PURPOSE:Treatment of pediatric cancers often requires gonadotoxic therapies, making fertility preservation (FP) an essential component of comprehensive oncologic care. However, surgeon exposure to oncofertility care varies. This study evaluated pediatric surgeon knowledge, comfort, and perceptions regarding FP counseling and care. METHODS:A cross-sectional survey was administered to lead surgeon investigators from 52 sites across North America participating in the Pediatric Surgical Oncology Research Collaborative (PSORC) to evaluate surgeon perceptions on FP. Surgeon knowledge and comfort levels providing FP services to patients with cancer diagnoses were ascertained. RESULTS:Ninety-three percent of invited PSORC surgeons completed the survey. Most respondents desired further educational opportunities in pediatric oncofertility (92%) and supported incorporation of FP training into fellowship education (83%). Among surgeons without a dedicated FP program, 96% were interested in establishing one, and 43% reported active program development. A minority (10.4%) of respondents reported no fertility preservation counseling at all at their institutions. Surgeons at institutions with dedicated FP programs were significantly more comfortable performing FP procedures (p = 0.003) and risk stratifying patients (p = 0.042). They were also more knowledgeable about FP options (p = 0.003), outcomes of ovarian tissue cryopreservation (OTC) and testicular tissue cryopreservation (TTC) for fertility and hormone restoration (p = 0.005), and options for future hormone and fertility restoration (p = 0.002). DISCUSSION:Significant opportunities exist to expand surgeon education, institutional resources, and multidisciplinary FP infrastructure. Respondents were highly motivated to increase educational opportunities and resources to improve fertility care for pediatric oncology patients.

INTRODUCTION:Despite the broader adoption of minimally invasive liver surgery (MILS) for adults, its application in the pediatric population has been mainly documented in single-center reports. We aimed to summarize the current state of evidence of MILS in children. METHODS:We systematically reviewed PubMed, Scopus, Web of Science, and Cochrane Library databases according to the PRISMA statement for children (<18 years) undergoing MILS (end-of-search date: 03/03/2024). The National Cancer Database (NCDB) was queried for children (<18 years) undergoing MILS (2010-2021). RESULTS:Forty-one articles reporting on 211 patients were included. The approach was laparoscopic in 91.5%, robotic in 5.7%, and hand-assisted 2.8%. The mean age was 5.4 ± 4.4 years, the mean lesion size was 6.2 ± 3.4 cm, and the most common indications were hepatoblastoma (28.9%) and hydatid cyst (27.0%). Most underwent cyst surgery (43.0%), followed by minor liver resection (37.7%). Five (2.4%) had conversion to open. The mean operative time was 162.1 ± 99.1 min and mean length of stay was 6.4 ± 4.9 days. Over a mean follow-up of 22.9 ± 24.6 months, one patient died (0.5%). Forty-six children underwent MILS in NCDB (45 laparoscopic, 1 robotic). The mean age was 4.7 ± 5.7 years and mean tumor size was 1.0 ± 1.3 cm. Most underwent wedge/segmental resection (47.8%). Five (10.9%) had conversion to open and mean length of stay was 6.3 ± 5.0 days. CONCLUSION:This first systematic review and NCDB analysis establishes feasibility benchmarks of MILS in children for both benign and malignant liver lesions. MILS is associated with 2-11% conversion rate and mean length of stay of 6 days. LEVEL OF EVIDENCE:Level III evidence.

