Clinical Outcomes of Diffuse Sclerosing Variant Papillary Thyroid Carcinoma in Pediatric Patients

Objectives/Hypothesis The diffuse sclerosing variant of papillary thyroid carcinoma (DSV) may be more aggressive than conventional well‐differentiated non‐DSV related papillary thyroid carcinomas (N‐PTC). Study Design Retrospective chart review. Methods Retrospective review of clinical outcomes for...

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Published in:The Laryngoscope 2022-05, Vol.132 (5), p.1132-1138
Main Authors: Brady, Charles, Manning, Scott C., Rudzinski, Erin, Paulson, Vera, Wang, Xing, Liu, Yajuan J., Parikh, Sanjay R., Bonilla‐Velez, Julianna, Hawkins, Douglas S., Dahl, John
Format: Article
Language:English
Subjects:
Adenocarcinoma
Carcinoma, Papillary
Child
Clinical outcomes
Diffuse sclerosing variant
Humans
Laryngoscopy
Metastasis
molecular analysis
papillary thyroid carcinoma
pediatric thyroid surgery
Pediatrics
progression of disease
Retrospective Studies
Thyroid cancer
Thyroid Cancer, Papillary - pathology
Thyroid Cancer, Papillary - surgery
Thyroid Neoplasms - pathology
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Summary:Objectives/Hypothesis The diffuse sclerosing variant of papillary thyroid carcinoma (DSV) may be more aggressive than conventional well‐differentiated non‐DSV related papillary thyroid carcinomas (N‐PTC). Study Design Retrospective chart review. Methods Retrospective review of clinical outcomes for patients 21 years of age or younger who underwent initial surgery for PTC at a single institution from January 1, 2005 to April 1, 2020. Genomic analysis was performed using targeted next‐generation sequencing. Data were analyzed using Fischer's exact test and Kaplan–Meier curve log‐rank test. Results Our cohort consisted of 72 patients, nine with DSV and 63 with N‐PTC. Age at diagnosis was comparable (15.4 vs. 16.2 years, respectively, P = .46). DSV were more likely to be in the high‐risk American Thyroid Academy pediatric risk group (100% vs. 41.3%, P = .004), to present with regional cervical lymph node metastases (100% vs. 60.3%, P = .036), and to present with distant metastases (67% vs. 22%, P = .005). No mortality seen in either group over 27.5 (interquartile range 14.8, 46.00) months average follow‐up. Throughout the follow‐up period, DSV were more likely to experience progression than N‐PTC (hazard ratio = 5.7 [95% confidence interval 1.7–20.0; P = .0056]). In a subset of 19 patients with aggressive disease who had molecular testing as part of clinical care we detected RET fusions in nearly all DSV compared to a minority of N‐PTC (83% vs. 15.4%, P = .0095). Conclusions Pediatric patients with DSV have more advanced disease at diagnosis and are more likely to experience progression of disease compared to patients with N‐PTC. The prevalence of RET fusions in our cohort recapitulates the frequency of this alteration described in prior studies. Level of Evidence 4 Laryngoscope, 132:1132–1138, 2022
ISSN:0023-852X
1531-4995
DOI:10.1002/lary.29926