Papillary Thyroid Carcinoma in Children: Prevalence, Morphology, Molecular and Cytogenetic Characteristics, and Prognostic Determinants Publisher Pubmed

Summary: Why can childhood thyroid cancer look severe yet end well? A review notes spread at diagnosis but favorable survival and limited follow-up data. #ThyroidCancer #Pediatrics

Abdollahi A ; Avarjeh M
Source: Journal of Pediatric Endocrinology and Metabolism Published:2026

Abstract

Papillary thyroid carcinoma (PTC) stands as the most frequently encountered endocrine malignancy in children and adolescents. A notable clinical feature of pediatric PTC is that while young patients often present with advanced disease – including lymph node involvement and distant metastases – long-term survival rates remain high. This presentation underscores pediatric PTC as a biologically distinct entity. The molecular landscape differs fundamentally from adult disease, with gene fusions involving RET/PTC, NTRK, and ALK serving as primary drivers, while BRAF V600E point mutations play a lesser role. This narrative review examines the prevalence, morphological features, molecular and cytogenetic characteristics, and prognostic determinants of pediatric papillary thyroid carcinoma. We explore how these children frequently present with multifocal tumors, extensive nodal metastases, and pulmonary spread yet maintain favorable clinical outcomes. The review critically evaluates current approaches to surgery, radioactive iodine therapy, and risk-adapted surveillance strategies. We also consider the growing role of targeted therapies – particularly RET and NTRK inhibitors – in managing refractory or metastatic disease, while acknowledging that long-term safety data in children remain limited. Available evidence indicates that pediatric PTC represents a biologically and clinically distinct entity requiring a tailored management paradigm. While outcomes are generally favorable, significant questions remain about epidemiology, molecular drivers, treatment resistance, and long-term survivorship effects. Moving forward, progress will depend on international collaboration, standardized data collection, and extended follow-up to develop pediatric-specific biomarkers and therapeutic approaches. © 2026 Walter de Gruyter GmbH, Berlin/Boston.