PURPOSE:This study analyzed the effect of pulmonary metastatic burden at diagnosis and response to chemotherapy on disease-free and overall survival in patients with hepatoblastoma (HB), hypothesizing that long-term survival can be achieved in the setting of extensive pulmonary metastatic disease requiring metastasectomy. METHODS:An analysis of a multicenter dataset including children with HB and pulmonary metastatic disease at diagnosis was performed. Patients who presented with >10 pulmonary metastases were defined as having extensive metastatic disease. Kaplan Meier and univariate Cox Proportional Hazards analyses assessed disease-free survival (DFS) and overall survival (OS), analyzing the effect of pulmonary metastatic disease extent and modality of clearance. RESULTS:Among 315 patients with HB, pulmonary metastatic disease at diagnosis (n = 62) was associated with poorer DFS and OS (both p > 0.05). Of patients with pulmonary metastases, 23 (37 %) had extensive disease. Clearance of metastases occurred in 59 (95 %) patients: 32 (54 %) with chemotherapy alone and 27 (46 %) with chemotherapy and metastasectomy. Survival was similar between patients with >10 vs. ≤10 lung metastases and between those who had clearance of lung metastases by chemotherapy alone vs. chemotherapy and metastasectomy (all p > 0.05). Among 16 patients who required liver transplantation, all patients who underwent surgical clearance of metastatic disease were alive at last follow-up. CONCLUSION:Among patients with HB who had pulmonary metastatic disease at diagnosis, long-term survival is possible regardless of the extent of pulmonary disease. A combination of neoadjuvant chemotherapy and metastasectomy can be utilized to achieve lung clearance.

BACKGROUND:Sentinel lymph node (SLN) biopsy is a critical staging tool in skin and soft tissue cancers. Although technetium lymphoscintigraphy is the most common adjunct, indocyanine green (ICG) demonstrates a promising alternative in pediatric, adolescent, and young adult (AYA) skin and soft tissue cancers. METHODS AND OBJECTIVES:Evaluate the safety and efficacy of ICG in SLN biopsy (SLNB) in diverse pediatric and AYA solid cancers in a multi-institutional environment. This is a prospective, observational study conducted at 13 member institutions in the Pediatric Surgical Oncology Research Collaborative between 2019 and 2023. Eligible patients underwent SLNB for primary or recurrent solid skin and soft tissue tumors. Peritumoral ICG was administered intraoperatively. Outcomes included SLN detection rates, histopathology of excised specimens, and surgeon-reported assessment of utility. Safety was assessed through adverse event reporting. RESULTS:Forty SLNB procedures yielded 84 specimens: 13 melanoma (32.5%), 12 squamous cell carcinoma (30%), 11 non-rhabdomyosarcoma soft tissue sarcoma (27.5%), two rhabdomyosarcoma (5%), one Ewing sarcoma (2.5%), and one malignant peripheral nerve sheath tumor (2.5%). Histologic evaluation identified 81 lymph nodes, 75 benign (89.3%) and six malignant (7.1%); three specimens were adipose tissue (3.6%). Of 81 lymph nodes, 76% were ICG-positive. ICG had a sensitivity of 79.5%, a specificity of 33%, and a positive predictive value of 96%. All malignant nodes were ICG-avid. There were no adverse reactions to ICG at 30 days. CONCLUSIONS:ICG is a safe and effective adjunct for SLNB in pediatric and AYA soft tissue and skin malignancies.

INTRODUCTION:Although relatively rare, pediatric pancreatic tumors have been increasingly reported in the literature. Our aim was to systematically review the literature on the clinical characteristics, management, and outcomes of pediatric pancreatic tumors. METHODS:We performed a systematic review of the MEDLINE, Scopus, Web of Science, and Cochrane Library databases (end-of-search date: June 2022) according to the Preferred Reporting Items for Systematic Reviews and Meta-analyses statement. RESULTS:Sixty-four studies reporting in 1021 pediatric patients (< 18 y) were included. The most common tumor histology was solid pseudopapillary neoplasm (SPN) (70.4%), followed by pancreatoblastoma (12.7%), and pancreatic neuroendocrine tumor (pNET) (9.7%). Most children were symptomatic (range: 71.4%-100.0%) and presented with abdominal pain, palpable mass, or nausea and vomiting. The head of the pancreas was the most common location for most tumors (range: 31.3%-70.0%). About 14% of SPNs were characterized as malignant. Surgical resection was performed in 93.5% of patients, and the most common operations were pancreaticoduodenectomy (32.9%), distal pancreatectomy (42.1%), and enucleation (12.2%). Margin-negative resection was achieved in 92.7%. Neoadjuvant chemotherapy was administered in 8.8%. The most common complications observed were fistula in 12.9%, fluid collection in 3.4%, and exocrine insufficiency in 2.7%. The 5-y survival rates were 97.4% for SPNs, 36.7% for pancreatoblastomas, and 75.0% for pNETs. Recurrence was seen in 4.8% of SPNs, 20.5% of pancreatoblastomas, and 4.4% of pNETs. CONCLUSIONS:Among the common pediatric pancreatic tumors, pancreatoblastoma was associated with the worst prognosis and highest recurrence rate. Complete surgical resection remains the mainstay of treatment for pediatric pancreatic tumors with relatively low reported morbidity.

