Risk factors for disease progression and prognosis of pediatric head and neck rhabdomyosarcoma / 中华实用儿科临床杂志

ABSTRACT

Objective:To explore the risk factors for disease progression and the prognosis with different disease events in a historical cohort of 56 pediatric patients with head and neck rhabdomyosarcoma (HNRMS).Methods:From June 1, 2013 to June 30, 2019, 56 pediatric patients diagnosed as HNRMS treated in the Department of Pedia-trics, Beijing Tongren Hospital, Capital Medical University were recruited in the present study.Clinical data, including general information about the diagnosis, treatment, prognostic factors, and follow-up results were collected.Patients were divided into non disease-progression (NPD) groups, including non-event (NE) group and disease-relapse (RD) group, and disease-progression (PD) group based on the disease event.Clinical data and the prognosis in NE group, RD group and PD group were compared.Risk factors for disease progression in pediatric patients with HNRMS were further analyzed by univariate and multivariate analyses of NPD group and PD group.Results:The median follow-up time of all 56 patients was 31.8 months (3.5-74.6 months). There were 30, 12, and 14 patients in NE group, RD group and PD group, respectively.The estimated 5-year overall survival rate was 100.0%, 82.5%, and 11.9% in NE group, RD group and PD group, respectively, which was statistically significant ( P<0.001). Compared with those of NE group, significantly higher rates of patients with a minimum tumor size of 5 cm ( P=0.008) and non-initial radiation therapy ( P=0.001) were detected in PD group.In addition, a significantly higher rate of patients with a minimum tumor size of 5 cm ( P=0.002), and more advanced postoperative pathological stage ( H=12.982, P=0.009) and risk of disease severity( H=18.679, P<0.001) were detected in PD group than those of RD group.The univariate analysis demonstrated that tumor size > 5 cm, alveolar rhabdomyosarcoma, non-initial radiation therapy, advanced postoperative pathological stage and high risk of disease severity were significant risk factors for disease progression of HNRMS.The multivariate analysis result showed that tumor size > 5 cm ( OR=6.75, 95% CI 1.02-44.78), alveolar rhabdomyosarcoma ( OR=7.10, 95% CI 1.08-46.57), and non-initial radiation therapy ( OR=15.26, 95% CI 2.38-97.70) were independent risk factors for disease progression of HNRMS. Conclusions:Pediatric patients with HNRMS in disease progression have a significantly worse overall survival rate, and their prognosis quite differs from those with disease relapse or non-disease events.A minimum tumor size of 5 cm, alveolar rhabdomyosarcoma and non-initial radiation therapy are independent risk factors for disease progression of HNRMS.Pediatric patients with disease progression of HNRMS are susceptible to advanced pathological stage and risk of disease severity.