Age Stratifies Disease Extent in Pediatric and Young Adult Fusion-Positive Papillary Thyroid Carcinoma
Sule Canberk, Zubair W Baloch, Anna Maria Carillo, Amber Isaza, Rita Barros, Mya Bojarsky, Andrew J BauerAbstract
Context
Fusion-positive pediatric papillary thyroid carcinoma (PTC) often presents with regional nodal or distant disease, but factors explaining heterogeneity within fusion-positive tumors are incompletely defined.
Objective
To determine whether age, fusion group, or sex is associated with baseline N1b and/or M1 disease in fusion-positive pediatric and young adult PTC.
Design
Retrospective analysis of the international PEDIMAP cohort.
Setting
Multi-institutional pediatric and young adult thyroid carcinoma cohort.
Patients
Patients aged 0-25 years with PTC, documented somatic kinase fusion, and available fusion-partner information.
Intervention(s)
None.
Main Outcome Measure(s)
Advanced baseline presentation, defined as AJCC N1b and/or M1 disease. Associations were tested using Firth penalized logistic regression.
Results
Of 101 kinase fusion-positive PTCs identified among 646 PTCs, 2 RET-fusion cases were excluded because fusion-partner information was unavailable, yielding 99 fusion-positive tumors with known fusion partners: RET, n = 52; NTRK, n = 28; and other non-RET/NTRK kinase fusions, n = 19. Among 95 patients with evaluable N and M stage, 64 (67.4%) had N1b and/or M1 disease. Age 0–14 years was associated with higher odds of advanced presentation than age 15–25 years (adjusted OR, 4.67; 95% CI, 1.84–12.75; P = 0.001). No significant difference in advanced presentation was found between NTRK and RET fusions (adjusted OR, 0.83; 95% CI, 0.29–2.50; P = 0.739).
Conclusions
Within fusion-positive pediatric and young adult PTC, younger age was associated with greater baseline disease extent, whereas no significant difference in N1b/M1 presentation was found between RET and NTRK fusion groups.These findings support careful baseline assessment of regional and distant disease in younger fusion-positive patients.