BACKGROUND:Real-time intraoperative discrimination of malignant tissue during pediatric solid tumor resection is limited, constraining margin optimization; the utility of indocyanine green (ICG) fluorescence in this population remains incompletely defined. STUDY DESIGN:Prospective, observational, multi-institutional study (2019-2023) of pediatric, adolescent, and young adult patients undergoing resection of intra-abdominal solid malignancies following preoperative ICG administration. The primary endpoint was diagnostic accuracy of ICG fluorescence for tumor-containing specimens; secondary endpoints included association with margin status and surgeon-perceived intraoperative benefit. RESULTS:Forty-three operations in 42 patients yielded 55 specimens, of which 47 (85%) were malignant. Diagnostic performance was highest in hepatic tumors (sensitivity 83%, positive predictive value 83%) and lower in adrenal and heterogeneous tumor types. ICG avidity was not associated with improved negative margin rates (86% vs 75%, p=0.47). Surgeons reported intraoperative benefit in 67% of cases, most commonly improved visualization. No ICG-related adverse events occurred. CONCLUSIONS:ICG fluorescence provides reliable tumor identification in hepatic malignancies but does not correlate with margin-negative resection, indicating limited utility for margin guidance. These data support tumor-specific application and further investigation of optimized fluorescence strategies, including dosing, timing, and alternative fluorophores, in pediatric oncologic surgery.

BACKGROUND:Renal medullary carcinoma (RMC) is an aggressive tumor associated with sickle cell trait. Despite treatment advances for other rare renal tumors, RMC survival remains poor. We aimed to describe the contemporary management and survival of children and adults with RMC. PROCEDURE:In this multicenter retrospective cohort study, Pediatric Surgical Oncology Research Collaborative sites searched their databases for patients diagnosed with RMC (2000-2022). Descriptive statistics were calculated and survival analyses performed using Kaplan-Meier and Cox regression. RESULTS:Thirty-four patients with RMC were identified. Median age was 19 years (IQR: 15-28; range: 7-52). Most were male (24/34; 71%), Black (27/32; 84%), had sickle cell trait or disease (30/33; 91%), presented with metastatic disease (27/34; 79%), and were symptomatic at presentation (32/34; 94%). Median overall survival (OS) was 24 months from diagnosis (16 months for children, 28 months for adults, p = 0.6). Receipt of platinum-based chemotherapy (23/34; 68%) was associated with significantly higher OS than other regimens (35 vs. 5 months, p < 0.001). Nephrectomy (24/34; 71%) was associated with significantly improved OS compared with non-operative management (34 vs. 7 months, p = 0.001). Immunotherapy, targeted therapy, or radiation therapy were not associated with significant differences in OS, nor were age, sex, race, sickle cell status, SMARCB1/INI-1, stage, nephrectomy approach, retroperitoneal lymph node dissection, gross residual disease, margins, or tumor size. CONCLUSIONS:RMC survival remains poor despite newer therapies. Nephrectomy and platinum-based chemotherapy should be considered in locally advanced and metastatic disease. Coordinated international cooperative group studies are needed to meaningfully improve RMC survival.

