenital lung malformations. METHODS: Following a National Society approval, an electronic survey including 35 items on post-natal management was designed, focusing on surgical, anesthesiology, radiology and pneumology aspects. The survey was conducted contacting all pediatric surgical units performing thoracic surgery. RESULTS: 39 pediatric surgery units (97.5%) participated in the study. 13 centers (33.3%) were classified as high-volume (Group A), while 26 centers (66.7%) were low volume (Group B). Variances in diagnostic imaging protocols were observed, with Group A performing fewer CT scans compared to Group B (p = 0.012). Surgical indications favored operative approaches for asymptomatic CLM and pulmonary sequestrations in both groups, while a wait-and-see approach was common for congenital lobar emphysema. Surgical timing for asymptomatic CLM differed significantly, with most high-volume centers operating on patients younger than 12 months (p = 0.02). Thoracoscopy was the preferred approach for asymptomatic CLM in most of centers, while postoperative long-term follow-up was not performed in most of the centers. CONCLUSION: Thoracoscopic approach seems uniform in asymptomatic CLM patients and variable in symptomatic children. Lack of uniformity in surgical timing and preoperative imaging assessment has been identified as key areas to establish a common national pattern of care for CLM.

Image-guided core-needle or surgical biopsy for neuroblastoma diagnosis in children: A systematic review and meta-analysis from the International Society of Pediatric Surgical Oncology (IPSO).

BACKGROUND: Image-guided core-needle biopsy (IGCNB) is a widely used and valuable clinical tool for tissue diagnosis of pediatric neuroblastoma. However, open surgical biopsy remains common practice even if children undergo more invasive and painful procedures. This review aims to determine the diagnostic accuracy and safety of IGCNBs in pediatric patients with neuroblastoma. METHODS: We conducted a systematic review of peer-reviewed original articles published between 1980 and 2023, by searching "pediatric oncology," "biopsy," "interventional radiology," and "neuroblastoma." Exclusion criteria were patients older than 18 years, studies concerning non-neurogenic tumors, case reports, and language other than English. Both the systematic review and meta-analysis were conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. RESULTS: A total of 533 abstracts articles were analyzed. Of these, eight retrospective studies met inclusion criteria (490 infants, 270 surgical biopsies [SB], 220 image-guided biopsies). Tissue adequacy for primary diagnosis (SB: n = 265, 98%; IGCNB: n = 199, 90%; p = .1) and biological characterization (SB: n = 186, 95%; IGCNB: n = 109, 89%; p = .15) was similar with both biopsy techniques, while intraoperative transfusion rate (SB: n = 51, 22%; IGCNB: n = 12, 6%; p = .0002) and complications (%) (SB: n = 58, 21%; IGCNB: n = 14, 6%; p = .005) were higher with surgical biopsy. Length of stay was similar in both groups; however, no additional data about concurrent diagnostic or treatment procedures were available in the analyzed studies. CONCLUSIONS: IGCNB is a safe and effective strategic approach for diagnostic workup of NB and should be considered in preferance to SB wherever possible.

Enhanced Recovery after Surgery Applied to Pediatric Laparoscopic Cholecystectomy for Simple Cholelithiasis: Feasibility and Teaching Insights.

BACKGROUND: Same-day discharge after a cholecystectomy is a common practice in the adult population and has been demonstrated as safe and viable for children as well. However, there is a lack of comprehensive teaching models for pediatric cholecystectomy. Drawing inspiration from standardized outpatient procedures, this study aimed to assess the clinical outcomes and feasibility of teaching programs and an Enhanced Recovery After Surgery (ERAS) protocol following ambulatory laparoscopic cholecystectomy in pediatric patients. METHODS: In 2015, an ERAS pathway for laparoscopic cholecystectomy (LC) was implemented, focusing on admission procedures, surgery timing, anesthetic choices, analgesia, postoperative feeding, mobilization, and pain assessment. Day-case surgery was not applicable for acute cholecystitis, choledochal lithiasis, sickle cell disease, and hereditary spherocytosis cases. The protocol was employed for a group of attending surgeons and fellows, as well as a group of residents under the supervision of experienced surgeons. A retrospective analysis was conducted to evaluate the feasibility and effectiveness of ambulatory cholecystectomy in children and its utilization in training pediatric surgical trainees. RESULTS: Between 2015 and 2020, a total of 33 patients were included from a cohort of 162 children who underwent LC, with 15 children operated on by senior surgeons and 18 by young surgeons. The primary diagnoses were symptomatic gallbladder lithiasis (n = 32) and biliary dyskinesia (n = 1). The median age at the time of surgery was 11.3 years (interquartile range (IQR) 4.9-18), and the median duration of surgery was 54 min (IQR 13-145). One intraoperative complication occurred, involving gallbladder rupture and the dissemination of lithiasis into the peritoneal cavity. Three patients (9%) required an overnight stay, while no postoperative complications or readmissions within 30 days were observed. ERAS was successfully implemented in 30 patients (91%). No significant differences in surgical outcomes were noted between senior and young surgeons. At an average follow-up of 55 months, no long-term sequelae were identified. CONCLUSIONS: These findings align with the current trend of increasing use of outpatient laparoscopic cholecystectomy and underscore its feasibility in the pediatric population. The application of a structured ERAS protocol appears viable and practical for training the next generation of pediatric surgeons. LEVEL OF EVIDENCE: Level III.

