Ten cases of @entity980 of the biliary tree (RMS/BT) in @entity1 are reported in this review of the Intergroup @entity980 Study (IRS) I and II. RMS/BT constitutes 0.8% of evaluable @entity5 on IRS I and II. @entity3883 with or without abdominal distention, @entity221 , and @entity1277 is the typical presentation. Attribution of these symptoms to @entity99 commonly delayed definitive treatment. Demonstration of a mass in the porta @entity99 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 @entity1 . Included in this group are all four of the surviving @entity1 . 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 @entity1 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.

XXXX of the biliary tree in childhood. A report from the Intergroup @entity980 Study.

@entity1 :: ('9606', 'Species') :: ['children', 'patients'] @entity3883 :: ('MESH:D041781', 'Disease') :: ['Intermittent obstructive jaundice'] @entity5 :: ('MESH:D009369', 'Disease') :: ['tumors'] @entity1277 :: ('MESH:D001068', 'Disease') :: ['loss of appetite'] @entity980 :: ('MESH:D012208', 'Disease') :: ['Rhabdomyosarcoma', 'rhabdomyosarcoma'] @entity99 :: ('MESH:D056486', 'Disease') :: ['hepatitis'] @entity221 :: ('MESH:D005334', 'Disease') :: ['fever']
@entity980 :: (MESH:D012208,Disease) :: ['Rhabdomyosarcoma']