INTRODUCTION:Solid pseudopapillary neoplasm (SPN) comprises 2% of pancreatic tumors. We aimed to examine the clinical characteristics, management, and outcomes of SPN in children and adults using US-based national cancer registry data. METHODS:In this retrospective National Cancer Database analysis, children (<18 y) and adults (≥18 y) with SPN were included (2004-2020). RESULTS:A total of 1325 patients (231 children, 1094 adults) with SPN were identified. There were no differences between groups regarding tumor size, metastasis, or stage. Most patients in both groups underwent surgical treatment (96.5% versus 92.1%, P = 0.056), whereas a smaller proportion of children received chemotherapy compared to adults (0.9% versus 4.2%, P = 0.01). The 10-year overall survival rate was 99.1% in children versus 88.0% in adults. In multivariable Cox regression, when adjusted for tumor location and receipt of chemotherapy and radiation, adulthood (hazard ratio [HR]: 5.32, 95% confidence interval [95% CI]: 1.26-22.49, P = 0.02), Charlson-Deyo score ≥2 (HR: 3.04, 95% CI: 1.21-7.63, P = 0.02) compared to 0, stage II (HR: 2.48, 95% CI: 1.27-4.82, P = 0.008), stage III (HR: 6.82, 95% CI: 1.50-31.02, P = 0.01), and stage IV (HR: 5.94, 95% CI: 2.49-14.16, P < 0.001) compared to stage I were associated with an increased risk of mortality, whereas Hispanic compared to White patients (HR: 0.44, 95% CI: 0.21-0.95, P = 0.04) and surgically treated patients had a decreased risk of mortality (HR: 0.10, 95% CI: 0.05-0.21, P < 0.001). CONCLUSIONS:Despite similar stage presentation, children with SPN exhibit prolonged survival compared to adults. Most patients underwent surgical treatment, whereas children were less likely to undergo chemotherapy than adults.

INTRODUCTION:A quality improvement initiative targeting emergency transfer (ET) rate reduction resulted in the development and implementation of the safety officer (SO) intervention. SO leveraged on-site pediatric hospitalists to promote institutional safety practices around care escalation for all patients admitted to the target unit, including those admitted to surgical subspecialty services. METHODS:An interdisciplinary team developed the SO intervention, which included ad hoc support of communication algorithms and attendance at all Rapid Response Team (RRT) activations on the target unit. To evaluate SO implementation, we applied the Reach, Effectiveness, Adoption, Implementation, and Maintenance framework. We obtained data through postimplementation surveys, manual case review, and electronic health record reporting. We analyzed survey data using descriptive statistics and clinical data using statistical process control charts. RESULTS:Most SOs completed training (85%). The survey response rate was 30%; 40% were hospitalist clinicians, 24% were RRT participants, 18% were surgeons, and 18% were bedside nurses. Many respondents believed SO improved the unit's safety culture (56%). Nurses reported improved ability to escalate care (94%), and intensive care unit clinicians reported enhanced RRT practices. SO involvement in RRTs increased from 0% to 78%. Non-SO clinicians did not believe SO detracted from ownership of their patients (97%), and SOs felt they could effectively balance competing responsibilities (92%). We did not observe a change in ET rates. CONCLUSION:By implementing the SO intervention, we successfully integrated hospitalists for managing care escalations and improved perceived safety culture. Further study is needed to evaluate whether SO can improve rare adverse events like ETs.

