期刊
ESMO OPEN
卷 6, 期 3, 页码 -出版社
ELSEVIER
DOI: 10.1016/j.esmoop.2021.100183
关键词
medullary thyroid cancer; neuroendocrine neoplasms; tyrosine kinase inhibitors; RET inhibitors; endocrine surgery
类别
Medullary thyroid cancer (MTC) is a rare neuroendocrine tumor that requires multidisciplinary management. Various oncogenic pathways are involved in MTC pathogenesis, and targeted therapies have shown promising results. Multikinase inhibitors such as vandetanib and cabozantinib have been approved for advanced MTC treatment.
Medullary thyroid cancer (MTC) represents a rare neuroendocrine neoplasm originating from neoplastic C-cells in the thyroid gland. While localized disease is potentially curable with an optimized surgical approach, the number of relapses is high, and a considerable number of patients present with primary metastatic disease. Multidisciplinary management including standardized surveillance following surgery, but also early involvement of medical oncologists, is therefore important. Several oncogenic pathways are involved in the pathogenesis of MTC including vascular endothelial growth factor receptor, epidermal growth factor receptor, MET, and most importantly RET, and the multi-tyrosine kinase inhibitors vandetanib and cabozantinib have been approved for advanced MTC based on data from phase III studies. As activating RET mutations represent the most important driver, specific RET inhibitors were introduced and suggest high response rates with limited off-target toxicities. The current review provides a practical overview on clinical presentation and management from early to advanced MTC.
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