Surgical Lymph Node Staging in Extremity Rhabdomyosarcoma: The EpSSG RMS 2005 Trial Experience

Background: The European pediatric soft tissue Sarcoma Study Group (EpSSG) RMS 2005 study recommends a lymph node biopsy for extremity rhabdomyosarcoma (RMS). The aim of our study was to analyze the results of the lymph node sampling strategies used, such as sentinel node biopsy (SNB) and nodal sampling (NS), and compare the outcome of patients undergoing different nodal staging techniques. Methods: All non-metastatic (M0) patients registered in the EpSSG RMS 2005 study with an RMS of the extremity, presenting between 2005 and 2016, were included for analysis of the lymph node sampling techniques used. The secondary objective was to compare the results and outcome for the different sampling procedures. Results: Of 198 patients, 144 had clinically/radiologically negative nodes (cN0), and 72/144 underwent a biopsy (26 SNB/46 NS). Final nodal status was upstaged to pN1 in 11/72 (15.3%) patients—6 after SNB and 5 after NS. In 54 radiologically malignant/suspicious-appearing nodes, 34 NS biopsies were performed, resulting in downstaging to N0 in 9/34 (26.5%) patients. 5-years overall survival (OS) of N0 patients versus N1 patients was 82.5% (95% confidence interval CI 74.7–88.0) versus 46.5% (95% CI 32.2–59.7). 5-years OS in N0 patients was not significantly different in biopsied and non-biopsied patients (p = 0.88). However, in N1 patients, survival was significantly better in biopsied compared with non-biopsied patients (p = 0.006). Conclusion: Lymph node staging plays a crucial role in determining appropriate treatment strategies. Pathology of sampled lymph nodes can upstage or downstage the lymph node status, guiding treatment decisions based on the stage.