Machens Andreas, Dralle Henning
Department of Visceral, Vascular and Endocrine Surgery, University Hospital, Medical Faculty, University of Halle-Wittenberg, Halle, Germany.
Department of General, Visceral and Transplantation Surgery, Division of Endocrine Surgery, University of Duisburg-Essen, Essen, Germany.
Recent Results Cancer Res. 2025;223:247-266. doi: 10.1007/978-3-031-80396-3_10.
Medullary thyroid cancer (MTC) is an infrequent calcitonin-secreting thyroid malignancy that can vary a great deal in tumor biology and progression. The most important determinant of distant metastases, which represents the single greatest risk factor of poor cancer-specific survival, is lymph node metastasis in the neck and mediastinum. In patients with node-positive MTC, systematic compartment-oriented dissection of central and lateral neck nodes at the initial operation promotes biochemical cure and decreases the risk of locoregional recurrence in previously dissected lymph node compartments. Routine calcitonin screening in nodular thyroid disease and genomic screening, flanked by advances in ultrasound and functional imaging modalities, have been pivotal in making the diagnosis early and enabling risk-reducing surgical interventions in patients with sporadic and hereditary MTC, without compromising clinical outcome. To assess a RET carrier's risk of medullary thyroid cancer, all that is needed is patient age, underlying RET mutation, and biomarker levels. Recently, risk-reducing surgery, consisting in hemithyroidectomy with or without diagnostic ipsilateral central lymph node dissection, has been advocated for sporadic MTC clinically confined to one thyroid lobe that is desmoplasia negative on intraoperative frozen sectioning. These recent developments reflect the current shift from reactive to increasingly preemptive, risk-reducing surgery for MTC.
甲状腺髓样癌（MTC）是一种罕见的分泌降钙素的甲状腺恶性肿瘤，其肿瘤生物学特性和进展情况差异很大。远处转移的最重要决定因素是颈部和纵隔淋巴结转移，这是癌症特异性生存率低的单一最大风险因素。对于淋巴结阳性的MTC患者，初次手术时对中央区和侧颈部淋巴结进行系统性分区清扫可促进生化治愈，并降低先前清扫过的淋巴结分区局部复发的风险。结节性甲状腺疾病的常规降钙素筛查以及基因组筛查，再加上超声和功能成像技术的进步，对于早期诊断以及在不影响临床结局的情况下对散发性和遗传性MTC患者进行降低风险的手术干预起到了关键作用。要评估RET基因携带者患甲状腺髓样癌的风险，只需了解患者年龄、潜在的RET基因突变和生物标志物水平即可。最近，对于临床局限于一个甲状腺叶且术中冰冻切片显示无促纤维增生性改变的散发性MTC，有人主张进行包括患侧甲状腺叶切除及同侧中央区淋巴结清扫（有或无诊断性清扫）的降低风险手术。这些最新进展反映了目前对MTC手术从反应性手术向越来越具有预防性的降低风险手术的转变。
