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Familial Nonmedullary Thyroid Carcinoma: A Retrospective Analysis of 117 Families 被引量:4
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作者 Ya-Bing Zhang Xiao-Xin Wang +4 位作者 Xi-Wei Zhang Zheng-Jiang Li Jie Liu Zhen-Gang Xu Ping-Zhang Tang 《Chinese Medical Journal》 SCIE CAS CSCD 2018年第4期395-401,共7页
Background: The first and most important step in characterizing familial nonmedullary thyroid carcinoma (NMTC) is to distinguish the true familial patients, which is the prerequisite for all accurate analyses. This... Background: The first and most important step in characterizing familial nonmedullary thyroid carcinoma (NMTC) is to distinguish the true familial patients, which is the prerequisite for all accurate analyses. This study aimed to investigate whether patients from families with ≥3 first-degree relatives affected with NMTC have different characteristics than patients from families with only two affected members, and to compare these patients with those with sporadic disease. Methods: We analyzed the clinicopathological features and prognosis of 209 familial and 1120 sporadic cases of NMTC. Familial patients were further divided into two subgroups: families with two affected members and families with ≥3 affected members. Results: The familial group had a significantly higher risk of bilateral growth, multifocality, extrathyroidal extension, and lateral lymph node metastasis than the sporadic group (P 〈 0.05). These main features were also different between the group with ≥3 affected members and the sporadic group. The only difference between the two affected members' group and the sporadic group was incidence of multifocality (P 〈 0.05). The probability of disease recurrence in patients from families with ≥3 affected members was significantly higher than that in sporadic cases (14.46% vs. 5.27%; P = 0.001), while the probability in patients from families with two affected members was similar to that in sporadic patients (6.35% vs. 5.27%; P = 0.610). The Kaplan–Meier survival analysis showed a statistically significant difference in disease-free survival between the two subgroups (85.54% vs. 93.65%; P = 0.045). Conclusions: Patients from families with ≥3 members affected by NMTC have more aggressive features and a worse prognosis than those from families with only two affected members. Patients from families with ≥3 affected first-degree relatives may be considered to have true familial NMTC. 展开更多
关键词 carcinoma nonmedullary thyroid True familial
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Clinicopathologic Features of Familial Nonmedullary Thyroic Carcinoma 被引量:1
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作者 Yu-Fang Fan Bo Zhang +7 位作者 Xiao Yang Zhong-Hua Shang Hong-Feng Liu Yong Xie Yue-Wu Liu Wei-Sheng Gao Qiong Wu Xiao-Yi Li 《Chinese Medical Journal》 SCIE CAS CSCD 2015年第8期1037-1041,共5页
Background: Familial nonmedullary thyroid carcinoma (FNMTC) is a variant of nonmedullary thyroid carcinoma(NMTC) with particular clinicopathologic features. In recent years, a number of studies have shown that FN... Background: Familial nonmedullary thyroid carcinoma (FNMTC) is a variant of nonmedullary thyroid carcinoma(NMTC) with particular clinicopathologic features. In recent years, a number of studies have shown that FNMTC is more invasive than sporadic NMTC(SNMTC). The purpose of this study was to explore the differences in clinicopathologic features of FNMTC between different types of families and to determine in which of these families more invasive FNMTC occurred. Methods: We retrospectively reviewed all patients with thyroid carcinoma admitted to Peking Union Medical College Hospital from January 2009 to July 2013 in the database. Of all 2000 cases, 55 met the inclusive criteria for FNMTC and were studied. There are two different grouping methods. The first is that all samples were allocated to families with three or more first-degree relatives affected (FNMTC-3 group) and families with only two affected