Evidence-Based Surgical Guidelines for Treating Children With Rhabdomyosarcoma

Surgery remains the cornerstone of treatment for rhabdomyosarcoma (RMS) in children. However, there is considerable variation in surgical management practices worldwide, highlighting the need for standardized Clinical Practice Guidelines (CPG).

The CPG development involved assembling a multidisciplinary group, prioritizing 10 key topic areas, conducting evidence searches, and synthesizing findings. Recommendations were voted on using the GRADE (Grading of Recommendations, Assessment, Development, and Evaluations) methodology.

The panel recommended regional lymph node evaluation for patients with paratesticular RMS who are more than 10 years old and extremity RMS. Other suggestions included pretreatment re-excision for incompletely resected RMS, preoperative radiation therapy for unresectable tumors, maintaining a 0.5 cm resection margin, and tumor bed marking with surgical clips. The panel also suggests resection of residual metastatic disease following chemotherapy, resection of relapsed disease, and the least invasive approach for managing patients presenting with obstruction.

This CPG provides evidence-based surgical management recommendations for RMS that can be adapted to diverse resource settings.