Uchino Shinya, Noguchi Shiro, Kawamoto Hitoshi, Yamashita Hiroyuki, Watanabe Shin, Yamashita Hiroto, Shuto Shigeru
Noguchi Thyroid Clinic and Hospital Foundation, 6-33 Noguchi Naka-machi, Beppu, Oita 874-0932, Japan.
World J Surg. 2002 Aug;26(8):897-902. doi: 10.1007/s00268-002-6615-y. Epub 2002 Apr 30.
First-degree relatives of persons with thyroid cancer are known to be at relatively high risk for the disease. To better understand the clinicopathologic characteristics of familial nonmedullary thyroid carcinoma (FNMTC), we carried out a retrospective study in which we identified individuals treated at our institution who had at least one first-degree relative with the disease. We used data obtained from our patient records to compare the features of 258 cases of the disease with the features of sporadic papillary or follicular thyroid carcinoma in another group of patients. The 258 patients represented 154 families and were selected from among 6458 patients with papillary or follicular thyroid carcinoma who underwent thyroidectomy between 1946 and 2000. Compared to the patients with sporadic disease, the FNMTC patients were more likely to have intraglandular dissemination (28.5% vs. 40.7%; p < 0.0001) and multiple benign nodules (29.8% vs. 41.5%; p <0.0001). There were no significant differences between the two types of patients in terms of gender, age, tumor diameter, adhesion to or invasion of the surrounding tissues, macroscopic metastasis observed at surgery, histology, presence of single benign nodules, presence of chronic thyroiditis, microscopic metastasis, or rate of lymph node metastasis. Recurrence was statistically frequent in the FNMTC patients compared with that in the sporadic disease patients (16.3% vs. 9.6%; p = 0.0005), and the disease-free survival rate was significantly poorer in the FNMTC patients (p = 0.0041 by the Wilcoxon test and p <0.0001 by the log-rank test). No significant difference in the overall survival rate was found between the two groups. Multivariate analysis by Cox's proportional hazards method showed FNMTC to be an independent predictor of shorter disease-free survival (risk ratio 1.88; confidence interval 1.35-2.54; p = 0.0003). Locoregional recurrence in the ipsilateral or contralateral lymph nodes and contralateral thyroid lobe was significantly more frequent in the FNMTC patients than in the sporadic disease patients, whereas no difference was found regarding distant metastases. We conclude that FNMTC is a clinically distinct entity with an aggressive nature. Because of the frequent presence of benign nodules, multifocality, and high rate of locoregional recurrence, total or near-total thyroidectomy with modified radical neck dissection in FNMTC patients is recommended.
甲状腺癌患者的一级亲属被认为患该病的风险相对较高。为了更好地了解家族性非髓样甲状腺癌（FNMTC）的临床病理特征，我们进行了一项回顾性研究，确定了在我们机构接受治疗且至少有一位患该病的一级亲属的个体。我们使用从患者记录中获得的数据，将258例该疾病患者的特征与另一组散发性乳头状或滤泡状甲状腺癌患者的特征进行比较。这258例患者代表154个家庭，是从1946年至2000年间接受甲状腺切除术的6458例乳头状或滤泡状甲状腺癌患者中挑选出来的。与散发性疾病患者相比，FNMTC患者更易出现腺内播散（28.5%对40.7%；p<0.0001）和多发良性结节（29.8%对41.5%；p<0.0001）。在性别、年龄、肿瘤直径、与周围组织的粘连或侵犯、手术时观察到的宏观转移、组织学、单个良性结节的存在、慢性甲状腺炎的存在、微观转移或淋巴结转移率方面，这两类患者之间没有显著差异。与散发性疾病患者相比，FNMTC患者的复发在统计学上更为频繁（16.3%对9.6%；p = 0.0005），并且FNMTC患者的无病生存率明显较差（Wilcoxon检验p = 0.0041，对数秩检验p<0.0001）。两组之间的总生存率没有显著差异。采用Cox比例风险法进行多变量分析显示，FNMTC是无病生存期较短的独立预测因素（风险比1.88；置信区间1.35 - 2.54；p = 0.0003）。FNMTC患者同侧或对侧淋巴结及对侧甲状腺叶的局部区域复发明显比散发性疾病患者更频繁，而在远处转移方面未发现差异。我们得出结论，FNMTC是一种具有侵袭性的临床独特实体。由于良性结节常见、多灶性以及局部区域复发率高，建议对FNMTC患者行全甲状腺切除或近全甲状腺切除并改良根治性颈淋巴结清扫术。
