Risk factors associated with earlier age of onset in familial pancreatic carcinoma
Risk factors associated with earlier age of onset in familial pancreatic carcinoma
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DOI：
10.1002/cncr.20700
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发表时间：
2004-12-15
期刊：
影响因子：
6.2
通讯作者：
Gibbs, JF
中科院分区：
文献类型：
--
作者：
James, TA;Sheldon, DG;Gibbs, JF
背景资料。据估计，所有胰腺癌中有5-10%与遗传有关。本研究的目的是描述家族性胰腺癌的临床和病理特征，并确定家族性和散发性胰腺癌人群在人口学、危险因素和预后方面的潜在差异。方法：对诊断为胰腺癌的患者进行回顾性分析，以确定有相关家族倾向的患者。对家族性亚组进行了人口统计学分析和临床特征和治疗结果的评估，并将结果与非家族性或散发性人群的观察结果进行了比较。结果：826名患者中有30名(3.6%)患有家族性胰腺癌。在家族性队列和散发性队列中，基线人口学、可切除性和转移情况相似。家族性队列的平均发病年龄略低(57.6岁，而散发性队列为61岁)。然而，家族性人群在50岁时确诊的患者比例明显高于散发性人群(36.7%vs.18.3%；P=0.017)。阳性吸烟史更常与家族性胰腺癌相关(87%对66%；P=0.06)。家族性胰腺癌患者的中位生存期为7个月，散发性胰腺癌患者的中位生存期为6个月。结论家族性胰腺癌患者的年龄较非家族性胰腺癌患者早。在有遗传倾向的患者中，吸烟可能在胰腺癌的风险或促进中发挥重要作用。(C)2004年美国癌症协会。
BACKGROUND. An estimated 5-10% of all pancreatic adenocarcinomas have a hereditary association. The objective of the current study was to characterize the clinical and pathologic features of familial pancreatic carcinoma and to determine potential differences in demographics, risk factors, and outcomes between familial and sporadic pancreatic carcinoma populations.METHODS. A retrospective review was performed to identify patients diagnosed with pancreatic carcinoma who had an associated familial disposition. Demographic analyses and assessment of clinical features and treatment outcomes were performed for the familial subgroup, and the results were compared with observations made in the nonfamilial, or 'sporadic', population.RESULTS. Thirty of 826 patients (3.6%) had familial pancreatic carcinoma. Baseline demographics, resectability, and metastases were similar in both the familial cohort and the sporadic cohort. The mean age of onset was slightly lower in the familial cohort (57.6 years, compared with 61 years in the sporadic cohort). However, the familial population had a significantly greater proportion of patients who were diagnosed at age < 50 years compared with the sporadic population (36.7% vs. 18.3%; P = 0.017). A positive smoking history was more commonly associated with familial pancreatic carcinoma (87% vs. 66%; P = 0.06). The overall median survival durations were 7 months and 6 months for the familial group and the sporadic group, respectively.CONCLUSIONS. Patients with familial pancreatic carcinoma present at an earlier age compared with their counterparts who have nonfamilial disease. Smoking may play a significant role in the risk or promotion of pancreatic carcinoma in patients with an inherited predisposition. (C) 2004 American Cancer Society.