Rhabdomyosarcoma of the biliary tree in childhood. A report from the Intergroup Rhabdomyosarcoma Study.

Ten cases of rhabdomyosarcoma of the biliary tree (RMS/BT) in children are reported in this review of the Intergroup Rhabdomyosarcoma Study (IRS) I and II. RMS/BT constitutes 0.8% of evaluable tumors on IRS I and II. Intermittent obstructive jaundice with or without abdominal distention, fever, and loss of appetite is the typical presentation. Attribution of these symptoms to hepatitis commonly delayed definitive treatment. Demonstration of a mass in the porta hepatitis by ultrasound led quickly to surgical consultation and an exploratory laparotomy. Intraoperative cholangiography was a valuable technique in establishing the level of biliary tree obstruction and verifying a functioning drainage procedure. Resection of the mass with only microscopic or minimal gross residual disease was possible at the initial surgery in six of the ten patients. Included in this group are all four of the surviving patients. The continuity of bile flow was usually maintained by variations of a Roux-en-Y jejunostomy. Histology was embryonal RMS with some botryoid elements. Consultation with a surgical pathologist during the course of the surgery was of invaluable assistance. Multi-drug chemotherapy and radiotherapy consistent with the IRS protocols were given postoperatively. A second-look surgery was useful in evaluating residual or recurrent disease. Four patients are surviving free of disease at 6 months, 3, 6 1/4, and 6 1/2 years from diagnosis, respectively. This review demonstrates that a multidisciplinary approach in RMS/BT can lead to long-term survival.