Abstract
Background: 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).
Methods: The CPG development involved assembling a
multidisciplinary group, prioritizing ten key topic areas, conducting
evidence searches, and synthesizing findings. Recommendations were voted
on using the GRADE (Grading of Recommendations, Assessment, Development,
and Evaluations) methodology. Recommendations: 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 pre-treatment 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. Conclusion: This CPG provides evidence-based
surgical management recommendations for RMS that can be adapted to
diverse resource settings.