摘要
目的分析家族性甲状腺非髓样癌（FNMTC）患者的临床特征。方法回顾性分析2008年1月至2012年11月在天津医科大学肿瘤医院诊治的32个FNMTC家系的66例患者的临床病理资料，同时按比例随机抽取同期收治的18：2例散发性甲状腺乳头状癌（SPTC）作为对照。为更加客观地分析具有亲子关系的FNMTC患者的临床特征，另引用文献中的部分数据进行结合分析。结果FNMTC患者的中位年龄为44岁，女性57例（86．4％），其中具有同胞关系的家系23个，共47例（71．2％）患者；具有亲子关系的9个，共19例（28．8％）患者；第一代9例，第二代10例。FNMTC和SPTC在病灶多中心性、病灶双侧性、淋巴结转移、中央区淋巴结转移、伴发桥本氏甲状腺炎和复发方面的差异均有统计学意义（均P〈0．05）。将家系进一步分为具有同胞关系和具有亲子关系与SPTC进行比较时，仅中央区淋巴结转移率高的特点表现在具有同胞关系的家系中，病灶双侧性及复发率高的特点则呈现在具有亲子关系的家系中（均P〈0．05）。此外，第二代FNMTC患者的中位确诊年龄较第一代早、男性患者比例高，且具有病灶多中心性、病灶双侧性及淋巴结转移率高的特点。结论FNMTC恶性程度可能更高，且第二代FNMTC患者可表现出遗传早现现象。在临床工作中，应更加仔细地询问患者一级亲属中甲状腺癌的发病情况，并对其家庭成员进行长期随访，以便向患者和家属提供更加个体化的诊治和观察建议。
Objective To analyze the clinical and biological features of familial nonmedullary thyroid carcinoma （FNMTC）. Methods Clinical data of 66 FNMTC cases of 32 pedigrees was retrospectively analyzed, compared with that of 182 control cases taken randomly from the patients with sporadic papillary thyroid carcinoma （SPTC）, who were diagnosed and treated in Tianjin Cancer Hospital between January 2008 and November 2012. The features of FNMTC of the first and second generations were objectively analyzed. Some data quoted from the literature were also used for the analysis. Results The median age at diagnosis of all the 66 FNMTC patients was 44 years, and 57 （86.4%） were females. Moreover, 71.2% （47 patients, 23 pedigrees） of the FNMTC patients exhibited a sibling relationship, and 28.8% （ 19 patients, 9 pedigrees） of the FNMTC patients exhibited a parent-offspring relationship, and 9 cases in the first generation and 10 cases in the second generation. There were significant differences between the FNMTC group and SPTC group in terms of tumor multicentricity, tumor bilaterality, lymph node metastasis, central lymph node metastasis, concomitant chronic thyroiditis and recurrence （ P 〈 0.05 ）. Compared with SPTC, sibling FNMTC presented a higher rate of central lymph node metastasis, while parent-offspring FNMTC showed frequent tumor bilaterality and a higher rate of recurrence （ P 〈 O. 05 ）. Besides, patients in the second generation were diagnosed at an earlier age and had a higher male rate, the tumors were more frequently multifocal and bilateral, and had a higher rate of lymph node metastasis. Conclusions FNMTC may be more aggressive than SPTC and patients in the second generation may exhibit the ＂anticipation＂ phenomenon. It＇s necessary to make sufficient detailed interrogation and long-term follow- up of the patients and their family for providing individual recommendations for clinical management.
出处
《中华肿瘤杂志》
CAS
CSCD
北大核心
2014年第3期202-206,共5页
Chinese Journal of Oncology
基金
国家自然科学基金（81272282）
天津市高等学校科技发展基金（20110127）
