甲状腺髓样癌（MTC）是一种特殊类型的甲状腺癌，其恶性程度高于分化型甲状腺癌，早期即可发生区域淋巴结转移，其中上纵隔淋巴结转移发生率为18.2%～27.5%。然而，上纵隔淋巴结转移通常缺乏特异性症状，且受胸骨与锁骨阻隔，常规超声检查难以发现病灶。因此，有效的诊断与评估手段尤为重要。颈胸部增强CT能够较好地评估MTC上纵隔淋巴结转移情况及其与周围重要结构的关系，新型的68Ga 标记的CTR-FAPI正电子发射断层扫描/计算机断层扫描（68Ga-CTR-FAPI PET/CT）在显示MTC淋巴结及远处转移方面具有良好的诊断效能。当MTC出现上纵隔淋巴结转移时，规范的上纵隔淋巴结清扫是目前可能实现根治的唯一方式，并对病人预后具有直接影响。常用的手术方式包括经颈部切口直视下清扫、开胸清扫、经颈部入路腔镜辅助清扫、胸腔镜清扫及腔镜辅助联合胸腔镜清扫。腔镜手术具备放大、照明及拓展视野的优势，可克服直视手术中胸骨及锁骨阻挡带来的局限，同时避免开胸手术的创伤。对于合适的病人，经颈部入路腔镜辅助、胸腔镜或腔镜辅助联合胸腔镜方式能够实现规范化、微创化的上纵隔淋巴结清扫，其临床价值值得进一步探索与推广。此外，随着新型靶向药物的应用，尤其是针对 RET 基因突变的高选择性RET抑制剂（如普拉替尼、赛普替尼）的成功研发，为部分原本无法行完整病灶切除的MTC病人带来了新的治疗希望。术前基于病人肿瘤的基因突变信息选择合适的靶向药物进行新辅助治疗，可望取得满意疗效。
Abstract
Medullary thyroid carcinoma (MTC) is a distinct subtype of thyroid carcinoma with higher malignancy than differentiated thyroid carcinoma and regional lymph node metastasis can occur at an early stage. The incidence of superior mediastinal lymph node metastasis is reported to be 18.2%-27.5%. However, such metastases usually lack specific symptoms and are often overlooked due to obstruction by the sternum and clavicle, which limits the detection of lesions by routine ultrasound examination. Therefore, effective diagnostic and assessment modalities are of particular importance. Contrast-enhanced CT of the neck and chest can provide a reliable evaluation of superior mediastinal lymph node metastasis in MTC and its relationship with adjacent critical structures. The novel 68Ga-labeled CTR-FAPI positron emission tomography/computed tomography (68Ga-CTR-FAPI PET/CT) has shown promising diagnostic performance in detecting nodal and distant metastases of MTC. When superior mediastinal lymph node metastasis is present, standardized dissection of the superior mediastinum is currently the only potentially curative approach and has a direct impact on prognosis. Common surgical approaches include transcervical direct dissection, thoracotomy, transcervical endoscopic-assisted dissection，thoracoscopic dissection，and combined transcervical endoscopic-assisted thoracoscopic dissection. Endoscopic surgery offers the advantages of magnification, illumination and extended visualization, helping to overcome the limitations of open surgery imposed by the sternum and clavicle while avoiding the trauma of thoracotomy. For appropriately selected patients, transcervical endoscopic-assisted thoracoscopic or combined approaches can achieve standardized and minimally invasive superior mediastinal lymph node dissection with clinical value warranting further exploration and promotion. In addition, with the advent of novel targeted therapies, especially highly selective RET inhibitors such as pralsetinib and selpercatinib for RET mutations, new therapeutic opportunities have emerged for patients with MTC who were previously unsuitable for complete surgical resection. Preoperative neoadjuvant therapy guided by the patient’s tumor mutation profile may yield favorable outcomes.
关键词
甲状腺髓样癌 /
上纵隔转移 /
腔镜辅助 /
胸腔镜 /
新辅助治疗
Key words
medullary thyroid carcinoma /
superior mediastinal metastasis /
video-assisted /
thoracoscopy /
neoadjuvant therapy
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