Adherence to Treatment, Response and Patterns of Failure in Pediatric Parameningeal Rhabdomyosarcoma: Experience From a Tertiary Cancer Care Center From India

The study was aimed at evaluating adherence to treatment protocol and outcome in pediatric parameningeal rhabdomyosarcoma (PM-RMS). We analyzed the characteristics, treatment administered, outcomes and patterns of failure of pediatric PM-RMS, who were treated with multimodality therapy between January 2005 and December 2013. Univariate and multivariate analysis (MVA) was completed to evaluate the impact of various prognostic factors. Thirty-seven patients were treated at our institution. Majority of them had the primary disease in paranasal sinuses (n = 13). Majority of the patients belonged to group III (n = 30) and stage III (n = 24). The overall response rate to treatment was 52.5% (n = 21). At a mean follow-up of 19.1 months, 23 patients developed disease progression. The actuarial rates of failure-free survival and overall survival (OS) at 2 years were 40% and 67.5%, respectively. Patients who received > 20 weeks of intended chemotherapy schedule (P = 0.02) and had complete response to first-line treatment (P = 0.0004) were found to have superior failure-free survival on MVA. Complete response was the lone determinant of superior OS on MVA (P = 0.006). Majority of patients with PM-RMS present with advanced stage disease. Response to first-line treatment is a significant predictor of superior progression-free survival and OS in these patients.