Impact of extensive surgery in a multidisciplinary approach of parameningeal rhabdomyosarcoma in children, adolescent and young adult population
Menée à l'aide d'une méthode de randomisation mendélienne et de données 1992-2021 portant sur 31 patients atteints d'un rhabdomyosarcome paraméningé (âge médian : 6 ans ; durée médiane de suivi : 81 mois), cette étude analyse la survie et le contrôle local après l'intégration d'une chirurgie extensive dans le traitement de la maladie
Context and Objectives: Parameningeal (PM) site is a well-known unfavorable site for rhabdomyosarcoma (RMS). PM RMS are usually considered unresectable and treated by radiochemotherapy. This study reviews our experience with a multidisciplinary approach, including extensive surgery.
Method: We included all patients treated for PM RMS requiring extensive surgery from January 1992 to December 2021.
Results: Thirty-one patients were included with a median age of 6 years (range: 6 months–17 years). The primary site was the infratemporal fossa in 81 %, nasopharynx in 13 %, and middle ear/paranasal sinus in one case each. At diagnosis, 23 % had lymph node involvement, and 13 % had distant metastases. Twenty-six patients received neoadjuvant chemotherapy before extensive surgery, while five underwent extensive surgery after radiochemotherapy. Free flap reconstruction was needed for 71 % of patients. Adjuvant radiotherapy (median dose: 50 Gray) was performed in 24 patients, with a median delay of 7.5 weeks post-surgery (range). Seven recurrences (23 %) were observed: four local relapses, two leptomeningeal spreads, and one distant metastasis. The median follow-up was 81 months (range). The median time to relapse was 12 months (range). Five-year event-free and overall survival rates were both 73.9 %, with a local failure-free survival of 85.7 %.
Conclusion: Our study suggests that incorporating extensive surgery in PM RMS treatment improves long-term local control and survival, even in advanced cases with unfavorable features.
European Journal of Surgical Oncology , résumé, 2025