Major resection for embryonal rhabdomyosarcoma of the biliary tree

Embryonal rhabdomyosarcoma of the biliary tree is a very rare tumor. Only 56 patients have been reported in the literature; 17 of them were long-term survivors. The recommended therapy is multidisciplinary: according to the Intergroup Rhabdomyosarcoma Study I and II (IRS I and II), the combination of major resection, chemotherapy, and radiation of the porta hepatis could improve the results. This report reviews our experience with four consecutive patients from a surgical point of view. We conclude that the classical definition of resectability cannot be applied to rhabdomyosarcomas of the biliary tree, because these polypoid tumors as a rule extend into the liver sectors and often affect both halves of the liver. Major resection with atypical reconstruction of the biliary tree is necessary in order to provide some promise of success. The problem of preoperatively determining the extent of the tumor is very important, as even intraoperative cholangiography cannot accurately demonstrate the true dimensions of the tumor in the sectorial bile ducts. The advantages and disadvantages of the procedures that come into question are discussed.