Surgical management of pediatric biliary rhabdomyosarcoma: Importance of differential diagnosis.

Rhabdomyosarcoma (RMS) comprises approximately 5% of all pediatric malignancies, and the biliary system is considered one of the rarest RMS locations. Awareness, knowledge, and early recognition of the disease are essential for accurate diagnosis and proper treatment of biliary RMS in a child with obstructive jaundice and suspicious radiological findings. We present two pediatric biliary RMS cases requiring different managements because of their primary evaluations at their referring facilities. A four-and-a-half-year-old boy was referred to our institution for liver transplantation following neoadjuvant chemotherapy for centrally located unresectable biliary RMS. The patient received a left lateral segment graft from a living donor with no complications during the post-transplant period. The second patient was a seven-year-old foreign boy with obstructive jaundice and a history of choledochal cyst resection. A tumoral mass was revealed during exploration, and macroscopic total resection of the lesion was performed. The final pathology result of the resected material was biliary RMS with microscopic residue on the bile duct margin and lymph node involvement. The patient was transferred to the Pediatric Oncology Division for systemic treatment following surgical recovery. Biliary RMS presents distinct challenges in terms of accurate diagnosis and successful management. A multidisciplinary approach is indispensable for effective treatment. Complete surgical resection has been proven to be the mainstay strategy in feasible cases. Contributions of pre- and postoperative chemotherapy and radiotherapy are crucial in extensive disease. Liver transplantation should be considered, with reasonable success rates, in persistent unresectable and non-metastatic cases.