Endocrine Unit, Department of Clinical and Experimental Medicine, University of Pisa, Via Paradisa 2, Pisa 56124, Italy.
Endocrine Unit, Department of Clinical and Experimental Medicine, University of Pisa, Via Paradisa 2, Pisa 56124, Italy.
Endocrinol Metab Clin North Am. 2019 Mar;48(1):285-301. doi: 10.1016/j.ecl.2018.11.006. Epub 2018 Dec 26.
Medullary thyroid cancer (MTC) is rare but aggressive. It can be cured only if intrathyroid at diagnosis. MTC can be sporadic (75%) or familial (25%) and the 2 forms are distinguished by RET mutations analysis. Calcitonin is the specific serum marker; its doubling time is the most important prognostic factor for survival and progression; 30% of MTC patients have distant metastases at diagnosis and, when progressing, systemic therapy with vandetanib or cabozantinib should be considered. Before starting this treatment, the possibility of using a local treatment should be evaluated to delay systemic therapy. A multidisciplinary team should care for these patients.
甲状腺髓样癌(MTC)较为罕见但侵袭性强。只有在诊断时位于甲状腺内才能被治愈。MTC 可散发(75%)或家族性(25%),两种形式可通过 RET 突变分析进行区分。降钙素是特异性血清标志物;其倍增时间是预测生存和进展的最重要预后因素;30%的 MTC 患者在诊断时已有远处转移,在进展时,应考虑使用凡德他尼或卡博替尼进行全身治疗。在开始这种治疗之前,应评估局部治疗的可能性,以延迟全身治疗。多学科团队应负责照顾这些患者。