Panel deliberation.
Approximately one third of RMS patients will develop progression or
relapse with a dismal but variable outcome depending on tumor biology
and stage51. Predictors of the outcome of RMS relapse
include unfavorable site and size, FOXO fusion status, presence and
number of metastases, lymph node metastases, prior multimodal therapy,
and interval to relapse. Lower risk patients may benefit from aggressive
local and systemic multimodal therapy52-59. Relapse
patients with initially small tumors, favorable site, Group I, Stage 1,
and FOXO fusion negative have favorable
outcome51,56,57,60-63. Resection of relapsed disease
is suggested as part of the armamentarium for potentially salvageable
patients to improve survival53-59.
The panel suggests biopsy and diversion when indicated for patients
with RMS presenting emergently with obstruction (Weak
recommendation; Certainty of evidence: Very Low, Supplemental Table
S9)
We identified two retrospective cohort studies enrolling 488 patients
assessing upfront debulking surgery versus initial biopsy for locally
advanced RMS. The overall certainty of evidence is very low. Pooled
results did not indicate a significant difference in mortality or
failure-free mortality.