Highlights
- •Pediatric non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) are heterogeneous.
- •The chances of being cured for NRSTS patients who relapsed are limited.
- •In the series, median post-relapse survival was 20 months, 10-year survival was 19.1%.
- •Survivals were better after local and late relapses and after secondary remission.
- •Prognostic factors were combined in a risk-adapted model.
Abstract
Purpose
Though the prognosis for patients with pediatric non-rhabdomyosarcoma soft tissue
sarcomas (NRSTS) is generally good, the chances of being cured after relapse are limited.
This report describes a series of relapsing NRSTS patients treated at a referral center
for pediatric sarcoma, investigating the pattern of relapse, salvage rates, and factors
correlating with the final outcome.
Methods
The analysis concerned 103 patients <21 years old with relapsing adult-type NRSTS
treated from 1985 to 2020. For risk-adapted stratification purposes, the patient outcome
was examined using univariable and multivariable analyses based on patients’ clinical
features at first diagnosis, first-line treatments, clinical findings at first relapse,
and second-line treatments.
Results
The first relapse occurred within 2–102 months (median 14 months) after patients'
first diagnosis and was local in 47%, metastatic in 34%, and both in 19%. Treatment
at relapse included chemotherapy in 72 patients, radiotherapy in 38, and surgery in
55. The median overall survival (OS) was 20 months. Post-relapse OS was 56.1%, 25.8%,
and 19.1% at 1, 5, and 10 years, respectively. Cox's multivariable regression analysis
showed that OS was significantly better for patients with local and late relapses
(occurring more than 12 months after their first diagnosis) and for those achieving
secondary remission.
Conclusion
The outcome of patients with recurrent NRSTS is poor. The above-mentioned variables
(type and time of relapse and achievement of secondary remission) were combined in
a risk-adapted model to develop a tool for estimating the chance of salvage and deciding
the best second-line treatment approach.
Keywords
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Article info
Publication history
Published online: May 14, 2022
Accepted:
March 29,
2022
Received in revised form:
March 14,
2022
Received:
February 17,
2022
Identification
Copyright
© 2022 Elsevier Ltd. All rights reserved.
