BACKGROUND: Reported overall survival (OS) rates of patients with localized Ewing sarcoma family of tumors (ESFT) are >80% when treated with the MSKCC P6 protocol. However, it has been associated with a 5.8% incidence of secondary leukemias. A modified P6 (mP6) protocol with reduced exposure to chemotherapy is presented. PROCEDURE: Thirty-one newly diagnosed ESFT patients were enrolled onto this phase II, single-arm, non-randomized protocol. Courses 1, 2 and 4 consisted of cyclophosphamide 4.2 g/m², doxorubicin 75 mg/m², and vincristine 2 mg/m² (CDV). Cycles 3 and 5 consisted of ifosfamide 9 g/m² and etoposide 500 mg/m² (IE). Course 5 ifosfamide was 14 g/m² if necrosis was <90%. RESULTS: Twenty-four patients had loco-regional disease and seven had metastases. The 4-year event-free survival (EFS) rate for patients with localized tumors is 83% and overall survival (OS) is 92%. The 3-year EFS rate for patients with distant metastases is 28% and OS rate is 42%. EWS-FLI1 fusion genes were detected in 17 cases (74%) and EWS-ERG in six cases (26%). Type 1 EWS-FLI1 variant was present in 6/7 metastatic patients and 3/16 loco-regional cases (P = 0.001). None of the patients experienced tumor progression before remission. All relapses occurred within 2 years from the end of treatment and local relapses (n = 3) happened in patients who did not receive radiation therapy. No secondary malignancies have been observed, median follow-up of 4.3 years for surviving patients. CONCLUSIONS: In this pilot study, the mP6 protocol produced a complete remission rate of 83% at 4 years in non-metastatic ESFT reducing the risk of secondary malignancies.
BACKGROUND: Reported overall survival (OS) rates of patients with localized Ewing sarcoma family of tumors (ESFT) are >80% when treated with the MSKCC P6 protocol. However, it has been associated with a 5.8% incidence of secondary leukemias. A modified P6 (mP6) protocol with reduced exposure to chemotherapy is presented. PROCEDURE: Thirty-one newly diagnosed ESFT patients were enrolled onto this phase II, single-arm, non-randomized protocol. Courses 1, 2 and 4 consisted of cyclophosphamide 4.2 g/m², doxorubicin 75 mg/m², and vincristine 2 mg/m² (CDV). Cycles 3 and 5 consisted of ifosfamide 9 g/m² and etoposide 500 mg/m² (IE). Course 5 ifosfamide was 14 g/m² if necrosis was <90%. RESULTS: Twenty-four patients had loco-regional disease and seven had metastases. The 4-year event-free survival (EFS) rate for patients with localized tumors is 83% and overall survival (OS) is 92%. The 3-year EFS rate for patients with distant metastases is 28% and OS rate is 42%. EWS-FLI1 fusion genes were detected in 17 cases (74%) and EWS-ERG in six cases (26%). Type 1 EWS-FLI1 variant was present in 6/7 metastatic patients and 3/16 loco-regional cases (P = 0.001). None of the patients experienced tumor progression before remission. All relapses occurred within 2 years from the end of treatment and local relapses (n = 3) happened in patients who did not receive radiation therapy. No secondary malignancies have been observed, median follow-up of 4.3 years for surviving patients. CONCLUSIONS: In this pilot study, the mP6 protocol produced a complete remission rate of 83% at 4 years in non-metastatic ESFT reducing the risk of secondary malignancies.
Authors: Javier E Oesterheld; Damon R Reed; Bhuvana A Setty; Michael S Isakoff; Patrick Thompson; Hong Yin; Masanori Hayashi; David M Loeb; Tiffany Smith; Rikesh Makanji; Brooke L Fridley; Lars M Wagner Journal: Pediatr Blood Cancer Date: 2020-05-09 Impact factor: 3.167
Authors: J Mora; O Cruz; C Lavarino; J Rios; M Vancells; A Parareda; H Salvador; M Suñol; R Carrasco; A Guillen; S Mañé; C de Torres Journal: Clin Transl Oncol Date: 2015-01-17 Impact factor: 3.405
Authors: J Mora; A Castañeda; S Perez-Jaume; A Lopez-Pousa; E Maradiegue; C Valverde; J Martin-Broto; X Garcia Del Muro; O Cruz; J Cruz; J Martinez-Trufero; J Maurel; M A Vaz; E de Alava; C de Torres Journal: Br J Cancer Date: 2017-08-08 Impact factor: 7.640
Authors: Adriana Rosé; Nicolas André; Viviana R Rozados; Leandro E Mainetti; Mauricio Menacho Márquez; María José Rico; Paula Schaiquevich; Milena Villarroel; Lauro Gregianin; Jaume Mora Graupera; Wendy Gómez García; Sidnei Epelman; Carlos Alasino; Daniel Alonso; Guillermo Chantada; O Graciela Scharovsky Journal: Ecancermedicalscience Date: 2016-09-06