Panel deliberation.
Most (61-75%) localized RMS are unresectable at presentation and resection with negative margins is achievable in only 12 to 18% of patients14-16. Opting for upfront versus delayed primary excision of RMS depends on feasibility of a resulting microscopic negative margins and function preservation14,17. While upfront resection for paratesticular RMS is almost always possible; this is not necessarily the case for other locations. There is no role for upfront resection when achieving negative margins is not feasible or when upfront resection is mutilating. Determinants of resectability include tumor site, size, and relationship to critical structures. RT can be safely omitted if upfront R0 resection can be achieved, and the tumor is negative for translocation. However, there is a paucity of well-defined clinical criteria to guide selection for upfront resection versus delayed primary excision.
Neoadjuvant chemotherapy decreases the size, often alters the anatomic relationship of tumors to critical structures and improves feasibility of function-preserving resection18-23. Delayed primary excision after neoadjuvant chemotherapy potentially qualifies patients for radiation dose reduction24,25 and improves overall survival for extremity and non-bladder-prostate genitourinary RMS14,17,26.
Debulking surgery offers no local control or survival advantages; therefore, debulking has no role in curative RMS resection. Biopsy sites should be planned to facilitate en bloc resection of the biopsy tract at the time of surgical local control.
The panel suggests pretreatment re-excision in patients with incompletely resected RMS (Weak recommendation, Certainty of evidence: Very Low, Supplemental Table S5).
We identified three retrospective cohort studies enrolling 284 patients assessing pretreatment re-excision versus no pretreatment re-excision. The overall certainty of evidence is very low. Pooled results did not show any difference in overall mortality or relapse. In patients with RMS of the extremity or trunk, mortality was significantly lower in those with pretreatment excision versus no pretreatment excision (OR 0.31, 95% CI 0.11 to 0.92).