Background: Rhabdomyosarcoma is the most frequent tumor of soft tissue in children. The survival rate of the patients is significantly increased since the 1970s. This study was undertaken to evaluate the 5- and 10-year survival rates of patients with rhabdomyosarcoma in a single center in Iran. 

Methods: A total of 77 children with rhabdomyosarcoma up to 15 years old who had been treated at the Hematology and Oncology Department in Ali Asghar Children Hospital from 1993 to 2003 were evaluated for their age and gender, as well as histology, stage and primary site of the tumor at diagnosis.

Results: The mean age of the patients was 6.58 years (SD=4.02, median 6 years). In this series, 46 patients (59.7%) were male and 31 (40.3%) female, and the mean survival time of patients was 8 years (95% CI: 8-9). The 5-year survival rate was the highest in patients with localized tumor, stage I and II (82.25% and 86.88% respectively). The survival rate of patients with embryonal tumor was 86.8%, in those with primary orbital tumor was 94%, and in those with genitourinary tumor was 85.71%. The 5- and 10-year overall survival rates were 79.54% and 77.92%, respectively.

Conclusions: Children with rhabdomyosarcoma of lower stage, embryonal histology, and orbital and genitourinary primary sites had a better survival rate. Poor prognosis was associated with metastasis of the tumor at the time of presentation, alveolar histology (48%), and tumor of the extremities (58%). In our study, only the stage of the tumor was significantly different in the variables (P=0.0077) because of the small number of patients. Children who survived the first 5 years after diagnosis were found to have an excellent survival rate.

Key words: rhabdomyosarcoma; survival rate; age; primary site; stage; pathology

                    World J Pediatr 2007;3(1):36-40