S Ferrari1, K Sundby Hall2, R Luksch3, A Tienghi4, T Wiebe5, F Fagioli6, T A Alvegard5, A Brach Del Prever6, A Tamburini7, M Alberghini8, L Gandola9, M Mercuri10, R Capanna11, S Mapelli12, A Prete13, M Carli14, P Picci15, E Barbieri16, G Bacci17, S Smeland2. 1. Department of Chemotherapy, Istituto Ortopedico Rizzoli, Bologna, Italy. Electronic address: stefano.ferrari@ior.it. 2. Oncology Department, The Norwegian Radium Hospital, Oslo, Norway. 3. Pediatric Oncology Division, Istituto Nazionale Tumori, Milan. 4. Department of Medical Oncology, S.Maria delle Croci Hospital, Ravenna, Italy. 5. Department of Cancer Epidemiology, and Paediatric Oncology, Lund University Hospital, Lund, Sweden. 6. Department of Pediatric Oncology, Ospedale Regina Margherita, Torino. 7. Pediatric Oncology Division, Meyer Hospital, Firenze. 8. Department of Pathology, Istituto Ortopedico Rizzoli, Bologna. 9. Radiotherapy Unit, Istituto Nazionale Tumori, Milano. 10. Department of Surgery, Istituto Ortopedico Rizzoli, Bologna. 11. Oncologic/Orthopaedic Surgery Division, Centro Traumatologico Ortopedico, Firenze. 12. Oncologic/Orthopaedic Surgery, Istituto Gaetano Pini, Milan. 13. Department of Pediatric Oncology, University Hospital, Bologna. 14. Department of Pediatric Oncology, University Hospital, Padova. 15. Experimental Oncology Divisions, Department of Musculoskeletal Oncology, Istituto Ortopedico Rizzoli, Bologna. 16. Radiotherapy Division, University Hospital, Bologna, Italy. 17. Department of Chemotherapy, Istituto Ortopedico Rizzoli, Bologna, Italy.
Abstract
BACKGROUND: High-dose chemotherapy (HDT) was added to conventional chemotherapy in Ewing sarcoma family tumor (EFT) patients, poor responders (PRs) to induction chemotherapy in order to improve their survival. PATIENTS AND METHODS: Patients aged ≤40 years with nonmetastatic Ewing sarcoma (ES) received vincristine (V), doxorubicin (A), cyclofosfamide (C), actinomycin (Ac), ifosfamide (I) and etoposide (E) (VACAc-IE regimen) as induction chemotherapy. As maintenance treatment, good responders (GR) received nine cycles of VACAc-IE regimen. PRs received three cycles of VAC-IE, mobilizing cycle with CE and HDT with Busulfan and Melphalan with stem cell support. RESULTS: Three hundred patients [median age 15 years (3-40 years)] entered the study. One patient refused local treatment, 242 (81%) underwent surgery [with radiotherapy (RT) in 80] and 57 (19%) RT alone. No toxic deaths were recorded. Overall GR were 146 (49%). Twenty-eight PR did not receive HDT. At a median follow-up of 64 months (21-116 months), 5-year overall and event-free survival (EFS) were 75% and 69%, respectively. Five-year EFS was 75% for GR, 72% for PR treated with HDT and 33% for PR who did not receive HDT. CONCLUSIONS: High-dose therapy added to the VACA-IE regimen in PR patients is feasible and effective. Selected groups of patients with ES can benefit from HDT.
BACKGROUND: High-dose chemotherapy (HDT) was added to conventional chemotherapy in Ewing sarcoma family tumor (EFT) patients, poor responders (PRs) to induction chemotherapy in order to improve their survival. PATIENTS AND METHODS: Patients aged ≤40 years with nonmetastatic Ewing sarcoma (ES) received vincristine (V), doxorubicin (A), cyclofosfamide (C), actinomycin (Ac), ifosfamide (I) and etoposide (E) (VACAc-IE regimen) as induction chemotherapy. As maintenance treatment, good responders (GR) received nine cycles of VACAc-IE regimen. PRs received three cycles of VAC-IE, mobilizing cycle with CE and HDT with Busulfan and Melphalan with stem cell support. RESULTS: Three hundred patients [median age 15 years (3-40 years)] entered the study. One patient refused local treatment, 242 (81%) underwent surgery [with radiotherapy (RT) in 80] and 57 (19%) RT alone. No toxic deaths were recorded. Overall GR were 146 (49%). Twenty-eight PR did not receive HDT. At a median follow-up of 64 months (21-116 months), 5-year overall and event-free survival (EFS) were 75% and 69%, respectively. Five-year EFS was 75% for GR, 72% for PR treated with HDT and 33% for PR who did not receive HDT. CONCLUSIONS: High-dose therapy added to the VACA-IE regimen in PR patients is feasible and effective. Selected groups of patients with ES can benefit from HDT.
Authors: Dagmar Adamkova Krakorova; Katerina Kubackova; Ladislav Dusek; Tomas Tomas; Pavel Janicek; Stepan Tucek; Jana Prausova; Igor Kiss; Iva Zambo Journal: Pathol Oncol Res Date: 2017-08-12 Impact factor: 3.201
Authors: Jeremy Whelan; Marie-Cecile Le Deley; Uta Dirksen; Gwénaël Le Teuff; Bernadette Brennan; Nathalie Gaspar; Douglas S Hawkins; Susanne Amler; Sebastian Bauer; Stefan Bielack; Jean-Yves Blay; Stefan Burdach; Marie-Pierre Castex; Dagmar Dilloo; Angelika Eggert; Hans Gelderblom; Jean-Claude Gentet; Wolfgang Hartmann; Wolf-Achim Hassenpflug; Lars Hjorth; Marta Jimenez; Thomas Klingebiel; Udo Kontny; Jarmila Kruseova; Ruth Ladenstein; Valerie Laurence; Cyril Lervat; Perrine Marec-Berard; Sandrine Marreaud; Jean Michon; Bruce Morland; Michael Paulussen; Andreas Ranft; Peter Reichardt; Hendrik van den Berg; Keith Wheatley; Ian Judson; Ian Lewis; Alan Craft; Heribert Juergens; Odile Oberlin Journal: J Clin Oncol Date: 2018-09-06 Impact factor: 44.544
Authors: Peter Peneder; Adrian M Stütz; Didier Surdez; Manuela Krumbholz; Sabine Semper; Mathieu Chicard; Nathan C Sheffield; Gaelle Pierron; Eve Lapouble; Marcus Tötzl; Bekir Ergüner; Daniele Barreca; André F Rendeiro; Abbas Agaimy; Heidrun Boztug; Gernot Engstler; Michael Dworzak; Marie Bernkopf; Sabine Taschner-Mandl; Inge M Ambros; Ola Myklebost; Perrine Marec-Bérard; Susan Ann Burchill; Bernadette Brennan; Sandra J Strauss; Jeremy Whelan; Gudrun Schleiermacher; Christiane Schaefer; Uta Dirksen; Caroline Hutter; Kjetil Boye; Peter F Ambros; Olivier Delattre; Markus Metzler; Christoph Bock; Eleni M Tomazou Journal: Nat Commun Date: 2021-05-28 Impact factor: 14.919