1/6. Embryonal rhabdomyosarcoma of the ampulla of vater with long-term survival following pancreaticoduodenectomy.rhabdomyosarcoma of the biliary tree is a rare cause of biliary tract obstruction in childhood. A 3-year-old child is reported here after presenting with obstructive jaundice secondary to an embryonal rhabdomyosarcoma of the ampulla of vater. He underwent pancreaticoduodenectomy followed by adjuvant chemotherapy and irradiation. He is now well and free of disease 5 years following treatment. This child appears to be the first long-term survivor who has required pancreaticoduodenal resection for this lesion.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
2/6. Embryonal rhabdomyosarcoma of the biliary tree in children: a case report.Embryonal rhabdomyosarcoma (ERS) in children is a very rare tumor. jaundice is the capital symptom. diagnosis can be made by echography, which shows echogenic formations without posterior conic shadows in a dilated biliary duct. The prognosis is bleak in spite of the combination of surgery, radiotherapy and chemotherapy.- - - - - - - - - - ranking = 4keywords = tree (Clic here for more details about this article) |
3/6. rhabdomyosarcoma of the biliary tree: the case for aggressive surgery.Embryonal rhabdomyosarcoma of the bile ducts is associated with a poor prognosis because complete resection rarely is considered possible. We reviewed the literature and report our experience with the management of 3 consecutive patients. The patients were 1, 4, and 9 yr old at presentation, and had jaundice, cachexia, and an abdominal mass. The tumor arose in the common hepatic duct in 2 patients, and in the left hepatic duct in the other. All had tumor extension into both lobes of the liver and residual disease was noted after resection. Two patients were given tumor bed irradiation and all received multiple-drug chemotherapy. Two patients are alive, jaundice free, and with no evidence of disease at 9 mo and 14 yr follow-up. The other patient was a long-term survivor, but developed local recurrent tumor after cessation of chemotherapy and expired 33 mo after diagnosis. The traditional anatomic criteria of hepatic tumor resectability may not apply to botryoid sarcoma of the bile ducts. Aggressive surgery combined with the new adjuvant therapies appears to provide the best chance for a longer survival.- - - - - - - - - - ranking = 4keywords = tree (Clic here for more details about this article) |
4/6. Biliary tree rhabdomyosarcoma: report of one case.A four-year-old boy was admitted due to abdominal distention for two years and jaundice of a few days duration. Abdominal ultrasound and CT scan of the abdomen showed marked dilatation of the common bile duct with two masses noted and a tumor extended to the left hepatic duct. Left hepatic lobectomy with Roux-en-Y right hepaticojejunostomy was performed which was histologically proved to be a embryonal type rhabdomyosarcoma. Chemotherapy was given postoperatively. The patient has had frequent follow-up for two years.- - - - - - - - - - ranking = 4keywords = tree (Clic here for more details about this article) |
5/6. rhabdomyosarcoma of the biliary tree.rhabdomyosarcoma (RMS) of the biliary tree is a rare tumor in children that has a very poor prognosis. Preoperatively, it is often mistaken for a choledochal cyst. We report a case of RMS of the biliary tree in a 4-year-old girl who presented with abdominal pain and obstructive jaundice. The RMS was diagnosed at laparotomy; excision was not possible due to its size and localization. Chemotherapy achieved complete regression of the tumor observed at second-look surgery. Preoperative chemotherapy can now avoid mutilating surgical procedures and improve survival.- - - - - - - - - - ranking = 6keywords = tree (Clic here for more details about this article) |
6/6. rhabdomyosarcoma of extrahepatic biliary tree: initial treatment with chemotherapy and conservative surgery.BACKGROUND: The very low frequency of rhabdomyosarcoma (RMS) of the extrahepatic biliary tree has impeded the development of a standardized form of treatment. PROCEDURE: Based on the good response of embryonal RMS to chemotherapy, we used a multi-drug protocol as the initial treatment of a 3-year-old girl after obtaining adequate transparietohepatic biliary drainage. The treatment achieved complete remission. Later conservative laparoscopic surgery revealed only residual scar tissue. The patient is alive and well 1 year after remission. DISCUSSION AND CONCLUSIONS: Management of this difficult tumor using modern less invasive techniques for diagnosis and treatment, with the help of preoperative chemotherapy, prevented extensive damage to the biliary tree and allowed complete recovery.- - - - - - - - - - ranking = 6keywords = tree (Clic here for more details about this article) |