Background: Lymph node status is a prognostic factor in Wilms tumor, and adequate lymph node sampling is strongly recommended. This study investigates the impact of lymph node ratio (LNR) (number of positive to examined lymph nodes) on overall survival in children with resected Wilms tumors. Methods: This retrospective National Cancer Database analysis included children (<18 years) who underwent resection with lymph node sampling for unilateral, non-metastatic Wilms tumor. Results: Among 2206 patients, the median age was three years, the median tumor size was 10.5 cm, and the median number of examined nodes was five. A total of 82.1% of patients had an LNR of 0, 5.4% had an LNR < 0.2, and 12.5% had an LNR ≥ 0.2. In multivariable Cox regression, LNR ≥ 0.2 was associated with worse survival (HR = 1.75, 95%CI: 1.03–2.97, p = 0.04), along with increasing age (HR = 1.11, 95%CI: 1.05–1.17, p < 0.001) and tumor size (HR = 1.03, 95%CI: 1.00–1.06, p = 0.03). Conclusions: LNR is an independent prognostic factor in Wilms tumor and may refine risk stratification and guide treatment decisions.

INTRODUCTION:Children with hepatoblastoma (HB) and other solid tumors frequently undergo intraoperative blood transfusion (IBT) with unknown impact on oncologic outcomes and scant data to guide transfusion in this population. This study tested the hypothesis that IBT is associated with poorer survival in children with HB. METHODS:A multicenter retrospective observational study of patients aged <18 years with HB who underwent primary tumor resection, including liver transplantation, from 2010 to 2019 was performed at 19 institutions. The association of IBT with risk of recurrence and death were determined using propensity score reweighted (PSR) Cox proportional hazards regression analyses. RESULTS:There were 338 patients identified who met inclusion criteria and had sufficient data for inclusion. Of those, 257 (76%) received IBT, including 253 (98%) who received packed red blood cells (pRBC), 84 (33%) who received plasma, and 28 (11%) who received platelets. IBT was associated with higher pretreatment extent of disease (p < 0.001), presence of annotation factors (+VPEFR: 50%, n = 129 vs. 37%, n = 30, p = 0.035), and complex resections (extended, meso-, or total hepatectomy: 54%, n = 139 vs. 27%, n = 22, p < 0.001); these differences were mitigated after applying propensity score weighting. Patients who received IBT had greater postoperative hemoglobin (g/dL) (median 10 (I8-11) vs. 9 (8-10), p = 0.013) and required more postoperative plasma and platelet transfusions (p < 0.05). Median follow-up was 4.4 (2.0-8.3) years. Compared with non-IBT patients, those with IBT had higher incidence of death (PSR HR 2.35, 95% CI 1.10-5.02). Recurrence did not significantly differ across groups (PSR HR = 0.82, 95% CI 0.45-1.48). CONCLUSION:IBT was associated with greater hazard of death. Postoperative hemoglobin levels suggested that unnecessary transfusions occurred and a pRBC-focused approach to IBT led to coagulopathy. Development of optimal transfusion strategies for HB are needed to minimize unnecessary transfusions.

BACKGROUND:Pediatric adrenocortical carcinoma (ACC) is a rare and aggressive cancer. The appropriateness of minimally invasive surgery (MIS), role of nodal dissection, and aggressiveness of surgery in patients with metastatic disease remain poorly understood. PATIENTS AND METHODS:We performed a retrospective review of patients < 18 years with ACC treated from 2012 to 2022 at 22 institutions participating in the Pediatric Surgical Oncology Research Collaborative. Data collected included demographics, clinical presentation, genetic predisposition, tumor characteristics, surgical approach, adjuvant treatment, and survival outcome. Survival probabilities were estimated with Kaplan-Meier methods. RESULTS:In all, 69 patients were included (median age: 8.4 years; 58% female), and 28% had Li-Fraumeni syndrome. At diagnosis, 36% had Stage IV disease and 28% had Stage I disease. A total of 55 (83%) patients underwent open adrenalectomy, and 11 (17%) underwent MIS. MIS approaches were employed for significantly smaller tumors (median: 4.4 cm, 38 g) compared with open cases (median: 9.6 cm, 246 g; p < 0.001), and none had tumor spillage (versus 22% in open cases; p = 0.087). Lymph node sampling was performed in 44% of open cases (mean yield: 2.96); no lymph nodes were sampled in MIS cases (p = 0.008). The 5 year overall survival (OS) for the entire cohort was 65.9%, and 5-year event-free survival (EFS) was 54.1%. Patients without metastases had significantly better survival than those with more than five metastatic lesions (5 year OS: 84.5% versus 11.9%; p < 0.001). CONCLUSIONS:Complete surgical resection remains paramount for cure in children with ACC. Despite aggressive attempts at tumor clearance, outcomes in children with metastatic disease remain poor. Nodal dissection remains infrequently and incompletely performed, limiting conclusions on impact.

