Panel deliberation.
Pretreatment re-excision is indicated when initial excisional biopsy or
resection leaves behind gross residual tumor, has microscopically
involved margins, or when the margin status is uncertain28-30. In such situations, pretreatment re-excision is
considered only when a wide re-excision can be achieved with the aim of
resecting all residual tumor with negative margins, without causing
significant surgical complications and undue delay in starting
chemotherapy. Pretreatment re-excision in RMS, particularly for trunk
and extremities30, plays a critical role in ensuring
margin-negative resection, downgrading risk stratification, and
de-intensification of therapy. Patients who achieve a negative resection
margin before starting chemotherapy with pretreatment re-excision are
classified as group 1, have improved survival, and potentially qualify
for reduced radiation dose31. All outcome analyses are
improved for group 1 in comparison to group 2 and group 3. Group 1
achieved by either upfront resection or pretreatment re-excision have
equally good outcomes28-30. In patients with group 1
disease, FOXO fusion-negative status may avoid the need for radiation
therapy32.
The panel suggests early preoperative radiation therapy for patients
with unresectable RMS. (Weak recommendation, Certainty of
evidence: Very Low, Supplemental Table S6)
We identified one retrospective cohort study enrolling 88 patients
assessing preoperative radiation therapy for unresectable tumors versus
no preoperative radiation therapy. The certainty of evidence is very
low. Pooled results did not indicate any significant difference in
overall mortality, mortality/event, or relapse. Incomplete resection was
significantly lower in patients undergoing preoperative RT versus no
preoperative RT (OR 0.02, 95% CI 0.00 to 0.08).