R Luksch1, A Tienghi2, K Sundby Hall3, F Fagioli4, P Picci5, E Barbieri6, L Gandola7, M Eriksson8, P Ruggieri9, P Daolio10, P Lindholm11, A Prete12, G Bisogno13, A Tamburini14, G Grignani15, M E Abate16, M Podda17, S Smeland3, S Ferrari16. 1. Department of Pediatrics, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan. Electronic address: roberto.luksch@istitutotumori.mi.it. 2. Department of Oncology, S. Maria delle Croci Hospital, Ravenna, Italy. 3. Department of Oncology, Norwegian Radium Hospital, Oslo University Hospital, Oslo, Norway. 4. Department of Pediatric Onco-Hematology, Regina Margherita Children's Hospital, Turin. 5. Department of Musculoskeletal Oncology, Experimental Oncology Division, Istituto Ortopedico Rizzoli, Bologna. 6. Department of Radiotherapy, University of Bologna, Policlinico Sant'Orsola, Bologna. 7. Department of Radiotherapy, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy. 8. Skane Onkologiska Klinik, Lund University Hospital and Lund University, Lund, Sweden. 9. Department of Musculoskeletal Oncology, Division of Surgery, Istituto Ortopedico Rizzoli, Bologna. 10. Oncologic/Orthopedic Surgery, Istituto Ortopedico G. Pini, Milan, Italy. 11. Department of Oncology and Radiotherapy, Turku University Hospital, Turku, Finland. 12. Department of Pediatric Oncology, University of Bologna, Policlinico Sant'Orsola, Bologna. 13. Department of Pediatric Oncology, University of Padua, Padua. 14. Department of Pediatric Onco-Hematology, Ospedale Meyer, Florence. 15. Department of Medical Oncology, Institute for Cancer Research and Treatment, Candiolo. 16. Department of Musculoskeletal Oncology, Division of Chemotherapy, Istituto Ortopedico Rizzoli, Bologna, Italy. 17. Department of Pediatrics, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan.
Abstract
BACKGROUND: The Italian Sarcoma Group and the Scandinavian Sarcoma Group designed a joint study to improve the prognosis for patients with Ewing's family tumors and synchronous metastatic disease limited to the lungs, or the pleura, or a single bone. PATIENTS AND METHODS: The study was opened in 1999 and closed to the enrollment in 2008. The program consisted of intensive five-drug combination chemotherapy, surgery and/or radiotherapy as local treatment, and consolidation treatment with high-dose busulfan/melphalan plus autologous stem cell rescue and total-lung irradiation. RESULTS: During the study period, 102 consecutive patients were enrolled. The median follow-up was 62 months (range 24-124). The 5-year event-free survival probability was 0.43 [standard deviation (SD) = 0.05] and the 5-year overall survival probability was 0.52 (SD = 0.052). Unfavorable prognostic factors emerging on multivariate analysis were a poor histological/radiological response at the site of the primary tumor [relative risk (RR) = 3.4], and incomplete radiological remission of lung metastases after primary chemotherapy (RR = 2.6). One toxic death and one secondary leukemia were recorded. CONCLUSIONS: This intensive approach is feasible and long-term survival is achievable in ∼50% of patients. New treatment approaches are warranted for patients responding poorly to primary chemotherapy.
BACKGROUND: The Italian Sarcoma Group and the Scandinavian Sarcoma Group designed a joint study to improve the prognosis for patients with Ewing's family tumors and synchronous metastatic disease limited to the lungs, or the pleura, or a single bone. PATIENTS AND METHODS: The study was opened in 1999 and closed to the enrollment in 2008. The program consisted of intensive five-drug combination chemotherapy, surgery and/or radiotherapy as local treatment, and consolidation treatment with high-dose busulfan/melphalan plus autologous stem cell rescue and total-lung irradiation. RESULTS: During the study period, 102 consecutive patients were enrolled. The median follow-up was 62 months (range 24-124). The 5-year event-free survival probability was 0.43 [standard deviation (SD) = 0.05] and the 5-year overall survival probability was 0.52 (SD = 0.052). Unfavorable prognostic factors emerging on multivariate analysis were a poor histological/radiological response at the site of the primary tumor [relative risk (RR) = 3.4], and incomplete radiological remission of lung metastases after primary chemotherapy (RR = 2.6). One toxic death and one secondary leukemia were recorded. CONCLUSIONS: This intensive approach is feasible and long-term survival is achievable in ∼50% of patients. New treatment approaches are warranted for patients responding poorly to primary chemotherapy.
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: Uta Dirksen; Bernadette Brennan; Marie-Cécile Le Deley; Nathalie Cozic; Henk van den Berg; Vivek Bhadri; Bénédicte Brichard; Line Claude; Alan Craft; Susanne Amler; Natalie Gaspar; Hans Gelderblom; Robert Goldsby; Richard Gorlick; Holcombe E Grier; Jean-Marc Guinbretiere; Peter Hauser; Lars Hjorth; Katherine Janeway; Heribert Juergens; Ian Judson; Mark Krailo; Jarmila Kruseova; Thomas Kuehne; Ruth Ladenstein; Cyril Lervat; Stephen L Lessnick; Ian Lewis; Claude Linassier; Perrine Marec-Berard; Neyssa Marina; Bruce Morland; Hélène Pacquement; Michael Paulussen; R Lor Randall; Andreas Ranft; Gwénaël Le Teuff; Keith Wheatley; Jeremy Whelan; Richard Womer; Odile Oberlin; Douglas S Hawkins Journal: J Clin Oncol Date: 2019-09-25 Impact factor: 50.717
Authors: Lianne M Haveman; Roelof van Ewijk; Elvira C van Dalen; Willemijn B Breunis; Leontien Cm Kremer; Henk van den Berg; Uta Dirksen; Johannes Hm Merks Journal: Cochrane Database Syst Rev Date: 2021-09-02