High incidence of head and neck squamous cell carcinoma in patients with Fanconi Anemia
High incidence of head and neck squamous cell carcinoma in patients with Fanconi Anemia
复制标题
DOI：
10.1001/archotol.129.1.106
复制
发表时间：
2003-01-01
影响因子：
--
通讯作者：
Singh, B
中科院分区：
文献类型：
--
作者：
Kutler, DI;Auerbach, AD;Singh, B
背景资料：范可尼贫血（FA）是一种罕见的常染色体隐性遗传疾病，其特征是高度的基因组不稳定性和易患癌症。最近的证据表明，头和颈部鳞状细胞癌（HNSCC）的发病率可能会增加患者FA.Objective：以确定累积发病率，肿瘤分布，和结果HNSCC患者FA.Design and Setting：我们分析了来自国际范可尼贫血登记处，前瞻性收集数据库FA患者的754名受试者的数据。HNSCC的累积发病率和2年的总体，无复发和疾病特异性survival.Results：在国际范可尼贫血登记处的754例患者中，19例（3%）有HNSCC。与普通人群相比，HNSCC的发病率明显更高（标准化发病率比为500; 95%可信区间为300-781）（P
Background: Fanconi anemia (FA) is a rare autosomal recessive disorder characterized by a high degree of genomic instability and predisposition to cancer development. Recent evidence suggests that the incidence of head and neck squamous cell carcinoma (HNSCC) may be increased in patients with FA.Objective: To determine the cumulative incidence, tumor distribution, and outcome of HNSCC in patients with FA.Design and Setting: We analyzed data from 754 subjects from the International Fanconi Anemia Registry, a prospectively collected database of patients with FA.Main Outcome Measures: Cumulative incidence of HNSCC and 2-year overall, relapse-free and disease-specific survival.Results: Of the 754 patients in the International Fanconi Anemia Registry, 19 (3%) had HNSCC. This is a significantly higher incidence of HNSCC compared with that observed in the general population (standardized incidence ratio, 500; 95% confidence interval, 300-781) (P