Background: Surgery remains the cornerstone of treatment for rhabdomyosarcoma (RMS) in children. However, there is considerable variation in surgical management practices worldwide, highlighting the need for standardized clinical practice guidelines (CPG). Methods: The CPG development involved assembling a multidisciplinary group, prioritizing ten key topic areas, conducting evidence searches, and synthesizing findings. Recommendations were voted on using the GRADE (Grading of Recommendations, Assessment, Development, and Evaluations) methodology. Recommendations: The panel recommended regional lymph node evaluation for patients with paratesticular RMS who are more than 10 years old and extremity RMS. Other suggestions included pre-treatment re-excision for incompletely resected RMS, preoperative radiation therapy for unresectable tumors, maintaining a 0.5 cm resection margin, and tumor bed marking with surgical clips. The panel also suggests resection of residual metastatic disease following chemotherapy, resection of relapsed disease, and the least invasive approach for managing patients presenting with obstruction. Conclusion: This CPG provides evidence-based surgical management recommendations for RMS that can be adapted to diverse resource settings.

Survival of Wilms tumor (WT) is >90% in high-resource settings but <30% in low-resource settings. Adapting a standardized surgical approach to WT is challenging in low-resource settings, but a local control strategy is crucial to improving outcomes. Objective: Provide resource-sensitive recommendations for the surgical management of WT. Methods: We performed a systematic review of PubMed and EMBASE through July 7, 2020, and used the GRADE approach to assess evidence and recommendations. Recommendations: Initiation of treatment should be expedited, and surgery should be done in a high-volume setting. Cross-sectional imaging should be done to optimize preoperative planning. For patients with typical clinical features of WT, biopsy should not be done before chemotherapy, and neoadjuvant chemotherapy should precede surgical resection. Also, resection should include a large transperitoneal laparotomy, adequate lymph node sampling, and documentation of staging findings. For WT with tumor thrombus in the inferior vena cava, neoadjuvant chemotherapy should be given before en bloc resection of the tumor and thrombus and evaluation for viable tumor thrombus. For those with bilateral WT, neoadjuvant chemotherapy should be given for 6–12 weeks. Neither routine use of complex hilar control techniques during nephron-sparing surgery, nor nephron-sparing resection for unilateral WT with a normal contralateral kidney is recommended. When indicated, postoperative radiotherapy should be administered within 14 days of surgery. Post-chemotherapy pulmonary oligometastasis should be resected when feasible, if local protocols allow omission of whole-lung irradiation in patients with non-anaplastic histology stage IV WT with pulmonary metastasis without evidence of extrapulmonary metastasis. Conclusion: We provide evidence-based recommendations for the surgical management of WT, considering the benefits/risks associated with limited-resource settings.