髓性甲状腺癌（MTC）占据了约5-10％的甲状腺癌症例。虽然肿瘤发生在甲状腺中，但它并不是源自甲状腺细胞，而是源自产生和释放一种称为降钙素（CT）的椭圆滤泡细胞或副滤泡细胞。从20世纪下半叶开始，人们逐渐研究和定义了MTC。本研究旨在分析MTC的历史、临床表现和生物学行为，生物体液和器械诊断，分子特征分析，遗传筛查，术前分期和器械操作等方面。这些方面在专业和熟练的操作者手中是不可或缺的，如高分辨率超声、CT扫描、MRI和PET/TC。我们将研究推荐和有争议的手术指征和程序，预防性早期手术和多发性内分泌肿瘤手术。此外，我们还将讨论病理解剖学分类和靶向治疗。血清CT的作用被评价为在术前诊断标记上起着不可争议和恒定的作用，对细胞学有遮蔽作用，并可作为预测疾病持久性的早期术后标记。通过进行全面的术前研究，可以减少不必要、无用、迟遇和扩展性的干预措施，支持因人制宜的手术方案，同时考虑到生活质量。最后，针对性治疗取得了巨大进展，对生存率产生了有利的影响。

Medullary thyroid carcinoma (MTC) constitutes approximately 5-10% of all thyroid cancers. Although the tumor forms in the thyroid, it doesn't originate from thyroid cells, but from the C cells or parafollicular cells which produce and release a hormone called calcitonin (CT). Starting from the second half of the 1900s, MTC was progressively studied and defined.This study aims to analyze the history, clinical presentation and biological behavior of MTC, bio-humoral and instrumental diagnosis, molecular profiling, genetic screening, preoperative staging and instrumental procedures, indispensable in expert and dedicated hands, such as high-resolution ultrasonography, CT-scan, MRI and PET/TC. We examine recommended and controversial surgical indications and procedures, prophylactic early surgery and multiple endocrine neoplasia surgery. Also, we discuss pathological anatomy classification and targeted therapies. The role of serum CT is valued both as undisputed and constant preoperative diagnostic marker, obscuring cytology and as early postoperative marker that predicts disease persistence.With a complete preoperative study, unnecessary or useless, late and extended interventions can be reduced in favor of tailored surgery that also considers quality of life. Finally, great progress has been made in targeted therapy, with favorable impact on survival.