Anaplastic thyroid cancer (ATC) is a devastating and usually incurable diagnosis. Clinical and pathologic diagnosis is best assessed at a tertiary center with concentrated ATC expertise. Expeditious multidisciplinary management is recommended for optimal patient outcomes. Based on multiinstitutional and population-based studies, multimodal therapy that includes chemoradiotherapy with surgery (when feasible) is the preferred initial treatment because it is associated with incrementally improved overall survival. In ATC that carries a BRAF V600E somatic mutation, combination therapy with BRAF and MEK inhibitors has shown promise but needs further study. Immunotherapeutic agents in neoadjuvant and metastatic settings are being investigated.
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