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Rhabdomyosarcoma of extrahepatic biliary tree: initial treatment with chemotherapy and conservative surgery

✍ Scribed by Pollono, Daniel G.; Tomarchio, Silvia; Berghoff, Rubén; Drut, Ricardo; Urrutia, Azucena; Cédola, Jorge

Publisher
John Wiley and Sons
Year
1998
Tongue
English
Weight
233 KB
Volume
30
Category
Article
ISSN
0098-1532

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✦ Synopsis

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.

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✍ George Imvrios; Vasilios Papanikolaou; Miltiadis Lalountas; Kaliopi Patsiaoura; 📂 Article 📅 2007 🏛 John Wiley and Sons 🌐 English ⚖ 258 KB

Approximately 60 cases of biliary papillomatosis have been reported in the world literature, while only 6 cases have been reported to be treated with liver transplantation. This rare disease, which is characterized by relapsing episodes of obstructive jaundice and cholangitis that lead to secondary