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Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in the pediatric and adolescent population, with 350 new cases diagnosed each year. While they can develop anywhere in the body, the genitourinary tract is the second most common primary location for an RMS to develop. Overall survival has improved through the increased use of protocols and multidisciplinary approaches. However, the guidelines for management continue to change as systemic and radiation therapeutics advance. Given the relative rarity of this disease compared to other non-solid childhood malignancies, healthcare providers not directly managing RMS may not be familiar with their presentation and updated management. This review aims to provide foundational knowledge of the management of RMSs with an emphasis on specific management paradigms for those arising from the genitourinary tract. The genitourinary tract is the second most common location for an RMS to develop but varies greatly in symptomology and survival depending on the organ of origin. As the clinical understanding of these tumors advances, treatment paradigms have evolved. Herein, we describe the breadth of presentations for genitourinary RMSs with diagnostic and treatment management considerations, incorporating the most recently available guidelines and societal consensus recommendations.

Choledochal cysts are a core pathology in pediatric surgery, affecting 1/100,000 live births in the western world, and 1/13,000 in eastern asia. These cysts are classified by the Todani classification, types I-V, in respect to their location and underlying pathophysiology. Infants and children presenting with stigmata of biliary disease should undergo evaluation for choledocal cyst. Workup includes axial imaging, ultrasonography, and laboratory investigation. A liver biopsy is necessary in neonates and newborns to rule out cystic biliary atresia, which would require further evaluation and management. Large choledochal cysts may be visualized on prenatal ultrasound, and warrant referral to a fetal care center for postnatal work up and monitoring. Management of choledochal cysts is dependent on the anatomic variant and spans from ERCP with sphincterotomy, to cyst excision with ductal and alimentary tract reconstruction. Type V choledochal cysts may require liver transplantation. Long term follow up is required due to an enhanced risk of future malignancy, even after resection.

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