first-degree relatives (FNMTC-2 group). The second is that all patients were divided into families with three or more affected first-degree relatives over two generations (FNMTC-3-2 group) and the other families. We compared the clinicopathologic features such as sex, age, tumor size, multifocality, location, complications by thyroiditis, complications by benign thyroid nodules, surgical procedure, capsule invasion, histological type, lymph node metastases, tumor node metastasis stage, and BRAF mutation between FNMTC-2 group and FNMTC-3 group. We also made the same comparison between FNMTC-3-2 group and other families. Results: No pronounced differences in clinicopathological features were present between FNMTC-2 group and FNMTC-3 group. The proportion of FNMTC-3-2 group aged 〈45 years was significantly higher than that in the other families (58.8% vs. 26.3%, P = 0.021). A similar difference was found in the proportion of lymph node metastasis (64.7% vs. 34.2%, P = 0.035). Conclusions: FNMTC-3-2 is more invasive than the other families. Early screening and positive treatment for members of these families are recommended. 展开更多
关键词 Clinical Pathology familial nonmedullary thyroid carcinoma
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家族性甲状腺非髓样癌的临床特征分析 被引量:6
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作者 高健 于洋 +6 位作者 李小龙 赵静 赵翠 赵敬柱 刘友忠 李亦工 高明 《中华肿瘤杂志》 CAS CSCD 北大核心 2014年第3期202-206,共5页
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... 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。 展开更多
关键词 甲状腺肿瘤 甲状腺非髓样癌 家族性 乳头状癌 遗传 临床特征
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遗传性甲状腺癌 被引量:7
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作者 陈曦 李宏为 《中国实用外科杂志》 CSCD 北大核心 2010年第10期828-830,共3页
遗传性甲状腺癌分两大类:家族性非髓样甲状腺癌(FNMTC)和遗传性甲状腺髓样癌(HMTC)。前者可单独发生或仅作为某一家族性肿瘤综合征的少见部分,后者则可分为多发性内分泌肿瘤2a型(MEN-2a)、多发性内分泌肿瘤2b型(MEN-2b)和家族性甲状腺... 遗传性甲状腺癌分两大类:家族性非髓样甲状腺癌(FNMTC)和遗传性甲状腺髓样癌(HMTC)。前者可单独发生或仅作为某一家族性肿瘤综合征的少见部分,后者则可分为多发性内分泌肿瘤2a型(MEN-2a)、多发性内分泌肿瘤2b型(MEN-2b)和家族性甲状腺髓样癌(FMTC)。FNMTC由抑癌基因突变引起,外显率不高,但多为多发性病灶,较散发病例更具侵袭性,局部浸润常见,易早期转移,复发率高。而HMTC是一种由原癌基因RET突变所致的高外显率的单基因显性遗传性疾病。早期诊断很重要,通过基因测序可发现无症状病例,并依照基因突变类型,决定预防性全甲状腺切除手术时机。 展开更多
关键词 家族性非髓样甲状腺癌 遗传性甲状腺髓样癌 基因诊断
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遗传性甲状腺癌诊治要点 被引量:4
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作者 陈曦 《中国实用外科杂志》 CAS CSCD 北大核心 2022年第6期712-715,共4页
随着甲状腺癌的高发,遗传性甲状腺癌也愈受关注。主要分为遗传性髓样癌和家族性非髓样甲状腺癌。前者主要由RET基因胚系突变所致,是多发性内分泌肿瘤2型的重要组成部分。根据突变位点不同,在婴幼儿期预防性全甲状腺切除可避免甲状腺髓... 随着甲状腺癌的高发,遗传性甲状腺癌也愈受关注。主要分为遗传性髓样癌和家族性非髓样甲状腺癌。前者主要由RET基因胚系突变所致,是多发性内分泌肿瘤2型的重要组成部分。根据突变位点不同,在婴幼儿期预防性全甲状腺切除可避免甲状腺髓样癌的发生,但需平衡疾病进展和手术并发症风险之间的利弊。密切监测血清降钙素水平,发现升高不应再推迟手术,基本术式为全甲状腺切除加双侧中央区清扫术,必要时行治疗性或预防性侧颈淋巴结清扫。后者是指在没有易感环境因素的情况下,家族一级亲属间有≥2例的非髓样甲状腺癌病人,其中少数以综合征形式存在,有已知的基因改变,大多数是非综合征形式的分化型甲状腺癌,可能是单基因或多基因的低外显率的常染色体显像遗传,多数报道较散发病例更具侵袭性,需要更积极的治疗。 展开更多
关键词 遗传性甲状腺髓样癌 多发性内分泌肿瘤2型 预防性全甲状腺切除 家族性非髓样甲状腺癌
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家族性甲状腺非髓样癌临床特点
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作者 刘森源 邱新光 《国际外科学杂志》 2015年第5期334-337,共4页
家族性甲状腺非髓样癌指家族一级亲属中有2人或2人以上患有甲状腺非髓样癌,并排除其它已知的家族遗传性肿瘤综合征(如Gardner综合征、Cowden病、Carney联合体、Werner综合征等)和共同的甲状腺癌危险因素暴露史,危险因素包括颈部辐射... 家族性甲状腺非髓样癌指家族一级亲属中有2人或2人以上患有甲状腺非髓样癌,并排除其它已知的家族遗传性肿瘤综合征(如Gardner综合征、Cowden病、Carney联合体、Werner综合征等)和共同的甲状腺癌危险因素暴露史,危险因素包括颈部辐射和碘缺乏等.虽然一些研究已经发现了几个遗传异质性较高的易感基因位点,但是家族性甲状腺非髓样癌在基因水平的发病机制尚未完全明确.大多数研究表明,与散发病例相比,家族性甲状腺非髓样癌具有发病年龄早、多病灶、易腺体外侵犯、淋巴结转移率高、复发率高、生存率低等特点. 展开更多
关键词 甲状腺非髓样癌 家族性 甲状腺乳头状癌 综合征 遗传 危险因素
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家族性非髓样甲状腺癌临床分析
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作者 董立国 蒲永东 +1 位作者 何建苗 徐石岩 《中国优生与遗传杂志》 2008年第10期118-119,共2页
目的探讨家族性非髓样甲状腺癌的遗传学、诊断及诊疗,为其早期诊断及正确治疗提供依据。方法总结家族性非髓样甲状腺癌患者的临床资料,对其遗传学、早期诊断及治疗等方面进行探讨。结果家族性非髓样甲状腺癌起源于甲状腺滤泡上皮细胞,... 目的探讨家族性非髓样甲状腺癌的遗传学、诊断及诊疗,为其早期诊断及正确治疗提供依据。方法总结家族性非髓样甲状腺癌患者的临床资料,对其遗传学、早期诊断及治疗等方面进行探讨。结果家族性非髓样甲状腺癌起源于甲状腺滤泡上皮细胞,它单独发生或作为家族性肿瘤综合征发生。多发性病灶和同时伴发腺瘤性甲状腺肿是其特征性临床表现,并且多发生在年轻人;合理的手术治疗可以取得满意的疗效。结论家族性非髓样甲状腺癌是一种遗传性疾病,早期诊断、合理的手术治疗可以取得满意的疗效。患者家族成员应长期随访。 展开更多
关键词 家族性非髓样甲状腺癌 遗传学 诊断 治疗
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