Portal de Periódicos da CAPES

Treatment of Ewing sarcoma family of tumors with a modified P6 protocol in children and adolescents

2011; Wiley; Volume: 57; Issue: 1 Linguagem: Inglês

10.1002/pbc.22813

1545-5017

Jaume Mora, Carmen de Torres, Andreu Parareda, Ferrán Torner, Patricia Galván, Eva Rodríguez, Teresa Cardesa, Héctor Salvador, Mariona Suñol, R. Huguet, Ofelia Cruz,

Testicular diseases and treatments

Abstract 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/m 2 , doxorubicin 75 mg/m 2 , and vincristine 2 mg/m 2 (CDV). Cycles 3 and 5 consisted of ifosfamide 9 g/m 2 and etoposide 500 mg/m 2 (IE). Course 5 ifosfamide was 14 g/m 2 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. Pediatr Blood Cancer 2011;57:69–75. © 2011 Wiley‐Liss, Inc.