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Purpose Current investigational priorities in the treatment of favorable histology Wilms tumor (FHWT) center on accurate staging and risk-stratification. The extent of lymph node (LN) sampling has not been clearly defined; its importance cannot be overstated as it guides adjuvant therapy. The identification of a minimum LN yield to minimize the risk of harboring occult metastatic disease could help development of surgical guidelines. This study focuses on using the beta-binomial distribution to estimate the risk of occult metastatic disease in patients with FHWT. Materials & methods The National Cancer Database was queried for patients with unilateral FHWT from 2004 to 2013. Data were used to characterize nodal positivity for patients who underwent surgery and had ≥1 positive LN and ≥2 LNs examined. The probability of missing a positive LN (i.e., false negative) for a given LN yield was calculated using an empirical estimation and the beta-binomial model. Patients were then stratified by tumor size. Results 422 patients met study criteria. To limit the chance of missing a positive LN to ≤10%, the empirical estimation and beta-binomialmodel estimated that 6 and 10 LNs needed to be sampled, respectively. Tumor size did not influence the result. Internal validation showed little variation to maintain a false negative rate ≤ 10%. Conclusions Using mathematical modeling, it appears that the desired LN yield in FHWT to reduce the risk of false-negative LN sampling to ≤10% is between 6 and 10. The current analysis represents an objective attempt to determine the desired surgical approach to LN sampling to accurately stage patients with FHWT. Level of evidence II

Recent advancements in technology are responsible for most of the progress in RMS treatment. PET-CT scanning has been shown to be superior to conventional metastatic workup. The use of proton beam therapy and brachytherapy to reduce the side effects of radiation is also showing promise. All cooperative oncology groups agree on surgical biopsy for diagnosis and staging of BP RMS. Patients are then grouped and risk classified before receiving chemotherapy. Regardless of local control strategy, oncologic outcomes appear to be similar for BP RMS. Alternative treatment strategies, which remain unproven, include brachytherapy and proton therapy.

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