Successful Treatment of Biliary Rhabdomyosarcoma With Neoadjuvant Chemotherapy and Liver Transplantation.

Abstract

Rhabdomyosarcoma, a malignant tumor with striated muscle differentiation, is rarely located in the biliary tract as the primary site. Because of its rarity, this tumor may cause diagnostic and treatment difficulties, and, so far, no therapeutic guidelines specific for this tumor location are available. Here, we describe a 2 -year -old female patient who presented with a 20 -cm progressive abdominal mass causing respiratory distress and feeding difficulties. Magnetic resonance imaging showed a hepatic mass extending into the peritoneal cavity, with cystic and solid components. The patient first received 12 weeks of chemotherapy (vincristine, cisplatin, doxorubicin, cyclophosphamide, actinomycin -D ), which reduced the abdominal circumference and the tumor size. The patient then underwent orthotopic liver transplant from her father following total hepatectomy. She was discharged 2 weeks posttransplant without complications, and 4 additional chemotherapy cycles were planned. Although no standard guidelines exist, tumor reduction before transplant appears to be a viable approach.