M Ben Arush1, V Minard-Colin2, V Mosseri3, A S Defachelles4, C Bergeron5, N Algret3, S Fasola6, N André7, E Thebaud8, N Corradini8, V Bernier9, H Martelli10, D Ranchère11, D Orbach12. 1. Pediatric Hematology Oncology Department, Rambam Health Care Campus, Haifa, Israel. 2. Paediatric and Adolescent Oncology Department, Gustave Roussy, Villejuif, France. 3. Department of Biostatistics, Institut Curie, Paris, France. 4. Paediatric Oncology Department, Centre Oscar Lambret, Lille, France. 5. Centre Leon Berard, Institut d'hematologie et d'Oncologie Pediatrique, Lyon, France. 6. Paediatric Haematology-Oncology Department, Trousseau Hospital Assistance Publique, Paris, France. 7. Paediatric Haematology-Oncology Department, La Timone Hospital, Marseille, France. 8. Hematology oncology pediatric department, Centre Hospitalier, Nantes, France. 9. Radiotherapy Department, Centre Alexis Vautrin, Nancy, France. 10. Paediatric Surgery Department, Bicetre Hospital, Le Kremlin Bicetre, France. 11. Pathology Department, Institut d'hematologie et d'Oncologie Pediatrique, Lyon, France. 12. Paediatric, Adolescent and Young Adults Department, Institut Curie, 75005 Paris, France. Electronic address: daniel.orbach@curie.fr.
Abstract
PURPOSE: Due to the extensive initial distant tumour spread in metastatic rhabdomyosarcoma, the importance of local treatment is sometimes underestimated. A retrospective study was conducted to identify the prognostic value of aggressive local treatment in paediatric metastatic rhabdomyosarcoma. PATIENTS: Patients with metastatic rhabdomyosarcoma aged 1-21 years treated in France from 1998 to 2011 according to European protocols MMT-4-89, 4-91, 98 and recent national guidelines were selected. Survival comparison were performed between patients with 'aggressive local treatment' (surgery and radiotherapy) and exclusive surgery or radiotherapy, after exclusion of patients with early progression. End-points were event-free and overall survival (OS). RESULTS: A total of 101 children, median age 9 years, with majority of primaries in unfavourable sites (73 patients, pts), T2 tumours (66 pts), alveolar subtypes (65 pts) and large tumours (>5 cm, 83 pts) received various chemotherapy regimens. On univariate and multivariate analyses, OS was better after 'aggressive local treatment' (49 pts; 44.3 ± 8%), than after exclusive surgery (10 pts; 18.8% ± 15.5%) or exclusive radiotherapy (29 pts; 16.1 ± 7.2%, P < 0.006). Moreover, OS was better in the case of surgery with complete resection (41.1 ± 10.2%) or microscopic residue (56.4 ± 14.9%) than macroscopic residue (20.0 ± 12.6%; P < 0.03). CONCLUSIONS: In this large retrospective analysis, OS appeared to be better for patients receiving 'aggressive local treatment' even after adjustment for the initial patient and tumour characteristics. Isolated debulking surgery is associated with a very poor outcome and should be avoided. Aggressive local treatment in patients with rhabdomyosarcoma, even with metastasis, should be seriously considered.
PURPOSE: Due to the extensive initial distant tumour spread in metastatic rhabdomyosarcoma, the importance of local treatment is sometimes underestimated. A retrospective study was conducted to identify the prognostic value of aggressive local treatment in paediatric metastatic rhabdomyosarcoma. PATIENTS: Patients with metastatic rhabdomyosarcoma aged 1-21 years treated in France from 1998 to 2011 according to European protocols MMT-4-89, 4-91, 98 and recent national guidelines were selected. Survival comparison were performed between patients with 'aggressive local treatment' (surgery and radiotherapy) and exclusive surgery or radiotherapy, after exclusion of patients with early progression. End-points were event-free and overall survival (OS). RESULTS: A total of 101 children, median age 9 years, with majority of primaries in unfavourable sites (73 patients, pts), T2 tumours (66 pts), alveolar subtypes (65 pts) and large tumours (>5 cm, 83 pts) received various chemotherapy regimens. On univariate and multivariate analyses, OS was better after 'aggressive local treatment' (49 pts; 44.3 ± 8%), than after exclusive surgery (10 pts; 18.8% ± 15.5%) or exclusive radiotherapy (29 pts; 16.1 ± 7.2%, P < 0.006). Moreover, OS was better in the case of surgery with complete resection (41.1 ± 10.2%) or microscopic residue (56.4 ± 14.9%) than macroscopic residue (20.0 ± 12.6%; P < 0.03). CONCLUSIONS: In this large retrospective analysis, OS appeared to be better for patients receiving 'aggressive local treatment' even after adjustment for the initial patient and tumour characteristics. Isolated debulking surgery is associated with a very poor outcome and should be avoided. Aggressive local treatment in patients with rhabdomyosarcoma, even with metastasis, should be seriously considered.
Authors: Monika Scheer; Tobias Dantonello; Peter Brossart; Dagmar Dilloo; Lothar Schweigerer; Simone Feuchtgruber; Monika Sparber-Sauer; Christian Vokuhl; Stefan S Bielack; Thomas Klingebiel; Ewa Koscielniak; Thekla von Kalle Journal: Pediatr Radiol Date: 2018-01-24
Authors: Emmanuelle Bompas; Loïc Campion; Antoine Italiano; Axel Le Cesne; Christine Chevreau; Nicolas Isambert; Maud Toulmonde; Olivier Mir; Isabelle Ray-Coquard; Sophie Piperno-Neumann; Esma Saada-Bouzid; Maria Rios; Jean-Emmanuel Kurtz; Corinne Delcambre; Pascale Dubray-Longeras; Florence Duffaud; Marie Karanian; François Le Loarer; Patrick Soulié; Nicolas Penel; Jean-Yves Blay Journal: Cancer Med Date: 2018-06-28 Impact factor: 4.452
Authors: S Gallego; D Bernabeu; M Garrido-Pontnou; G Guillen; N Hindi; A Juan-Ribelles; C Márquez; C Mata; J Orcajo; G Ramírez; M Ramos; C Romagosa; D Ruano; P Rubio; R Vergés; C Valverde Journal: Clin Transl Oncol Date: 2021-07-01 Impact factor: 3.405
Authors: Soledad Gallego; Yueh-Yun Chi; Gian Luca De Salvo; Minjie Li; Johannes H M Merks; David A Rodeberg; Sheila Terwisscha van Scheltinga; Leo Mascarenhas; Daniel Orbach; Meriel Jenney; Lynn Million; Veronique Minard-Colin; Suzanne Wolden; Ilaria Zanetti; David M Parham; Henry Mandeville; Rajkumar Venkatramani; Gianni Bisogno; Douglas S Hawkins Journal: Pediatr Blood Cancer Date: 2020-11-27 Impact factor: 3.167
Authors: Jürgen F Schäfer; Claudio Granata; Thekla von Kalle; Martin Kyncl; Annemieke S Littooij; Pier Luigi Di Paolo; Irmina Sefic Pasic; Rutger A J Nievelstein Journal: Pediatr Radiol Date: 2020-05-28
Authors: Roelof van Ewijk; Reineke A Schoot; Monika Sparber-Sauer; Simone A J Ter Horst; Nina Jehanno; Lise Borgwardt; Bart de Keizer; Johannes H M Merks; Alberto de Luca; Kieran McHugh; Thekla von Kalle; Jürgen F Schäfer; Rick R van Rijn Journal: Pediatr Radiol Date: 2021-06-17