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Multicenter Study
. 2025 Mar;47(3):974-982.
doi: 10.1002/hed.27994. Epub 2024 Nov 15.

Parameningeal Rhabdomyosarcoma: Results of the European Pediatric Soft Tissue Sarcoma Study Group RMS 2005 Study

Reineke A Schoot  1 , Pieter Taselaar  1 , Giovanni Scarzello  2 , Frederic Kolb  3 , Beatrice Coppadoro  4 , Simone Ter Horst  1   5 , Henry Mandeville  6 , Andrea Ferrari  7 , Raquel Hladun  8 , Sylvie Helfre  9 , Sima Ferman  10 , Anna Kelsey  11 , Marinka L F Hol  1   12 , Christine Devalck  13 , Myriam Ben-Arush  14 , Daniel Orbach  15 , Julia Chisholm  16 , Meriel Jenney  17 , Veronique Minard-Colin  18 , Gianni Bisogno  4 , Johannes H M Merks  1   19
Affiliations
Multicenter Study

Parameningeal Rhabdomyosarcoma: Results of the European Pediatric Soft Tissue Sarcoma Study Group RMS 2005 Study

Reineke A Schoot et al. Head Neck. 2025 Mar.

Abstract

Background: Parameningeal (PM) site is an unfavorable characteristic in rhabdomyosarcoma (RMS). We described the treatment and outcome for patients with PM RMS and investigated the prognostic value of risk factors. We scored PM site by originating site and by highest risk extension.

Methods: Patients with PM RMS were treated within the European pediatric Soft tissue sarcoma Study Group (EpSSG) RMS 2005 study with risk-adapted, multi-modal treatment.

Results: Three-hundred-eighty-one patients with PM RMS were included. Radiotherapy was administered in 359 patients (77 with surgery). After a median follow-up of 75 months, 5-year event-free survival was 60% (95% confidence interval (CI) 55%-65%), 5-year overall survival was 65% (95% CI 60%-70%).

Conclusions: The outcome for patients with PM RMS has not improved in comparison to previous historical studies, despite the more rigorous application of radiotherapy (94% of patients). Signs of meningeal involvement, PM site, and age at diagnosis remained prognostic risk factors.

Trial registration: EudraCT number 2005-000217-35.

Keywords: head and neck; parameningeal; pediatric; prognostic risk factors; rhabdomyosarcoma.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

FIGURE 1
FIGURE 1
Outcome for patients with parameningeal rhabdomyosarcoma. [Color figure can be viewed at wileyonlinelibrary.com]
See this image and copyright information in PMC

References

    1. Merks J. H. M., De Salvo G. L., Bergeron C., et al., “Parameningeal Rhabdomyosarcoma in Pediatric Age: Results of a Pooled Analysis From North American and European Cooperative Groups,” Annals of Oncology 25, no. 1 (2014): 231–236. - PMC - PubMed
    1. Bisogno G., Jenney M., Bergeron C., et al., “Addition of Dose‐Intensified Doxorubicin to Standard Chemotherapy for Rhabdomyosarcoma (EpSSG RMS 2005): A Multicentre, Open‐Label, Randomised Controlled, Phase 3 Trial,” Lancet Oncology 19 (2018): 1061–1071. - PubMed
    1. Bisogno G., De Salvo G. L., Bergeron C., et al., “Vinorelbine and Continuous Low‐Dose Cyclophosphamide as Maintenance Chemotherapy in Patients With High‐Risk Rhabdomyosarcoma (RMS 2005): A Multicentre, Open‐Label, Randomised, Phase 3 Trial,” Lancet Oncology 20 (2019): 1566–1575. - PubMed
    1. Bisogno G., Minard‐Colin V., Zanetti I., et al., “Nonmetastatic Rhabdomyosarcoma in Children and Adolescents: Overall Results of the European Pediatric Soft Tissue Sarcoma Study Group RM2005 Study,” Journal of Clinical Oncology 41 (2023): 2342–2349, 10.1200/JCO.22.02093. - DOI - PubMed
    1. Schoot R. A., Chisholm J. C., Casanova M., et al., “Metastatic Rhabdomyosarcoma: Results of the European Paediatric Soft Tissue Sarcoma Study Group MTS 2008 Study and Pooled Analysis With the Concurrent BERNIE Study,” Journal of Clinical Oncology 40, no. 32 (2022): 3730–3740. - PMC - PubMed

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