C. Chidiac1, G. Giwa2, C. Ramdat3, R. Gorijavolu2, A. Biswas2, D. S. Rhee1 1The Johns Hopkins University School Of Medicine, Department Of Surgery- Division Of Pediatric Surgery, Baltimore, MD, USA 2The Johns Hopkins University School Of Medicine, Baltimore, MD, USA 3Johns Hopkins University, Baltimore, MD, USA
Introduction:
Retroperitoneal rhabdomyosarcoma, a rare and aggressive childhood cancer, requires a multimodal treatment approach, with surgery and radiation therapy being key components of local control. However, a comprehensive evaluation of their individual and combined effectiveness is essential. Our study aims to compare the effectiveness of surgery, radiation therapy and their combination in the management of retroperitoneal rhabdomyosarcoma.
Methods:
We conducted a retrospective review of patients under 18 years of age with retroperitoneal rhabdomyosarcoma from 2004 to 2020 using the National Cancer Database (NCDB). Patients were divided into 3 groups based on their treatment approaches: surgery alone, radiation therapy (RT) alone, and surgery + RT. Kaplan-Meyer survival curves and multivariate cox proportional regression accounting for age, sex, race, ethnicity, tumor size, metastasis were used to compare survival outcomes among these groups.
Results:
791 patients were included in our study, of which 188 (23.8%) underwent surgery exclusively, 319 (40.3%) received RT alone, and 284 (35.9%) underwent both surgery and RT. The median follow up time was 43 months. Children receiving RT alone were generally older (median age 9 years vs. 6 surgery alone vs. 4 surgery + RT, p<.001), more frequently female (47.7% vs 36.2% vs 38.7%, p=0.02), and have metastasis at diagnosis (25.4% vs. 5.4% vs 12.3%, P<.001), notably to the bones and lungs. Race, ethnicity, and tumor size were similar between the 3 groups. In addition, patients receiving surgery without RT were less likely to receive chemotherapy (87.2 vs. 98.1 RT alone vs. 98.2 surgery + RT, P<.0001). When comparing 5-year overall survival (OS) rates, patients treated solely with RT exhibited lower survival rates compared to those managed with surgery alone or surgery and RT (5-year OS 50% vs. 80% vs. 73%, respectively, p<.001). Subsequent multivariate analysis demonstrated that both undergoing surgery alone (Adjusted Hazard Ratio [aHR] 0.63, 95% CI 0.45-0.87, p=0.005) and undergoing a combined approach of surgery and RT (aHR 0.43, 95% CI 0.28-0.65, p<0.001) were correlated with a improved 5-year OS, relative to RT alone.
Conclusion:
In children with retroperitoneal rhabdomyosarcoma, treatment with RT alone was associated with worse outcomes compared to surgery and RT or surgery alone. Further study is warranted to better understand the role of surgery in local control for this patient population.
