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.