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. 2025 Oct;32(10):7751-7761.
doi: 10.1245/s10434-025-17908-3. Epub 2025 Jul 24.

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

Sheila Terwisscha van Scheltinga  1 , Johannes H M Merks  2   3 , Florent Guerin  4 , Timothy Rogers  5 , Ross J Craigie  6 , Gabriela Guillén  7 , Federica De Corti  8 , Patrizia Dall'Igna  8 , Raquel Dávila Fajardo  2   9 , Gianni Bisogno  10 , Andrea Ferrari  11 , Daniel Orbach  12 , Meriel Jenney  13 , Julia C Chisholm  14 , Véronique Minard-Colin  15 , Maya Cesen  16 , Nina Jehanno  17 , Laura S Hiemcke-Jiwa  2   18 , Ilaria Zanetti  10 , Beatrice Coppadoro  10 , Alida F W van der Steeg  2 , Max M van Noesel  2   3 , Marc H W A Wijnen  2
Affiliations

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

Sheila Terwisscha van Scheltinga et al. Ann Surg Oncol. 2025 Oct.

Abstract

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.

Keywords: Biopsy; Extremity; Lymph nodes; Pediatric; Rhabdomyosarcoma; Sentinel node; Staging.

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Conflict of interest statement

Disclosure: Sheila Terwisscha van Scheltinga, Johannes H.M. Merks, Florent Guerin, Timothy Rogers, Ross J. Craigie, Gabriela Guillén, Federica De Corti, Patrizia Dall’Igna, Raquel Dávila Fajardo, Gianni Bisogno, Andrea Ferrari, Daniel Orbach, Meriel Jenney, Julia C. Chisholm, Véronique Minard-Colin, Maya Cesen MD, Nina Jehanno, Laura S. Hiemcke-Jiwa, Ilaria Zanetti, Beatrice Coppadoro, Alida F.W. van der Steeg, Max M. van Noesel, Marc H.W.A. Wijnen have no conflicts of interest to declare that may be relevant to the contents of this study.

Figures

Fig. 1
Fig. 1
Results of biopsy in clinically/radiologically negative and positive patients. SNB sentinel node biopsy, rNS random nodal sampling, tNS targeted nodal sampling, SN sentinel node, NSN non-sentinel node, RMS rhabdomyosarcoma, neg negative, pos positive
Fig. 2
Fig. 2
Overall survival by lymph node stage, 5-yr 5-years, OS overall survival, CI confidence interval
Fig. 3
Fig. 3
a Event-free survival by lymph node exploration in N0 and pN0. b Overall survival by lymph node exploration in N0 and pN0 patients, 5-yr 5-years, OS overall survival, CI confidence interval
Fig. 4
Fig. 4
a Event-free survival by lymph node exploration in N1 and pN1 patients. b Overall survival by lymph node exploration in N1 and pN1 patients, 5-yr 5-years, OS overall survival, CI confidence interval
See this image and copyright information in PMC

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