Katia Perruccio1, Valerio Cecinati2, Angela Scagnellato3, Massimo Provenzi4, Giuseppe Maria Milano5, Eleonora Basso6, Carla Manzitti7, Giovanni Cecchetto8, Rita Alaggio9, Martina Di Martino10, Amalia Schiavetti11, Fraia Melchionda12, Maria Carmen Affinita3, Stefano Chiaravalli13, Lucia Miglionico14, Rita Balter15, Angela Tamburini16, Gianni Bisogno3, Andrea Ferrari13. 1. 1 Pediatric Hematology/Oncology Unit, S. Maria della Misericordia Hospital, Perugia - Italy. 2. 2 Pediatric Hematology/Oncology Unit, Department of Hematology, Transfusion Medicine and Biotechnology, Pescara - Italy. 3. 3 Pediatric Hematology/Oncology Division, Padua University, Padua - Italy. 4. 4 Pediatric Hematology/Oncology Unit, Papa Giovanni XXIII Hospital, Bergamo - Italy. 5. 5 Hematology/Oncology Division, Ospedale Pediatrico Bambino Gesù IRCCS, Rome - Italy. 6. 6 Pediatric Onco-Hematology, Stem Cell Transplantation and Cellular Therapy Division, Regina Margherita Children's Hospital, Turin - Italy. 7. 7 Department of Pediatric Hematology/Oncology, Giannina Gaslini Children's Hospital, Genoa - Italy. 8. 8 Pediatric Surgery, Padua University, Padua - Italy. 9. 9 Pathology Department, Padua University, Padua - Italy. 10. 10 Pediatric Oncology Service, Department of Pediatrics, Second University, Naples - Italy. 11. 11 Pediatric Hematology/Oncology Unit, La Sapienza University, Rome - Italy. 12. 12 Department of Pediatrics, University of Bologna, S. Orsola-Malpighi Hospital, Bologna - Italy. 13. 13 Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milan - Italy. 14. 14 Pediatric Hematology/Oncology Unit, Casa Sollievo della Sofferenza, S.G. Rotondo (Foggia) - Italy. 15. 15 Pediatric Hematology/Oncology Unit, Azienda Ospedaliera Universitaria Integrata, Verona - Italy. 16. 16 Hematology/Oncology Unit, Meyer Pediatric Hospital, Florence - Italy.
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.
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.
Entities:
Keywords:
Biliary tract; Chemotherapy; Radiotherapy; Rhabdomyosarcoma; Surgery
Authors: Jamie M Aye; Wei Xue; Joshua D Palmer; David O Walterhouse; Michael A Arnold; Todd E Heaton; Rajkumar Venkatramani Journal: Pediatr Blood Cancer Date: 2021-01-26 Impact factor: 3.167
Authors: Juri Fuchs; Anastasia Murtha-Lemekhova; Markus Kessler; Patrick Günther; Alexander Fichtner; Jan Pfeiffenberger; Pascal Probst; Katrin Hoffmann Journal: Front Oncol Date: 2021-09-30 Impact factor: 6.244