Outcomes in rhabdomyosarcoma: Experience from a tertiary cancer center in India

Abstract

Background: Rhabdomyosarcoma (RMS) is an embryonal tumor seen commonly in children. There is a paucity of literature on RMS from India. The present study was conducted to look at the disease characteristics and outcome of RMS patients treated at our center. Methods: This was a retrospective study that included patients with RMS of all age groups treated at our center between 2001 and 2016. Overall survival (OS) and event-free survival (EFS) were calculated using the Kaplan–Meier method. Results: The study included 70 patients with a median age of 7 years (1–70 years) and 42/70 (60%) were male. The common sites of disease were parameningeal in 31 (44%) and extremity in 17 (24%) patients. Metastasis at presentation was seen in 22/70 (31%) patients. Embryonal (66%) followed by alveolar (9%) and pleomorphic (4%) were the common histologic subtype. The risk stratification showed good-risk in 13 (18%), intermediate-risk in 35 (50%), and high-risk in 22 (32%) patients. The median duration of follow-up was 70.4 months (Confidence interval: 26.1–114.8 months). The median EFS and OS in the study were 11 months and 21 months, respectively. The factors associated with better OS were male gender (P = 0.038), nonmetastatic disease (P = 0.028), good- and intermediate-risk subgroup (P = 0.002), complete surgical excision (P = 0.002), and radiotherapy (P = −0.002). Conclusion: Outcomes in RMS remain dismal with most patients presenting with advanced disease. Multimodality management with chemotherapy, surgery, and radiotherapy in patients with nonmetastatic disease improves outcome.