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
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.