Maxwell Evelyn Linda, Hall Francis T, Freeman Jeremy L
Mount Sinai Hospital, 600 University Avenue-401, Toronto, Ontario M5G 1X5, Canada.
Laryngoscope. 2004 Dec;114(12):2182-6. doi: 10.1097/01.mlg.0000149454.91005.65.
Familial non-medullary thyroid cancer (FNMTC) is a newly recognized disease entity and can be distinguished from the more common sporadic non-medullary thyroid cancer. The purpose of this study was to determine some of the potential distinguishing features of FNMTC.
Retrospective association study and matched-case control study.
Five hundred forty-three cases of well-differentiated follicular origin thyroid cancers were identified and collected in a database. Among this population, 24 cases of FNMTC were identified. A case of FNMTC was defined as a patient with the following two criteria: a well-differentiated follicular origin thyroid cancer and at least one first-degree relative with a well-differentiated epithelial origin thyroid cancer. The unmatched sporadic and FNMTC groups were compared using t test, Phi test, Cramer V test, and Pearson and Spearman correlation tests. Twenty-four FNMTC cases were matched to 24 sporadic cases based on age, gender, stage of disease at presentation, and tumor size. Clinicopathologic features, management, and outcome were analyzed statistically using a matched-proportional z test. Disease-free survival and disease-specific survival were analyzed using log-rank test and the Kaplan-Meier function. A P-value less than .05 was considered statistically significant.
: There was no significant difference in ionizing radiation exposure, disease multifocality, surgical management, or recurrence between the sporadic and FNMTC patients. Although FNMTC patients tend to have improved disease-free survival and disease-specific survival, the difference was not significant at the 5% level.
Although FNMTC is characterized by strong family history, these patients do not tend to have worse prognosis.
家族性非髓样甲状腺癌（FNMTC）是一种新认识的疾病实体，可与更常见的散发性非髓样甲状腺癌相区分。本研究的目的是确定FNMTC的一些潜在鉴别特征。
回顾性关联研究和配对病例对照研究。
在一个数据库中识别并收集了543例分化良好的滤泡源性甲状腺癌病例。在这一群体中，识别出24例FNMTC。一例FNMTC被定义为符合以下两条标准的患者：分化良好的滤泡源性甲状腺癌，以及至少一位患有分化良好的上皮源性甲状腺癌的一级亲属。使用t检验、Phi检验、Cramer V检验以及Pearson和Spearman相关检验对未配对的散发性和FNMTC组进行比较。根据年龄、性别、就诊时疾病分期和肿瘤大小，将24例FNMTC病例与24例散发性病例进行配对。使用配对比例z检验对临床病理特征、治疗和结局进行统计学分析。使用对数秩检验和Kaplan-Meier函数分析无病生存期和疾病特异性生存期。P值小于0.05被认为具有统计学意义。
散发性和FNMTC患者在电离辐射暴露、疾病多灶性、手术治疗或复发方面无显著差异。虽然FNMTC患者的无病生存期和疾病特异性生存期往往有所改善，但在5%的水平上差异不显著。
虽然FNMTC的特点是有很强的家族史，但这些患者的预后往往并不更差。
