Fundamentals and Recent Advances in the Evaluation and Management of Medullary Thyroid Carcinoma
Molecular and Cellular Endocrinology(2024)
摘要
Medullary thyroid carcinoma (MTC) is a rare primary neuroendocrine thyroid carcinoma that is distinct from other thyroid or neuroendocrine cancers. Most cases of MTC are sporadic, although MTC exhibits a high degree of heritability as part of the multiple endocrine neoplasia syndromes. REarranged during Transfection (RET) mutations are the primary oncogenic drivers and advances in molecular profiling have revealed that MTC is enriched in druggable alterations. Surgery at an early stage is the only chance for cure, but many patients present with or develop metastases. C-cell-specific calcitonin trajectory and structural doubling times are critical biomarkers to inform prognosis, extent of surgery, likelihood of residual disease, and need for additional therapy. Recent advances in the role of active surveillance, regionally directed therapies for localized disease, and systemic therapy with multi-kinase and RET-specific inhibitors for progressive/metastatic disease have significantly improved outcomes for patients with MTC.
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关键词
Medullary Thyroid Carcinoma,Thyroid Cancer,Neuroendocrine Tumor,Rearranged During Transfection (RET),Next Generation Sequencing,Calcitonin,Carcinoembryonic antigen,Calcitonin Doubling Time,Positron Emission Tomography,Thyroidectomy,Modified Radical Neck Dissection,External Beam Radiation Therapy,Thermal Ablation,Embolization,Multi-kinase Inhibitors,Tyrosine Kinase Inhibitors,RET Inhibitors,RET Inhibitor resistance,Peptide receptor radionuclide therapy,Immunotherapy
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