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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.

To assess the preoperative imaging impression and surgeon diagnostic accuracy for pediatric ovarian mature cystic teratomas (MCTs) DESIGN: Retrospective review SETTING: Eleven pediatric hospitals PARTICIPANTS: Patients ages 2 to 21 who underwent surgical management of an ovarian neoplasm or adnexal torsion with an associated ovarian lesion INTERVENTION: None MAIN OUTCOME MEASURES: Preoperative imaging impression, surgeon diagnosis, tumor markers, and pathology RESULTS: Our cohort included 946 females. Final pathology identified 422 (45%) MCTs, 405 (43%) other benign pathologies, and 119 (12%) malignancies. Preoperative imaging impression for MCTs had a 70% sensitivity, 92% specificity, 88% positive predictive value (PPV), and 79% negative predictive value (NPV). For the preoperative surgeon diagnosis, sensitivity was 59%, specificity 96%, PPV 92%, and NPV 74%. Some measures of diagnostic accuracy were affected by the presence of torsion, size of the lesion on imaging, imaging modality, and surgeon specialty. Of the 352 masses preoperatively thought to be MCTs, 14 were malignancies (4%). Eleven patients with inaccurately diagnosed malignancies had tumor markers evaluated and 82% had at least 1 elevated tumor marker, compared with 49% of those with MCTs.Diagnostic accuracy for the preoperative imaging impression and surgeon diagnosis is lower than expected for pediatric ovarian MCTs. For all ovarian neoplasms, preoperative risk assessment including a panel of tumor markers and a multidisciplinary review is recommended. This process could minimize the risk of misdiagnosis and improve operative planning to maximize the use of ovarian-sparing surgery for benign lesions and allow for appropriate resection and staging for lesions suspected to be malignant.

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.

Describe the current practice patterns and diagnostic accuracy of frozen section (FS) pathology for children and adolescents with ovarian masses DESIGN: Prospective cohort study from 2018 to 2021 SETTING: Eleven children's hospitals PARTICIPANTS: Females age 6-21 years undergoing surgical management of an ovarian mass INTERVENTIONS: Obtaining intraoperative FS pathology MAIN OUTCOME MEASURE: Diagnostic accuracy of FS pathology RESULTS: Of 691 patients who underwent surgical management of an ovarian mass, FS was performed in 27 (3.9%), of which 9 (33.3%) had a final malignant pathology. Among FS patients, 12 of 27 (44.4%) underwent ovary-sparing surgery, and 15 of 27 (55.5%) underwent oophorectomy with or without other procedures. FS results were disparate from final pathology in 7 of 27 (25.9%) cases. FS had a sensitivity of 44.4% and specificity of 94.4% for identifying malignancy, with a c-statistic of 0.69. Malignant diagnoses missed on FS included serous borderline tumor (n = 1), mucinous borderline tumor (n = 2), mucinous carcinoma (n = 1), and immature teratoma (n = 1). FS did not guide intervention in 10 of 27 (37.0%) patients: 9 with benign FS underwent oophorectomy, and 1 with malignant FS did not undergo oophorectomy. Of the 9 patients who underwent oophorectomy with benign FS, 5 (55.6%) had benign and 4 (44.4%) had malignant final pathology.FSs are infrequently utilized for pediatric and adolescent ovarian masses and could be inaccurate for predicting malignancy and guiding operative decision-making. We recommend continued assessment and refinement of guidance before any standardization of use of FS to assist with intraoperative decision-making for surgical resection and staging in children and adolescents with ovarian masses.

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.