Results of immunotherapy in childhood solid cancer have been so far, with the exception of neuroblastoma, quite disappointing. Lack of knowledge of the immune contexture of these tumors may have contributed to the failure of immunotherapies so far. Here, we systematically reviewed the literature regarding the immunology of Wilms tumor (WT), one of the most frequent pediatric solid tumors of the abdomen. In Wilms tumor patients the high cure rate of >90%, achieved by the combination of surgery and radio-chemotherapy, is at the expense of a high early and late toxicity. Moreover, treatment-resistant entities, such as diffuse anaplastic tumors or recurrent disease, still pose unsolved clinical problems. Successful immunotherapy could represent a novel and possibly less-toxic treatment option. Employing the PRISMA (Preferred Reporting Items for Systematic Review and Meta-Analysis) method of literature search, we analyzed the current knowledge of the immunological landscape of Wilms tumors in terms of tumor microenvironment, prognostic implications of single biomarkers, and immunotherapy response.

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Introduction Anorectal malformations (ARM) affect 1 in 5,000 newborns with a wide range of defects. In the absence of a visible fistula, the diagnosis and classification of ARM require an augmented pressure distal colostogram. This procedure can be done after a diverting colostomy has been performed and implies exposing the child to radiation. We hypothesized that high-resolution transperineal ultrasound could correctly diagnose the type of ARM, thus sparing radiation exposure. Case Description Four full-term male newborns with ARM and no visible anal opening were referred to our center for further management. A diverting descendostomy was performed in the first 48 hours of life in all cases. Prior to the reconstructive surgery, we performed a high-resolution transperineal ultrasound with 3D tomographic reconstruction of the perineal region to assess the urethra, the rectum, and a possible fistula. Findings were compared with a conventional augmented pressure distal colostogram. The image acquisition was fast and did not cause any additional distress to the children. Conclusion In all cases the results of the distal colostogram nicely correlated with the high-resolution transperineal ultrasound with 3D tomographic reconstruction. In the future, we envision a time when it can potentially replace the distal colostogram in preoperative assessment of ARM with no distress and exposure to radiation.

Introduction: In patients with anorectal malformations (ARM), the sacral ratio (SR) serves as an established predictive marker for functional prognosis and is derived from conventional radiographs. More recently, magnetic resonance imaging (MRI) has emerged as a diagnostic alternative for preoperative/baseline assessment of patients with ARM. With this study we aimed to evaluate if the SR could be reliable be measured from MRI images and if it correlated to SR measurements obtained from radiographs. Methods: Two raters analyzed MRI data and conventional radiographs from thirty-one subjects ( n = 17 with ARM; n = 14 controls). We calculated intra-class correlation coefficients (ICCs) to test inter-rater reliability and applied paired t -tests to examine if SR parameters from MRI were comparable with those from pelvic radiograph. We further computed Pearson's correlation coefficients to test the linear relationship between SR calculated from MRI and conventional radiographs. Results: The ARM and control groups did not statistically differ in their age and weight on the day of the MRI scan. Reliability analysis revealed an excellent inter-rater agreement for SR from radiograph parameters with an intraclass correlation coefficient (ICC) of 0.94, respectively of 0.86 for MRI (frontal plane), and of 0.84 for MRI (sagittal plane). The correlation coefficient between SR calculated from the sagittal MRI and SR calculated from radiograph images was significant and high ( r = 0.80, P < 0.001). The SRs from MRI images did not significantly differ from SRs from radiographs, but were also not statistically equivalent. Conclusion:Our results demonstrate that the SR can be derived from MRI images with good inter-rater reliability. The SR value is marginal higher when calculated on MRI, presumably due to inclusion of cartilaginous yet unossified structures.

IntroductionShifting the training from the operating room (OR) to simulation models has been proven effective in enhancing patient safety and reducing the learning time to achieve competency and increase the operative efficiency. Currently the field of pediatric surgery only offers few low-cost trainers for specialized training and these feature predominantly artificial and often unrealistic tissue. The aim of this study was to develop an easy access low-cost tissue-realistic simulation model for open training of esophageal atresia and to evaluate the acceptance in trainees and junior pediatric surgeons.Materials and methodsThe model is fashioned using reconfigured chicken skin from a chicken leg. To create a model of esophageal atresia, the chicken skin is dissected off the muscle and reconfigured around a foley catheter balloon to recreate the proximal pouch and a feeding tube to recreate the distal pouch. Surrounding structures such as the tracheo-esophageal fistula and the azygos vein can be easily added, obtaining a realistic esophageal atresia (Type C) prototype. Evaluation of model construction, usage and impact on user were performed by both a self-assessment questionnaire with pre- and post-training questions as well as observer-based variables and a revised Objective Structured Assessment of Technical Skills (OSATS) score.ResultsA total of 10 participants were constructing and using the model at two different timepoints. OSATS score for overall performance was significantly higher (p = 0.005, z = −2.78) during the second observational period [median (MD): 4,95% confidence interval CI: 3.4, 5.1] compared to the first (MD: 3, 95% CI 2.4, 4.1). Self-reported boost in confidence after model usage for performing future esophageal atresia (EA) repair and bowel anastomosis (BA) in general was significantly higher (EA: U = 1, z = −2.3, p = 0.021, BA: U = 1, z = −2.41, p = 0.016) in participants with more years in training/attending status (EA MD:5, BA MD: 5.5) compared to less experienced participants (EA MD: 1.5, BA: 1).ConclusionOur easy access low-cost simulation model represents a feasible and tissue realistic training option to increase surgical performance of pediatric surgical trainees outside the OR.