Highlights
- •Large prospective study of NRSTS with lymph node (LN) involvement in young patients.
- •Occult lymph node involvement is exceedingly rare.
- •Isolated LN metastases have a favourable prognosis so should be treated aggressively.
- •Complete enlarged LN excision and nodal bed radiation correlate with better outcome.
Abstract
Purpose
The aim of this paper is to better define the clinical features and outcomes of young
patients with non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) with regional and distant
lymph node (LN) metastases treated in a standardised fashion, we analysed LN involvement
in COG study ARST0332, which evaluated a risk-based treatment strategy for young patients
with all stages of NRSTS.
Patients and methods
Patients <30 years old with newly diagnosed NRSTS and LN metastases enrolled on ARST0332
were studied. Regional LN sampling was required for those with epithelioid sarcoma,
clear cell sarcoma or clinically/radiographically enlarged LNs. Tumour features and
extent of pre-enrolment resection determined treatment, including chemotherapy, radiotherapy,
and delayed surgery. Recommendations for LN metastases included LN dissection at the
time of primary tumour resection and dose-adapted radiotherapy based on extent of
LN resection.
Results
Twenty of 529 eligible and evaluable ARST0332 patients with NRSTS had LN metastases;
epithelioid sarcoma had the highest incidence (18%, 5 of 28). Pre-treatment imaging
identified LN enlargement in 19 of 20 patients; 1 had no pre-treatment LN imaging.
At 6.9 years median follow-up for surviving patients, 5-year overall survival was
85.7% (95% CI: 33.4%, 97.9%) for seven patients with isolated LN metastases and 15.4%
(95% CI: 2.5%, 38.8%) for 13 patients with additional extranodal metastases. LN recurrence
occurred in only one patient without LNs sampled at initial diagnosis.
Conclusion
LN metastases occur in about 4% of paediatric/young adult NRSTS, are limited to a
few histologic subtypes, and are rare in patients who did not have clinical or imaging
evidence of lymphadenopathy, suggesting that biopsies of non-enlarged LNs are not
necessary to identify occult involvement. Patients with isolated LN metastases have
high 5-year overall survival (∼85%) and should be treated with curative intent.
Clinicaltrials.gov Registry No.
NCT00346164.
Keywords
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Article info
Publication history
Published online: December 23, 2022
Accepted:
November 14,
2022
Received in revised form:
October 31,
2022
Received:
August 10,
2022
Footnotes
☆Statement of prior presentation: This research paper was presented at the American Society of Pediatric Hematology/Oncology annual meeting, April 2017.
Identification
Copyright
© 2022 Elsevier Ltd. All rights reserved.
