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Biliary botryoid rhabdomyosarcoma in a pediatric patient

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Biliary rhabdomyosarcomas are rare, making up about 1% of all liver malignancies. These tumors are often an embryonal subtype and generally large, measuring multiple centimeters. We present an 11-year-old female with a history of intermittent painless jaundice and imaging suspicious for a choledochal cyst. Endoscopy identified a lesion protruding from her ampulla, and pathology diagnostic of a botryoid variant of embryonal rhabdomyosarcoma. She improved with chemotherapy and radiation. Botryoid biliary rhabdomyosarcomas typically have rapid growth with early presentation of jaundice around 3.5years old; however, this case was reported in an older child with a protracted history of intermittent jaundice.


rhabdomyosarcomas, jaundice, pathology, diagnostic, embryonal, polypoid, magnetic, resonance, cholangiopancreatography, vincristine, adriamycin, cyclophosphamide