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Biliary tract rhabdomyosarcoma: a report from the Soft Tissue Sarcoma Committee of the Associazione Italiana Ematologia Oncologia Pediatrica

Abstract

Introduction

Rhabdomyosarcoma is a soft tissue malignant musculoskeletal tumor frequent in children. Biliary duct localization is extremely rare, but it is the most common cause of malignant obstructive jaundice in pediatric patients.

Methods

This report describes a series of 10 patients under 18 years of age with biliary tract rhabdomyosarcoma who were enrolled, from 1979 to 2004, in 3 consecutive Italian pediatric cooperative protocols that had been drawn up by the Soft Tissue Sarcoma Committee of the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP).

Results

Considering initial and delayed surgery, tumor resection was achieved in 7 cases, 3 complete with free margins (2 liver transplants) and 4 with microscopic residual disease. Chemotherapy was given to all patients and radiotherapy to 3. At present, 5 patients survive in complete remission 90-200 months after diagnosis while 4 died of disease progression or relapse and 1 of liver transplant-related complications.

Conclusions

Better outcomes in this series were associated with the feasibility of conservative surgery due to the favorable location of the tumor, in particular in the common bile duct. Chemotherapy and radiotherapy might obviate the need for demolitive surgery or liver transplant, which were linked to worse outcomes in our series.

Post author correction

Article Type: SHORT COMMUNICATION

DOI:10.5301/tj.5000692

Authors

Katia Perruccio, Valerio Cecinati, Angela Scagnellato, Massimo Provenzi, Giuseppe Maria Milano, Eleonora Basso, Carla Manzitti, Giovanni Cecchetto, Rita Alaggio, Martina Di Martino, Amalia Schiavetti, Fraia Melchionda, Maria Carmen Affinita, Stefano Chiaravalli, Lucia Miglionico, Rita Balter, Angela Tamburini, Gianni Bisogno, Andrea Ferrari

Article History

Disclosures

Financial support: This work was partially supported by the Fondazione Città della Speranza and MURST ex 60%.
Conflict of interest: The authors have no conflicts of interest related to this article.

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Authors

Affiliations

  • Pediatric Hematology/Oncology Unit, S. Maria della Misericordia Hospital, Perugia - Italy
  • Pediatric Hematology/Oncology Unit, Department of Hematology, Transfusion Medicine and Biotechnology, Pescara - Italy
  • Pediatric Hematology/Oncology Division, Padua University, Padua - Italy
  • Pediatric Hematology/Oncology Unit, Papa Giovanni XXIII Hospital, Bergamo - Italy
  • Hematology/Oncology Division, Ospedale Pediatrico Bambino Gesù IRCCS, Rome - Italy
  • Pediatric Onco-Hematology, Stem Cell Transplantation and Cellular Therapy Division, Regina Margherita Children’s Hospital, Turin - Italy
  • Department of Pediatric Hematology/Oncology, Giannina Gaslini Children’s Hospital, Genoa - Italy
  • Pediatric Surgery, Padua University, Padua - Italy
  • Pathology Department, Padua University, Padua - Italy
  • Pediatric Oncology Service, Department of Pediatrics, Second University, Naples - Italy
  • Pediatric Hematology/Oncology Unit, La Sapienza University, Rome - Italy
  • Department of Pediatrics, University of Bologna, S. Orsola-Malpighi Hospital, Bologna - Italy
  • Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milan - Italy
  • Pediatric Hematology/Oncology Unit, Casa Sollievo della Sofferenza, S.G. Rotondo (Foggia) - Italy
  • Pediatric Hematology/Oncology Unit, Azienda Ospedaliera Universitaria Integrata, Verona - Italy
  • Hematology/Oncology Unit, Meyer Pediatric Hospital, Florence - Italy

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