Local Control and Survival Outcomes in Pediatric Non-rhabdomyosarcoma
Soft Tissue Sarcoma: The Mayo Clinic Experience
Abstract
Objective: Pediatric patients with non-rhabdomyosarcoma soft tissue
sarcoma (NRSTS) undergo aggressive multimodality treatment. This single
institutional analysis of outcomes in this group explored factors
associated with local control and survival. Methods: Patients ≤18
years old diagnosed with NRSTS between 10/1990-11/2021 who received
radiotherapy (RT) were retrospectively identified. Overall Survival (OS)
and disease-free survival (DFS) analyses were performed using Kaplan
Meier methods. Cumulative incidence of local and distant failure were
estimated. Univariate analysis was performed. Results: Fifty-one
pediatric patients with NRSTS were included. Extremity tumors were most
common (33/51, 65%), with 80% T1 or T2 (AJCC 8 th)
and 90% non-metastatic. Surgery in 49/51 (96%) resulted in 32 (65%)
R0 resections. All received RT (median EQD2 50 Gy, range 42.4-76.9),
with brachytherapy or intraoperative (IORT) boosts in 21 cases (41%)
(mean EQD2 24.5 Gy). Thirty-four (66.7%) received chemotherapy. With
5.7 years median follow up, 5-year OS and DFS were 70.7% and 67.9%,
respectively. Positive margins were significantly associated with worse
OS on univariate analysis. Two-year local failure incidence for EBRT was
18.1% and 5.3% for EBRT+boost (HR 3.67, p=0.24). Among 6 observed
local failures, 5 occurred after EBRT alone, 3 of which had positive
margins. Among the EBRT+boost group, 2 had positive margins, neither
with local recurrence. Conclusion: Multimodality treatment of
pediatric NRSTS results in 5-year OS and DFS around 70%. R0 resection
is essential to optimize outcomes in this population. Although incidence
of R1 resections has declined in the modern era, IORT/brachytherapy
boost may have a role in selected patients at high-risk for local
recurrence.