The Evolution of Diagnosis and Management of Pediatric Biliary Tract Rhabdomyosarcoma

Background: The discovery and accurate diagnosis of a biliary tract mass in the pediatric population can prove to be extremely challenging. The purpose of this article is to give an overview of the evolution of management with regard to biliary tract rhabdomyosarcoma (RMS) in the pediatric population. Methods: Clinical research related to biliary tract RMS in the pediatric population is reviewed, focusing on the diagnosis, staging and treatment. Results: Over the past century the management of biliary tract RMS has transitioned through a spectrum of radical surgical procedures with poor outcomes. Over the past several decades there have been efforts to treat with chemotherapy based on known RMS chemosensitivity, with surgical interventions being utilized to manage a complex variation of disease manifestations in a case specific manner which further highlights the difficulty of managing this rare pathologic entity. This multimodal approach has significantly changed the landscape for those diagnosed with childhood RMS. The diagnosis of biliary tract RMS remains an extreme challenge since this is a rare tumor in children, may or may not be included in the differential diagnosis, and can be confused with other pathologic entities such as choledochal cysts. Conclusion: RMS involving the biliary tract is extremely rare, and remains the most common cause of neoplastic biliary obstruction in childhood. The goals of maintaining biliary tract integrity, overall quality of life and cure while avoiding excessive morbidity and mortality remain at the heart of this pediatric oncologic entity.