Department of Pathology, Sol Goldman Pancreatic Cancer Research Center at Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins School of Medicine, Baltimore, MD 21231, USA.
J Natl Cancer Inst. 2010 Jan 20;102(2):119-26. doi: 10.1093/jnci/djp466. Epub 2010 Jan 12.
Young-onset cancer is a hallmark of many familial cancer syndromes, yet the implications of young-onset disease in predicting risk of pancreatic cancer among familial pancreatic cancer (FPC) kindred members remain unclear.
To understand the relationship between age at onset of pancreatic cancer and risk of pancreatic cancer in kindred members, we compared the observed incidence of pancreatic cancer in 9040 individuals from 1718 kindreds enrolled in the National Familial Pancreas Tumor Registry with that observed in the general US population (Surveillance, Epidemiology, and End Results). Standardized incidence ratios (SIRs) were calculated for data stratified by familial vs sporadic cancer kindred membership, number of affected relatives, youngest age of onset among relatives, and smoking status. Competing risk survival analyses were performed to examine the risk of pancreatic cancer and risk of death from other causes according to youngest age of onset of pancreatic cancer in the family and the number of affected relatives.
Risk of pancreatic cancer was elevated in both FPC kindred members (SIR = 6.79, 95% confidence interval [CI] = 4.54 to 9.75, P < .001) and sporadic pancreatic cancer (SPC) kindred members (SIR = 2.41, 95% CI = 1.04 to 4.74, P = .04) compared with the general population. The presence of a young-onset patient (<50 years) in the family did not alter the risk for SPC kindred members (SIR = 2.74, 95% CI = 0.05 to 15.30, P = .59) compared with those without a young-onset case in the kindred (SIR = 2.36, 95% CI = 0.95 to 4.88, P = .06). However, risk was higher among members of FPC kindreds with a young-onset case in the kindred (SIR = 9.31, 95% CI = 3.42 to 20.28, P < .001) than those without a young-onset case in the kindred (SIR = 6.34, 95% CI = 4.02 to 9.51, P < .001). Competing risk survival analyses indicated that the lifetime risk of pancreatic cancer in FPC kindreds increased with decreasing age of onset in the kindred (hazard ratio = 1.55, 95% CI = 1.19 to 2.03 per year). However, youngest age of onset for pancreatic cancer in the kindred did not affect the risk among SPC kindred members.
Individuals with a family history of pancreatic cancer are at a statistically significantly increased risk of developing pancreatic cancer. Having a member of the family with a young-onset pancreatic cancer confers an added risk in FPC kindreds.
许多家族性癌症综合征的标志是年轻发病的癌症，但年轻发病的疾病在预测家族性胰腺肿瘤（FPC）家族成员中患胰腺癌的风险方面的意义仍不清楚。
为了了解胰腺癌发病年龄与家族成员中胰腺癌风险之间的关系，我们将美国国家家族性胰腺肿瘤登记处登记的 1718 个家族中的 9040 名个体的胰腺癌实际发病率与普通美国人群（监测、流行病学和最终结果）的观察发病率进行了比较。按家族性或散发性癌症家族成员、受累亲属数量、亲属中最早发病年龄以及吸烟状况对数据进行分层，计算标准化发病比（SIR）。进行竞争风险生存分析，以根据家族中胰腺癌的最早发病年龄和受累亲属数量，检查患胰腺癌的风险和因其他原因死亡的风险。
FPC 家族成员（SIR = 6.79，95%置信区间[CI] = 4.54 至 9.75，P <.001）和散发性胰腺癌（SPC）家族成员（SIR = 2.41，95%CI = 1.04 至 4.74，P =.04）患胰腺癌的风险均高于普通人群。家族中有年轻发病患者（<50 岁）并不会改变 SPC 家族成员的风险（SIR = 2.74，95%CI = 0.05 至 15.30，P =.59）与家族中无年轻发病病例的患者相比（SIR = 2.36，95%CI = 0.95 至 4.88，P =.06）。然而，在家族中有年轻发病病例的 FPC 家族成员中，风险更高（SIR = 9.31，95%CI = 3.42 至 20.28，P <.001）与家族中无年轻发病病例的患者相比（SIR = 6.34，95%CI = 4.02 至 9.51，P <.001）。竞争风险生存分析表明，FPC 家族中胰腺癌的终生发病风险随着家族中发病年龄的降低而增加（风险比= 1.55，95%CI = 1.19 至 2.03/年）。然而，家族中胰腺癌的最早发病年龄并不会影响 SPC 家族成员的风险。
有家族性胰腺癌病史的个体患胰腺癌的风险显著增加。家族中有年轻发病的胰腺癌患者会增加 FPC 家族的患病风险。
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