Identification of Pediatric Tumors Intraoperatively Using Indocyanine Green (ICG).

BACKGROUND: Fluorescence-guided surgery (FGS) with indocyanine green (ICG) is increasingly applied in pediatric surgical oncology. However, FGS has been mostly reported in case studies of liver or renal tumors. Applying novel technologies in pediatric surgical oncology is more challenging than in adult surgical oncology due to differences in tumor histology, biology, and fewer cases. No consensus exists on ICG-guided FGS for surgically managing pediatric solid tumors. Therefore, we reviewed the literature and discuss the limitations and prospects of FGS. METHODS: Using PRISMA guidelines, we analyzed articles on ICG-guided FGS for childhood solid tumors. Case reports, opinion articles, and narrative reviews were excluded. RESULTS: Of the 108 articles analyzed, 17 (14 retrospective and 3 prospective) met the inclusion criteria. Most (70.6%) studies used ICG to identify liver tumors, but the timing and dose of ICG administered varied. Intraoperative outcomes, sensitivity and specificity, were reported in 23.5% of studies. Fluorescence-guided liver resections resulted in negative margins in 90-100% of cases; lung metastasis was detected in 33% of the studies. In otolaryngologic malignancies, positive margins without fluorescence signal were reported in 25% of cases. Overall, ICG appeared effective and safe for lymph node sampling and nephron-sparing procedures. CONCLUSIONS: Despite promising results from FGS, ICG use varies across the international pediatric surgical oncology community. Underreported intraoperative imaging outcomes and the diversity and rarity of childhood solid tumors hinder conclusive scientific evidence supporting adoption of ICG in pediatric surgical oncology. Further international collaborations are needed to study the applications and limitations of ICG in pediatric surgical oncology.

Retroperitoneoscopic or Transperitoneal Approach for Neurogenic and Adrenal Tumors in Children? A Comparison on the Way to Enhanced Recovery in Pediatric Surgical Oncology.

BACKGROUND: Minimally invasive surgery is increasingly utilized for resection of neurogenic tumors in children. The minimally invasive retroperitoneoscopic approach was recently reported in children, but transperitoneal laparoscopy still remains the most common technique. The aim of this study is to compare a novel single-port retroperitoneoscopy (SPR) approach for pediatric neurogenic tumor resection with transperitoneal laparoscopic (TPL). METHODS: Patients undergoing minimally invasive resection of abdominal neurogenic tumors over 5 years at a single institution (from 2018 to 2022) were retrospectively reviewed. Tumor volume, stage, presence of image-defined risk factors (IDRFs), neoadjuvant chemotherapy, operative time, estimated blood loss (EBL), length of stay (LOS), complications, oral morphine equivalents per kilogram (OME/Kg), and time to chemotherapy were assessed and compared with SPR and TPL approaches. RESULTS: Eighteen and fifteen patients underwent TPL and SPR, respectively. No significant differences were found between the TPL and SPR approaches in terms of tumor characteristics and IDRFs. Patients who underwent SPR had a significantly faster recovery (p = 0.008) and less postoperative opioid use compared to those in TPL (p = 0.02), thus allowing an enhanced recovery after surgery (ERAS) protocol application. TPL and SPR approaches were performed in presence of IDRFs, respectively in 2 (11%) and 4 patients (27%), with a IDRFs-related conversion in one TPL procedure. Both approaches had one < Grade 3 Clavien Dindo complication, but not requiring further surgery. DISCUSSION: SPR approach can be considered as a safe and feasible minimally invasive approach for the resection of pediatric primary adrenal and neurogenic tumors. The retroperitoneoscopic approach performed using a single port technique represents a promising new frontier of ERAS application in pediatric surgical oncology. CONCLUSION: SPR is a viable surgical alternative in selected neurogenic abdominal tumors with limited IDRFs, thus allowing for the application of ERAS protocols in these patients. LEVEL OF EVIDENCE: Level III.