Adeline T Yang, Sin-Ting Tiffany Lai, Theodore W Laetsch, Tricia Bhatti, Zubair Baloch, Lea F Surrey, Aime T Franco, Julio C Marques Ricarte-Filho, Sogol Mostoufi-Moab, N Scott Adzick, Ken Kazahaya, Andrew J Bauer
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Molecular Landscape and Therapeutic Strategies in Pediatric Differentiated Thyroid Carcinoma.
There has been significant progress in understanding the molecular landscape of pediatric differentiated thyroid carcinoma (DTC) over the past 2 decades. Classification of pediatric DFC into 3 tiers, RAS-like mutant, BRAF mutant, and kinase fusions, accurately reflects an increasing risk for invasive behavior, including regional and distant metastasis. In clinical practice, somatic oncogene testing for nodules with indeterminate cytology per the Bethesda System for Reporting Thyroid Cytopathology provides objective data to optimize surgical planning. In addition, knowledge of the somatic oncogene for widely invasive carcinomas allows for incorporation of oncogene-specific inhibitory therapy both in the adjuvant and neoadjuvant setting. In the present review, we review the risk factors, clinical presentation, and evaluation of pediatric DTC, highlighting the correlation among ultrasound features, cytology, and oncogenic driver of the tumor. We subsequently propose an integrated, multimodal approach that can be used to improve diagnostic accuracy and reliability for preoperative planning as well as identify and discuss which pediatric patients may benefit from systemic oral targeted therapy.
期刊介绍:
Endocrine Reviews, published bimonthly, features concise timely reviews updating key mechanistic and clinical concepts, alongside comprehensive, authoritative articles covering both experimental and clinical endocrinology themes. The journal considers topics informing clinical practice based on emerging and established evidence from clinical research. It also reviews advances in endocrine science stemming from studies in cell biology, immunology, pharmacology, genetics, molecular biology, neuroscience, reproductive medicine, and pediatric endocrinology.