(1) Background: The wider adoption of a preoperative ultrasound and calcitonin screening complemented by an intraoperative frozen section has increased the number of patients with occult sporadic medullary thyroid cancer (MTC). These advances offer new opportunities to reduce the extent of the initial operations, minimizing operative morbidity and the risk of postoperative thyroxin supplementation without compromising the cure. (2) Methods: This systematic review of the international literature published in the English language provides a comprehensive update on the latest progress made in the risk-adapted surgery for sporadic and hereditary MTC guided by an intraoperative frozen section. (3) Results: The current evidence confirms the viability of a hemithyroidectomy for desmoplasia-negative sporadic MTC. To add an extra safety margin, the hemithyroidectomy may be complemented by a diagnostic ipsilateral central node dissection. Despite the limited extent of the surgery, all the patients with desmoplasia-negative sporadic tumors achieved a biochemical cure with excellent clinical outcomes. A hemithyroidectomy decreases the need for postoperative thyroxine substitution, but a total thyroidectomy may be required for bilateral nodular thyroid disease. Hereditary MTC is a different issue. Because each residual thyroid C cell carries its own risk of malignant progression, a total thyroidectomy remains mandatory for hereditary MTC. (4) Conclusion: In experienced hands, a hemithyroidectomy, which minimizes morbidity without compromising the cure, is an adequate therapy for desmoplasia-negative sporadic MTC.
（1）背景：术前超声与降钙素筛查的广泛应用，结合术中冰冻切片检查，使得隐匿性散发性甲状腺髓样癌（MTC）的检出率显著提高。这些进展为减少初始手术范围提供了新的可能，在确保治愈效果的同时，能够降低手术并发症风险及术后甲状腺素替代治疗的需求。（2）方法：本文通过对英文国际文献的系统性综述，全面总结了在术中冰冻切片引导下，针对散发性和遗传性MTC实施风险适应性手术的最新进展。（3）结果：现有证据证实，对于间质增生阴性的散发性MTC，单侧甲状腺切除术是可行的治疗方案。为增加安全边界，可在单侧甲状腺切除基础上联合同侧中央区淋巴结诊断性清扫。尽管手术范围有限，所有间质增生阴性的散发性MTC患者均实现了生化治愈，并获得了良好的临床预后。单侧甲状腺切除术降低了术后甲状腺素替代治疗的需求，但若合并双侧甲状腺结节性疾病，则仍需行全甲状腺切除术。遗传性MTC则需区别对待：由于残留的甲状腺C细胞均存在恶性进展风险，全甲状腺切除术仍是遗传性MTC的必需治疗方案。（4）结论：在经验丰富的医疗团队操作下，单侧甲状腺切除术是治疗间质增生阴性散发性MTC的适宜方案，能够在确保治愈效果的同时最大程度降低手术创伤。
Latest Progress in Risk-Adapted Surgery for Medullary Thyroid Cancer
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