Panel deliberation.
The incidence of regional lymph node involvement in rhabdomyosarcoma varies between 10-40% and depends on the fusion status and location of the tumor. Lymph node involvement in rhabdomyosarcoma is associated with worse outcome.1-13
Accurate staging and assessment of lymph node involvement play a crucial role in determining the appropriate treatment and prognosis for patients with rhabdomyosarcoma1-11. The purpose of surgical evaluation of regional lymph nodes is primarily diagnostic.
For primaries in all sites, clinically enlarged lymph nodes should be evaluated pathologically as approximately 75% of enlarged lymph nodes will be confirmed positive for tumor cells.
Clinically uninvolved regional lymph node evaluation is particularly essential for patients with fusion positive disease, those with paratesticular RMS who are more than 10 years old by means of nodal basin sampling and those with extremity or trunk primary by means of sentinel lymph node biopsy1-11. However, prophylactic radical node dissection is of no therapeutic value and is not recommended.
Radiation therapy (RT) is the therapeutic modality of choice for regional lymph node metastases.
The panel is uncertain about timing of resection (upfront versus delayed) in patients with RMS. (No recommendation, Certainty of evidence: Very Low, Supplemental Table S4).
We identified seven comparative retrospective cohort studies assessing delayed primary excision (DPE) versus upfront resection. Of the seven, only 6 had extractable data enrolling 279 patients. The overall certainty of evidence was very low. Pooled results showed no significant difference in overall mortality, relapse, or need for additional intensive therapy. Incomplete resection was significantly lower in those with delayed primary excision compared to upfront resection (OR 0.27, 95% CI 0.15 to 0.51) including patients with RMS of the liver-bile duct (OR 0.05, 95% CI 0.00 to 0.52), extremity (OR 0.37, 95% CI 0.17 to 0.79), and mixed population (OR 0.09, 95% CI 0.02 to 0.48).