OBJECTIVE:To evaluate for disparities in surgical care among US children with hepatoblastoma (HB) and hepatocellular carcinoma (HCC). STUDY DESIGN:In this retrospective National Cancer Database study (2004-2015), children aged <18 years with HB or HCC were included. Multivariable mixed-effects logistic regression was used to evaluate the association of sociodemographic factors (age, sex, race and ethnicity, insurance status, income, proximity to treating hospital) with the odds of undergoing surgical treatment after adjusting for disease-related factors (tumor size, metastasis, comorbidities) and hospital-level effects. Subgroup analyses by tumor histology were performed. RESULTS:A total of 811 children were included (HB: 80.9%; HCC: 19.1%), of which 610 (75.2%) underwent surgical treatment. Following adjustment, decreased odds of undergoing surgical treatment were associated with Black race (OR: 0.46 vs White, 95% CI [95% CI]: 0.26-0.80, P = .01), and having Medicaid (OR: 0.58 vs private, 95% CI: 0.38-0.88, P = .01) or no insurance (OR: 0.33 vs private, 95% CI: 0.13-0.80, P = .02). In children with HB, Black race was associated with decreased odds of undergoing surgical treatment (OR: 0.47 vs White, 95% CI: 0.25-0.89, P = .02). In children with HCC, Medicaid (OR: 0.10 vs private, 95% CI: 0.03-0.35, P < .001), or no insurance status (OR: 0.10 vs private, 95% CI: 0.01-0.83, P = .03) were associated with decreased odds of undergoing surgical treatment. Other than metastatic disease, no additional factors were associated with likelihood of surgical treatment in any group. CONCLUSIONS:Black race and having Medicaid or no insurance are independently associated with decreased odds of surgical treatment in children with HB and HCC, respectively. These children may be less likely to undergo curative surgery for their liver cancer.

BACKGROUND:Undifferentiated embryonal sarcoma of the liver (UESL) is a rare tumor for which there are few evidence-based guidelines. The aim of this study was to define current management strategies and outcomes for these patients using a multi-institutional dataset curated by the Pediatric Surgical Oncology Research Collaborative.METHODS:Data were collected retrospectively for patients with UESL treated across 17 children's hospitals in North America from 1989 to 2019. Factors analyzed included patient and tumor characteristics, PRETEXT group, operative details, and neoadjuvant/adjuvant regimens. Event-free and overall survival (EFS, OS) were the primary and secondary outcomes, respectively.RESULTS:Seventy-eight patients were identified with a median age of 9.9 years [interquartile range [IQR): 7-12]. Twenty-seven patients underwent resection at diagnosis, and 47 patients underwent delayed resection, including eight liver transplants. Neoadjuvant chemotherapy led to a median change in maximum tumor diameter of 1.6 cm [IQR: 0.0-4.4] and greater than 90% tumor necrosis in 79% of the patients undergoing delayed resection. R0 resections were accomplished in 63 patients (81%). Univariate analysis found that metastatic disease impacted OS, and completeness of resection impacted both EFS and OS, while multivariate analysis revealed that R0 resection was associated with decreased expected hazards of experiencing an event [hazard ratio (HR): 0.14, 95% confidence interval (CI): 0.04-0.6]. At a median follow-up of 4 years [IQR: 2-8], the EFS was 70.0% [95% CI: 60%-82%] and OS was 83% [95% CI: 75%-93%].CONCLUSION:Complete resection is associated with improved survival for patients with UESL. Neoadjuvant chemotherapy causes minimal radiographic response, but significant tumor necrosis.

