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).