Effects of alternating cyclophosphamide/pirarubicin/vincristine with ifosfamide/etoposide on children with intermediate-risk rhabdomyosarcoma

Objective To analyze the efficacy of alternating Cyclophosphamide/Pirarubicin/Vincristine (CAV) with Ifosfamide/Etoposide (IE) on patients with intermediate-risk Rhabdomyosarcoma (RMS).Methods Children with intermediate-risk RMS were studied.They first received chemotherapy regimen of alternating CAV with IE for 4-6 cycles.Then,local therapies (surgery and/or radiotherapy) were selectively given depending on tumor response.Total chemotherapy included 8-10 cycles.The tumor cell necrosis rate of surgical specimens was calculated after neoadjuvant chemotherapy.Results Thirty-eight children (1.4-18.0,mean 5 years old) with intermediate-risk RMS were treated in this institution between June 2003 and March 2012.The rate of response to neoadjuvant chemotherapy was 89.5％,including CR (n =12),GPR (n =6) and PR (n =16).Twenty-seven patients underwent local treatments,12 underwent surgery,7 underwent radiotherapy and the other 8 underwent both.Nine patients underwent second look operation and the tumor cell necrosis rate in the surgical specimens was ≥90％ in 6 patients and ＜90％ in 3 patients.The main side effect was bone marrow suppression,which was reversible.The median follow-up period was 22 months (range,4.5 to 97.7 months).Three-year overall survival (OS) and event-free survival (EFS) of all patients were 59.4％ and 47.3％,respectively.Three-year OS of patients with and without local treatment were 72.2％ and 33.3％,respectively (P =0.001).Three-year EFS of patients with and without local treatment were 53.3％ and 33.3％,respectively (P =0.025).All the 6 patients with tumor cell necrosis rate ≥90％ were still alive,but all the 3 patients with tumor cell necrosis rate ＜90％ died after relapse.Conclusions The chemotherapy regimen of alternating CAV with IE is effective for intermediate-risk RMS,showing a high rate of tumor cell necrosis and tolerable toxicities.Positive and standard comprehensive treatment could improve the survival of patients with RMS.\r\n\r\nKey words: \r\nRhabdomyosarcoma;  Child;  Drug therapy,combination;  Treatment outcome