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).