The Danish Polyposis Register, Hvidovre University Hospital, Copenhagen, Denmark.
Colorectal Dis. 2012 Aug;14(8):947-52. doi: 10.1111/j.1463-1318.2011.02844.x.
Duodenal adenomatosis in familial adenomatous polyposis results in a cancer risk that increases with age. Endoscopic surveillance has been recommended, but the effect has not yet been documented. The aim of this study was to present the results of long-term duodenal surveillance and to evaluate the risk of cancer development.
Follow up of patients in a previous study with gastroduodenoscopy in 1990-2010. Statistical analysis included the χ(2) test, actuarial method and Kaplan-Meier analysis.
Among 304 patients, 261 (86%) had more than one endoscopy. The median follow up was 14 (interquartile range, 9-17) years. The cumulative lifetime risk of duodenal adenomatosis was 88% (95% CI, 84-93), and of Spigelman stage IV was 35% (95% CI, 25-45). The Spigelman stage improved in 32 (12%) patients, remained unchanged in 88 (34%) and worsened in 116 (44%). Twenty (7%) patients had duodenal cancer at a median age of 56 (range, 44-82) years. The cumulative cancer incidence was 18% at 75 years of age (95% CI, 8-28) and increased with increasing Spigelman stage at the index endoscopy to 33% in Spigelman stage IV (P < 0.0001). The median overall survival was 6.4 years (95% CI, 1.7 to not estimated): 8 years after a screen-detected cancer vs 0.8 years (95% CI, 0.03-1.7) after a symptomatic cancer (P < 0.0001). The location of the mutation in the APC gene did not influence the risk of developing Spigelman stage IV (P = 0.46) or duodenal cancer (P = 0.83).
The risk of duodenal cancer in familial adenomatous polyposis is considerable, and regular surveillance and cancer prophylactic surgery result in a significantly improved prognosis.
家族性腺瘤性息肉病中的十二指肠腺瘤会导致癌症风险随年龄增长而增加。已建议进行内镜监测，但尚未记录其效果。本研究旨在介绍长期十二指肠监测的结果，并评估癌症发展的风险。
对 1990-2010 年进行胃十二指肠镜检查的先前研究中的患者进行随访。统计分析包括卡方检验、 actuarial 方法和 Kaplan-Meier 分析。
在 304 名患者中，261 名（86%）进行了多次内镜检查。中位随访时间为 14 年（四分位间距，9-17 年）。终生十二指肠腺瘤的累积风险为 88%（95%CI，84-93），Spigelman 分期 IV 为 35%（95%CI，25-45）。32 名（12%）患者的 Spigelman 分期改善，88 名（34%）患者不变，116 名（44%）患者恶化。20 名（7%）患者在中位年龄为 56 岁（范围，44-82 岁）时患有十二指肠癌。75 岁时的累积癌症发病率为 18%（95%CI，8-28），并且随着索引内镜时 Spigelman 分期的增加而增加至 Spigelman 分期 IV 时的 33%（P <0.0001）。总生存中位数为 6.4 年（95%CI，1.7 至未估计）：筛查发现的癌症后为 8 年，症状性癌症后为 0.8 年（95%CI，0.03-1.7）（P <0.0001）。APC 基因突变的位置不影响发展为 Spigelman 分期 IV（P = 0.46）或十二指肠癌（P = 0.83）的风险。
家族性腺瘤性息肉病中十二指肠癌的风险相当大，定期监测和癌症预防性手术可显著改善预后。
