BACKGROUND Preoperative evaluation of Image Defined Risk Factors (IDRFs) in neuroblastoma (NB) is crucial for determining suitability for upfront resection or tumor biopsy. IDRFs are linked with a higher potential morbidity at operation and lessen the chance of complete tumor resection. The IDRFs do not all carry the same weight in predicting tumor complexity and surgical risk. In this study we aimed to assess and categorize the degrees of surgical complexity (Surgical Complexity Index, SCI) in NB resection.  PROCEDURE A panel of 15 surgeons was involved in an electronic Delphi consensus survey to identify and score a set of shared items predictive and/or indicative of surgical complexity, including the number of preoperative IDRFs. Risk categories included - (a) Standard risk; (b) Moderate risk; (c) High risk; (d) Very high risk. A shared agreement included the achievement of at least 75% consensus focused on a single category or, alternatively, on the sum between the prevailing category and an immediately closest one. RESULTS After 3 Delphi rounds, agreement was established on 25/27 items (92.6%). A severity score was established for each item ranging from 0 to 3 with an overall SCI range varying from a minimum score of zero to a maximum score of 29 points for any given patient.  CONCLUSIONS A consensus on a SCI to stratify the risks related to tumor resection was established by the panel experts. This index will now be deployed to critically assign a better severity score to IDRFs involved in NB surgery.

Background: Thyroid gland malignancy is rare in pediatrics (0.7% of tumors); only 1.8% are observed in patients < 20 yrs with a higher prevalence recorded in females and adolescents. Risk factors include genetic syndromes - MEN disorders, autoimmune disease and ionizing radiation exposure. Radiotherapy is also linked with increased risk of secondary thyroid cancers. The present study describes the clinical features and surgical outcomes of primary and secondary thyroid tumors. Methods: Institutional data was collected on pediatric patients with thyroid cancer during 2000 - 2020 from 8 International Surgical Oncology centers. Statistical analysis was performed using GraphPad Prism. Results: Of 255 cases of thyroid cancer, only 13 (5.1%) were secondary tumors. Primary thyroid malignancies were more likely to be multifocal in origin (odds ratio [OR] 1.993, 95% confidence interval [CI] 0.7466-5.132, p 0.2323), had bilateral glandular location (OR 2.847, 95% CI 0.6835-12.68, p 0.2648) and proved metastatic at 1st diagnosis (OR 1.259, 95% CI 0.3267-5.696 p>0.999). Secondary tumors showed a higher incidence of disease relapse (OR 1.556, 95% CI 0.4579-5.57, p 0.4525) and surgical morbidity (OR 2.042, 95% CI 0.7917-5.221, p 0.1614) including hypoparathyroidism and recurrent laryngeal nerve injury. Overall survival (OS) was 99% at 1 year and 97% after 10 years. No EFS differences were evident with primary vs. secondary tumors (Chi square 0.7307, p 0.39026). Conclusions: This multicenter study demonstrates excellent survival for pediatric thyroid malignancy. Secondary tumors exhibit greater disease relapse (15.8% vs 10.5%) and a higher incidence of surgical related complications (36.8% vs 22.2%).

Objective Ovarian mature teratoma (OMT) is a common ovarian tumor found in the pediatric population. In 10% to 20% of cases, OMT occurs as multiple synchronous or metachronous lesions on ipsi- or contralateral ovaries. Ovarian sparing surgery (OSS) is recommended to preserve fertility, but total oophorectomy (TO) is still performed. This study reviews the clinical data of patients with OMT, and analyzes risk factors for second events. Design A retrospective review of all girls under 18 years of age with OMTs was performed. Data on clinical features, imaging, laboratory studies, surgical reports, follow-up second events and their management were retrieved. Results Overall, 350 children were identified. Eighteen patients (5%) presented with a synchronous bilateral form at diagnosis. Surgery was performed by laparotomy (85%) and laparoscopy (15%). OSS and TO were performed in 59% and 41% of cases respectively. Perioperative tumor rupture occurred in 23 cases, independently of the surgical approach. Twenty-nine second events occurred (8.3%) in a median time of 30.5 months from diagnosis (ipsilateral: 8 cases including one malignant tumor, contralateral: 18 cases, both ovaries: 3 cases). A large palpable mass, bilateral forms at diagnosis and perioperative rupture had a statistical impact on the risk of second event, whereas type of surgery or approach did not. Conclusion This study is a plea in favor of OSS as the first choice of treatment of OMT when possible. Close follow-up during the first five years is mandatory considering the risk of 8.3% of second events especially in cases with risk factors.

Objective. Hyperechoic lung images are largely detected prenatally but their underlying etiology is still poorly defined. The aim of the study was to determine the concordance between pre and postnatal diagnosis of prenatal hyperechoic lung images. Design. Retrospective monocentric study Setting. University Hospital of Necker-Enfants malades from January 2009 to December 2018 Population. All fetuses with prenatal hyperechoeic lung images. Methods. Prenatal ultrasound evaluation was performed by a fetal medicine specialist. Postnatal diagnosis was based on CT-scan. Pre- and postnatal features were retrieved from medical charts. Main outcome measures. Accuracy of the prenatal diagnosis in the identification of the malformations and the prediction of postnatal symptoms. Results. 75 patients were included. Main prenatal diagnoses were bronchopulmonary sequestrations (BPS) (n=24-32%), pulmonary cystic malformations (PCM) (n=19-25%), congenital lobar emphysemas (CLE) (n=15-20%). Mediastinal shift was observed in 18 cases (24%); in utero intervention was required in 2. For BPS, the prenatal detection of a systemic arterial supply had a sensitivity of 96%, a specificity of 83%, a PPV of 77% and an NPV of 98%. For PCM, the prenatal detection of a cystic component had a sensitivity of 69%, a specificity of 69 %, a PPV of 67% and a NPV of 71%. All 16 neonates with prenatal isolated mediastinal shift were asymptomatic at birth. Seven neonates (CLE=5, BPS=1, BC=1) showed respiratory distress that were not predicted prenatally. Conclusions. Hyperechoic lung malformations reflect a heterogeneous group of lesions. Symptoms at birth are present in 9% and cannot be predicted by prenatal features.