家族性非髓样甲状腺癌临床特点分析

Clinicopathological features of familial non-medullary thyroid carcinoma

目的:探讨家族性非髓样甲状腺癌(familial non-medullary thyroid carcinoma,FNMTC)的临床特点。方法:回顾性分析天津医科大学肿瘤医院2011年12月至2015年1月收治的10个不同家系24例FNMTC患者,对照组随机选择此期间182例散发性非髓样甲状腺癌(sporadic non-medullary thyroid carcinoma,SNMTC),对比家族性和散发性之间,家族性第一代与第二代之间临床病理学特征。包括年龄、性别、双侧、多灶性、腺外侵犯、淋巴结转移、是否伴发良性结节及桥本甲状腺炎、预后。结果:FNMTC比SNMTC更易发生双侧病变(66.7%vs.17.0%,P<0.001),呈多灶性(45.8%vs.25.8%,P<0.050),伴发良性结节(70.8%vs.40.7%,P<0.010)和中央淋巴结转移(75.0%vs.35.2%,P<0.001)。家族性高危患者的比例高于散发组(33.3%vs.8.8%,P<0.05)。FNMTC中第二代患者疾病诊断年龄早于第一代[平均(45.6±10.3)vs.(35.3±5.4),P<0.05]。结论:双侧病变,多灶性,伴发良性结节和高中央淋巴结转移率是FNMTC的临床特征,其第二代患者疾病诊断年龄早于第一代。

Objective： To analyze the clinicopathological features of familial non-medullary thyroid carcinoma （FNMTC）. Methods： A retrospective investigation was performed in 24 FNMTC patients from 10 families and 182 sporadic cases who were diagnosed and treated in Tianjin Medical University Cancer Institute and Hospital. Clinicopathological features were analyzed between familial group and sporadic group as well as between the first generation and the later generations of FNMTC patients. Results： Compared with the patients with sporadic cancer, FNMTC patients were more likely to exhibit bilaterality （66.7% vs. 17.0%, P〈0.001）, multifocality （45.8% vs. 25.8%, P=0,041）, benign nodules （70.8% vs. 40.7%, P=0.005）, and central lymph node metastasis （75.0% vs. 35.2%, P〈0.001）. The proportion of American Thyrofd Association （ATA} high-risk patients with FNMTC was higher than the sporadic group （33.3% vs. 8.8%, P=0.029）. Comparison of the disease variants between the first generation and the later generations in FNMTC group showed that the latter were presented with disease at an earlier age at the time of diagnosis （mean 45.6±10.3 vs. 35.3±5.4, P=0.031）. Conclusion： Bilaterality, multifocality, presence of benign nodules, and central lymph node metastasis are special features of FNMTC. Moreover, the second generation has a younger age at the time of diagnosis compared with the first generation of FNMTC patients.