Familial vs sporadic papillary thyroid carcinoma: a matched-case comparative study showing similar clinical/prognostic behaviour
Familial vs sporadic papillary thyroid carcinoma: a matched-case comparative study showing similar clinical/prognostic behaviour
复制标题
DOI：
10.1530/eje-13-0865
复制
发表时间：
2014-02-01
影响因子：
5.8
通讯作者：
Cavaco, Branca M.
中科院分区：
文献类型：
--
作者：
Pinto, Antonio E.;Silva, Giovani L.;Cavaco, Branca M.
目的：家族性非髓样甲状腺癌已被认为是一种侵袭性的临床实体。本研究的目的是探讨家族性与散发性甲状腺乳头状癌(PTC)的潜在特征以及生物学和临床侵袭性。设计：配对病例对照研究。方法：对107例家族性PTC患者和两个亚组，一个有三个或三个以上受累因素的患者(n=32)和另一个仅包括指标病例的亚组(n=61)与散发性PTC患者(n=107)进行比较，按年龄、性别、pTNM疾病范围和大约随访时间进行匹配。对组织学变异、甲状腺外侵犯、血管侵犯、肿瘤多灶性和双侧生长进行评估。用DNA流式细胞仪分析可用样本中的倍体类型。按Kaplan-Meier(K-M)法估计无病生存(DFS)和总生存(OS)。结果：家族性PTC无1例死亡(中位数72个月)，散发性PTC 5例(4.7%)(中位数90个月)。家族性甲状腺癌的多灶性显著高于散发性甲状腺癌(P=0.035)，在有3个或3个以上受累因素的家族性甲状腺癌队列中，肿瘤有更频繁地向甲状腺外扩散的趋势(P=0.054)。DNA倍体状态差异无统计学意义。K-M分析显示两种疾病在DFS或OS方面没有显著差异。结论：家族性PTC与散发性PTC相比，似乎具有相似的临床/预后行为。
Objective: Familial non-medullary thyroid cancer has been proposed as an aggressive clinical entity. Our aim in this study is to investigate potential distinguishing features as well as the biological and clinical aggressiveness of familial vs sporadic papillary thyroid carcinoma (PTC). We assessed clinicopathological characteristics, outcome measures and DNA ploidy.Design: A matched-case comparative study.Methods: A series of patients with familial PTC (n=107) and two subgroups, one with three or more affected elements (n=32) and another including index cases only (n=61), were compared with patients with sporadic PTC (n=107), matched by age, gender, pTNM disease extension and approximate follow-up duration. Histological variant, extrathyroidal extension, vascular invasion, tumour multifocality and bilateral growth were evaluated. Ploidy pattern was analysed in available samples by DNA flow cytometry. The probabilities of disease-free survival (DFS) and overall survival (OS) were estimated according to the Kaplan-Meier (K-M) method.Results: No patient with familial PTC died of disease during follow-up (median, 72 months), contrarily to five patients (4.7%) (P=0.06) with sporadic PTC (median, 90 months). There was a significantly higher tumour multifocality in familial PTC (index cases subgroup) vs sporadic PTC (P=0.035), and a trend, in the familial PTC cohort with three or more affected elements, to show extrathyroidal extension (P=0.054) more frequently. No difference was observed in DNA ploidy status. The K-M analyses showed no significant differences between both entities in relation to DFS or OS.Conclusion: Apart from multifocality, familial PTC appears to have similar clinical/prognostic behaviour when compared with sporadic forms of the disease.