Abstract：
Medullary thyroid carcinoma (MTC) is an uncommon malignancy of neuroendocrine origin derived from the parafollicular C cells. Although infrequent, the interest in this cancer exceeds its incidence owing to its distinctive features and its characteristic association with other endocrine tumors. Although the majority of MTCs are sporadic, hereditary varieties occur in isolation or as a part of multiple endocrine neoplasia type 2 syndrome (MEN 2). Currently, complete surgical resection of the tumor and nodal metastases with a curative intent remains the mainstay of therapy. The role of adjuvant therapy is limited, although radiotherapy and newer targeted therapies are routinely used for metastatic disease. The lack of consensus in the available guidance regarding the most appropriate diagnostic, therapeutic and follow-up strategies has caused substantial variability in clinical practice. Therefore, this review summarizes the latest available evidence and guidelines on the management of MTC with an emphasis on diagnosis, surgical treatment and follow-up.
摘要：
甲状腺髓样癌（MTC）是一种罕见的神经内分泌恶性肿瘤，起源于滤泡旁C细胞。虽然不常见，由于其独特的特征以及与其他内分泌肿瘤的特征性关联，对这种癌症的兴趣超过了其发病率。尽管大多数MTC是零星的，遗传性变种单独发生或作为多发性内分泌肿瘤2型综合征（MEN2）的一部分。目前,具有治愈性目的的完整手术切除肿瘤和淋巴结转移仍然是治疗的主要手段.辅助治疗的作用是有限的，尽管放疗和较新的靶向治疗通常用于转移性疾病。在关于最合适的诊断的现有指南中缺乏共识，治疗和随访策略在临床实践中引起了巨大的差异。因此,这篇综述总结了关于MTC管理的最新可用证据和指南，重点是诊断，手术治疗和随访。
