Machens Andreas, Lorenz Kerstin, Weber Frank, Dralle Henning
Department of Visceral, Vascular and Endocrine Surgery, Martin Luther University Halle-Wittenberg, Ernst-Grube-Straße 40, D-06097 Halle (Saale), Germany.
Department of General, Visceral and Transplantation Surgery, Section of Endocrine Surgery, University of Duisburg-Essen, Hufelandstraße 55, D-45147 Essen, Germany.
J Clin Endocrinol Metab. 2021 Jul 13;106(8):e2968-e2979. doi: 10.1210/clinem/dgab214.
Risk factors of lymph node and distant metastases have rarely been analyzed in hereditary and sporadic medullary thyroid cancer (MTC) using large genetic-clinical data sets.
This comprehensive investigation aimed to explore risk factors of lymph node and distant metastases and interdependencies between age at thyroidectomy, primary tumor size, lymph node metastasis, and distant metastasis in patients with hereditary and sporadic MTC.
We performed comparative analyses of risk factors of metastasis, stratified by hereditary MTC (4 mutational risk categories) and sporadic MTC.
There were 1115 patients with hereditary MTC (307 patients) or sporadic MTC (808 patients). Age at thyroidectomy increased proportionately from 12.2, 22.7, 34.3, and 49.8 years for patients with decreasing mutational risk, compared with 52.1 years for patients with sporadic MTC. Metastatic primary tumors overall were 10.7 to 19.4 mm larger in node-positive patients and 15.9 to 19.3 mm larger in distant metastatic patients at thyroidectomy than nonmetastatic tumors. Distant metastases were noted in 13% to 50% of node-positive vs 0% of node-negative hereditary MTC, and in 23.5% of node-positive vs 1.7% of node-negative sporadic MTC. In multivariable logistic regression analysis for sporadic MTC, lymph node metastasis contributed to distant metastasis (odds ratio 12.4) more than primary tumor size (odds ratios of 7.8, 5.5, and 2.4 for tumors measuring >60, 41-60, and 21-40 mm, respectively).
When thyroidectomy is performed before lymph node metastases have developed, distant metastases are exceptional, both in patients with hereditary MTC (irrespective of mutational risk level) and patients with sporadic MTC.
利用大型遗传临床数据集对遗传性和散发性甲状腺髓样癌（MTC）的淋巴结及远处转移危险因素进行分析的情况较为少见。
这项全面调查旨在探究遗传性和散发性MTC患者的淋巴结及远处转移危险因素，以及甲状腺切除年龄、原发肿瘤大小、淋巴结转移和远处转移之间的相互关系。
我们对转移危险因素进行了比较分析，按遗传性MTC（4种突变风险类别）和散发性MTC进行分层。
共有1115例遗传性MTC患者（307例）或散发性MTC患者（808例）。随着突变风险降低，遗传性MTC患者的甲状腺切除年龄从12.2岁、22.7岁、34.3岁和49.8岁呈比例增加，而散发性MTC患者为52.1岁。在甲状腺切除时，有淋巴结转移的患者中转移性原发肿瘤总体比无转移肿瘤大10.7至19.4毫米，有远处转移的患者中则大15.9至19.3毫米。在遗传性MTC中，13%至50%有淋巴结转移的患者出现远处转移，而无淋巴结转移的患者为0%；在散发性MTC中，有淋巴结转移的患者为23.5%，无淋巴结转移的患者为1.7%。在散发性MTC的多变量逻辑回归分析中，淋巴结转移对远处转移的影响（比值比12.4）大于原发肿瘤大小（肿瘤直径>60毫米、41 - 60毫米和21 - 40毫米时的比值比分别为7.8、5.5和2.4）。
在淋巴结转移发生前进行甲状腺切除时，无论是遗传性MTC患者（无论突变风险水平如何）还是散发性MTC患者，远处转移都很罕见。
