Florent Guérin1, Timothy Rogers2, Véronique Minard-Colin3, Mark N Gaze4, Sheila Terwisscha5, Max Van Noesel5, Federica De Corti6, Gabriela Guillén Burrieza7, Gian Luca De Salvo8, Anna Kelsey9, Daniel Orbach10, Andrea Ferrari11, Christophe Bergeron12, Gianni Bisogno13, Hélène Martelli1. 1. Department of Paediatric Surgery, Bicêtre Hospital, Hôpitaux Universitaires Paris-Sud, Le Kremlin-Bicetre, France. 2. Department of Paediatric Surgery, University Hospitals Bristol NHS Foundation trust, Bristol, United Kingdom. 3. Département d'Oncologie de l'Enfant et de l'Adolescent, Institut Gustave Roussy, Villejuif, France. 4. Department of Oncology, University College London Hospitals NHS Foundation Trust, London, United Kingdom. 5. Department of Paediatric Surgery, Prinses Máxima Centrum voor Kinderoncologie, Utrecht, the Netherlands. 6. Pediatric Surgery Unit, Department of Woman's and Child's Health, University Hospital of Padova, Padova, Italy. 7. Department of Paediatric Surgery, University Hospital Vall d'Hebron, Barcelona, Spain. 8. Clinical Trials and Biostatistics Unit, Istituto Oncologico Veneto IOV-IRCCS, Padova, Italy. 9. Department of Paediatric Histopathology, Royal Manchester Children Hospital, Manchester, United Kingdom. 10. SIREDO Oncology Center (Care, Innovation and Research for Children, Adolescents and Young Adults with Cancer), Institut Curie, PSL University, Paris, France. 11. Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy. 12. Institut d'Hématologie et d'Oncologie Pédiatrique, Centre Léon Bérard, Lyon, France. 13. Hematology Oncology Division, Department of Women's and Children's Health, University of Padova, Padova, Italy.
Abstract
OBJECTIVES: To evaluate the impact of local therapies on the outcome of patients with liver-bile duct rhabdomyosarcoma (LBDRMS). METHODS: Data of 30 patients included in the EpSSG-RMS 2005 study were analyzed. RESULTS: The median age at diagnosis was 3 years (11 months-8 years). All patients had non-alveolar histology. Fifteen patients had a tumor > 5 cm and six had enlarged regional lymph nodes on imaging. Eight patients (27%) had primary surgery (1 R0). Six of them received external beam radiotherapy (EBRT). All are in first complete remission (CR1) except one (R1, EBRT+ , local relapse, death). Six patients (20%) received EBRT without surgery: one had local relapse and died. Sixteen patients (53%) underwent delayed surgery, with 12 achieving R0 margins, which were higher than those in the primary surgery group (P = 0.003). Three patients with R0 margins received EBRT; one had a metastatic relapse and died. Nine patients with R0 resection did not receive EBRT, three relapsed locally (two deaths). Four R1 patients received additional EBRT without relapses. Local relapse occurred in two among 19 patients with EBRT and three among 11 without EBRT (P = 0.326). At a median follow-up of 61 months (48-84 months), five patients died; all had a tumor size > 5 cm (P = 0.01). The five-year overall survival was 85% (95% CI, 65-94), and event-free survival was 76% (95% CI, 54-89). CONCLUSION: This analysis did not show any significant difference in outcome between irradiated and nonirradiated patients. Local relapse in LBDRMS is related to initial tumor size and is often fatal.
OBJECTIVES: To evaluate the impact of local therapies on the outcome of patients with liver-bile duct rhabdomyosarcoma (LBDRMS). METHODS: Data of 30 patients included in the EpSSG-RMS 2005 study were analyzed. RESULTS: The median age at diagnosis was 3 years (11 months-8 years). All patients had non-alveolar histology. Fifteen patients had a tumor > 5 cm and six had enlarged regional lymph nodes on imaging. Eight patients (27%) had primary surgery (1 R0). Six of them received external beam radiotherapy (EBRT). All are in first complete remission (CR1) except one (R1, EBRT+ , local relapse, death). Six patients (20%) received EBRT without surgery: one had local relapse and died. Sixteen patients (53%) underwent delayed surgery, with 12 achieving R0 margins, which were higher than those in the primary surgery group (P = 0.003). Three patients with R0 margins received EBRT; one had a metastatic relapse and died. Nine patients with R0 resection did not receive EBRT, three relapsed locally (two deaths). Four R1 patients received additional EBRT without relapses. Local relapse occurred in two among 19 patients with EBRT and three among 11 without EBRT (P = 0.326). At a median follow-up of 61 months (48-84 months), five patients died; all had a tumor size > 5 cm (P = 0.01). The five-year overall survival was 85% (95% CI, 65-94), and event-free survival was 76% (95% CI, 54-89). CONCLUSION: This analysis did not show any significant difference in outcome between irradiated and nonirradiated patients. Local relapse in LBDRMS is related to initial tumor size and is often fatal.
Authors: Jacquelyn N Crane; Wei Xue; Amira Qumseya; Zhengya Gao; Carola A S Arndt; Sarah S Donaldson; Douglas J Harrison; Douglas S Hawkins; Corinne M Linardic; Leo Mascarenhas; William H Meyer; David A Rodeberg; Erin R Rudzinski; Barry L Shulkin; David O Walterhouse; Rajkumar Venkatramani; Aaron R Weiss Journal: Pediatr Blood Cancer Date: 2022-03-06 Impact factor: 3.838
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