Background Tumor characteristics affect surgical complexity and outcomes of partial nephrectomy (PN). Objective To develop an Arterial Based Complexity (ABC) scoring system to predict morbidity of PN. Design, Setting, and Participants Four readers independently scored contrast-enhanced computed tomography images of 179 patients who underwent PN. Intervention Renal cortical masses were categorized by the order of vessels needed to be transected/dissected during PN. Scores of 1, 2, 3S, or 3H were assigned to tumors requiring transection of interlobular and arcuate arteries, interlobar arteries, segmental arteries, or in close proximity of the renal hilum, respectively during PN. Outcome Measurements and Statistical Analysis Interobserver variability was assessed with kappa values and percentage of exact matches between each pairwise combination of readers. Linear regression was used to evaluate the association between reference scores and ischemia time, estimated blood loss, and estimated glomerular filtration rates (eGFR) at 6 wk and 6 mo after surgery adjusted for baseline eGFR. Fisher’s exact test was used to test for differences in risk of urinary fistula formation by reference category assignment. Results and Limitations Pairwise comparisons of readers’ score assignments were significantly correlated (all p <0.0001); average kappa = 0.545 across all reader pairs. The average proportion of exact matches was 69%. Linear regression between the complexity score system and surgical outcomes showed significant associations between reference category assignments and ischemia time (p <0.0001) and estimated blood loss (p = 0.049). Fisher’s exact test showed a significant difference in risk of urinary fistula formation with higher reference category assignments (p = 0.028). Limitations include use of a single institutional cohort to evaluate our system. Conclusions The ABC scoring system for PN is intuitive, easy to use, and demonstrated good correlation with perioperative morbidity. Patient Summary The ABC scoring system is novel anatomy-reproducible tool developed to help patients and doctors understand the complexity of renal masses and predict the outcomes of kidney surgery.

Purpose To assess interobserver variability of R.E.N.A.L., PADUA, and C-Index systems among observers with varying degrees of clinical experience and each system's subscale correlation with surgical outcome metrics. Methods Computed tomography images of 90 patients who underwent open, laparoscopic, or robot-assisted laparoscopic partial nephrectomy were scored by 1 radiology fellow, 2 urology fellows, 1 radiology resident, and 1 secondary school student. Agreement among readers was determined calculating intraclass correlation coefficients. Associations between radiology fellow scores (reference standard as reader with greatest clinical experience), ischemia time, and percent change in postoperative eGFR were evaluated using Spearman correlation. Results Agreement using C-Index method (ICC = 0.773) was higher than with PADUA (ICC = 0.677) or R.E.N.A.L (ICC = 0.660). Agreement between reference and secondary school student was lower than with other physicians, although the differences were not statistically significant. The reference's scores were significantly (p <0.05) associated with ischemia time on all three scoring systems and with percent change in eGFR at 6 weeks using C-index (p = 0.016). Tumor size, nearness to sinus, location relative to polar lines (R.E.N.A.L.) and tumor size, renal sinus involvement and collecting system involvement (PADUA) correlated with ischemia time (all p ≤0.001). No R.E.N.A.L. or PADUA subscales significantly correlated with percent change in postoperative eGFR. Conclusions Clinical experience reduces interobserver variability of existing nephrometry systems though not significantly and less so when using directly measureable anatomic variables. Consistently, only measures of tumor size and distance to intrarenal structures were useful in predicting clinically relevant outcomes.

Background:The SIOP-Renal Tumor Study Group (RTSG) does not advocate invasive procedures to determine histology before the start of therapy. This may induce misdiagnosis-based treatment initiation, but only for a relatively small percentage of approximately 10% of non-Wilms tumors (non-WTs). MRI could be useful for reducing misdiagnosis, but there is no global consensus on differentiating characteristics. Purpose: To identify MRI characteristics that may be used for discrimination of newly diagnosed pediatric renal tumors. Study Type: Consensus process using a Delphi method. Population: Not applicable.

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BackgroundRhabdomyosarcoma (RMS), one of the most common soft tissue sarcomas of childhood, is very rare in the neonatal period (0.4–2% of cases). In order to gain a deeper understanding of this disease at such age, patient and tumor features, as well as treatment modality and outcome need to be reported.Case presentationWe describe two cases with congenital RMS treated at Bambino Gesù Children’s Hospital between 2000 and 2016. They represent only 2.24% of all RMS patients diagnosed during that period in our Institution; this data is in agreement with the incidence reported in the literature. They reflect the two different clinical forms in which the disease may manifest itself. One patient, with the alveolar subtype (positive for specific PAX3-FOXO1 fusion transcript) and disseminated disease, had a fatal outcome with central nervous system (CNS) progression despite conventional and high dose chemotherapy. The other child, with the localized embryonal subtype, was treated successfully with conservative surgery and conventional chemotherapy, including prolonged maintenance therapy. He is disease free at 7 years of follow-up.ConclusionsRMS can also be diagnosed during the neonatal period. Given the young age, disease management is often challenging, and especially for the alveolar subtype, the outcome is dismal despite intensified multimodality therapy. In fact, it characteristically manifests with multiple subcutaneous nodules and progression most commonly occurs in the CNS (Rodriguez-Galindo et al., Cancer 92(6):1613–20, 2001). In this context, CNS prophylaxis could play a role in preventing leptomeningeal dissemination, and molecular studies can allow a deeper tumor characterization, treatment stratification and identification of new potential therapeutic targets.