Background Historically, surgical biopsy (SB) for diagnosis of pediatric solid tumors was considered necessary to provide adequate tissue for histologic and molecular analysis. Less invasive biopsy techniques such as image-guided core needle biopsy (CNB), have shown comparable accuracy with decreased morbidity in some adult studies. However, data regarding the safety and efficacy of CNB in pediatric tumors is limited. This study’s aim was to assess the overall rate of successful diagnosis and safety of CNB compared to SB in children with malignancies. Methods A PRISMA compliant systematic review was performed in MEDLINE via PubMed, Embase and CINAHL Plus database searches from 2010-2023. Studies were included with relevance to the following clinical question: For children with concern for malignancy requiring biopsy for diagnosis, how does CNB compare to open or laparoscopic/thoracoscopic SB in terms of safety and diagnostic efficacy? Data for patients ≤ 21 years requiring biopsy for diagnosis of liver tumors, neuroblastoma (NB), soft tissue sarcoma (STS), and lymphoma were included. Results Twenty-seven studies including 2477 patients met inclusion criteria, with 2065 undergoing CNB and 412 SB. Of the 2477 patients, 820 patients had NB, 307 liver tumors, 96 STS, 151 lymphoma, and 1103 patients were from studies that included multiple diagnoses. The average complication rate for CNB was 2.9% compared to 21.4% for SB (p<0.001). Bleeding was the most common complication in both groups, but significantly higher after SB (22.1% vs 2.3%) (p<0.001). CNB was diagnostic in 90.8% of patients compared to 98.8% who underwent SB (p<0.001). Conclusions Rates of successful diagnosis were greater than 90% for both CNB and SB, though significantly higher for SB. Conversely, complication rates were close to ten times higher after SB compared to CNB. Given its relatively lower risk profile, CNB can be a safe and useful diagnostic tool for children with solid malignancies. Research focused on enhancing CNB’s diagnostic accuracy while maintaining low morbidity should be further explored. Level of Evidence Treatment study, Level III

Halix, Sarah J MD; Robbins, Alexandria J MD; Cameron, Danielle B. MD, MPH; Baertschiger, Reto M. MD, PhD; Roach, Jonathan MD; Brown, Erin G. MD; Aldrink, Jennifer H. MD; Rodeberg, David A. MD; Cost, Nicholas MD; Snaman, Jennifer MD; Le, Hau D MD; on behalf of the American Pediatric Surgical Association Cancer Committee Author Information

Two percent of pediatric malignancies arise primarily in the liver; roughly 60% of these cancers are hepatoblastoma (HB). Despite the rarity of these cases, international collaborative efforts have led to the consistent histological classification and staging systems, which facilitate ongoing clinical trials. Other primary liver malignancies seen in children include hepatocellular carcinoma (HCC) with or without underlying liver disease, fibrolamellar carcinoma (FLC), undifferentiated embryonal sarcoma of the liver (UESL), and hepatocellular neoplasm not otherwise specified (HCN-NOS). This review describes principles of surgical management of malignant pediatric primary liver tumors, within the context of comprehensive multidisciplinary care.

Synoptic operative notes for pediatric surgical oncology provide standardized and structured documentation of surgical procedures performed on pediatric patients with cancer. These reports capture essential details such as preoperative diagnosis, intraoperative findings, surgical technique, and tumor characteristics in a concise and uniform format. By promoting consistency, accuracy, and completeness in reporting, synoptic operative notes facilitate effective communication among multidisciplinary healthcare teams, enhance quality assurance efforts, and streamline data extraction for research purposes. The integration of synoptic reporting within electronic medical record systems further enhances accessibility and usability, ensuring efficient documentation practices and improved patient care outcomes in pediatric surgical oncology.