Background and Aims

The greatest known risk factor for duodenal cancer in familial adenomatous polyposis (FAP) is Spigelman stage (SS) IV duodenal polyposis. Endoscopic surveillance is recommended in FAP patients with SS 0 to IV, and prophylactic duodenectomy should be considered in SS IV. Cancer occurs in patients without SS IV polyposis. We assessed the relationship of SS and other factors with duodenal cancer in FAP.

Methods

We performed a case-control study on 18 FAP patients with duodenal cancer and 85 randomly selected FAP control subjects with similar age characteristics. Demographic, clinical, and endoscopic features were compared using univariate and logistic regression analyses to assess factors associated with duodenal cancer.

Results

Fifty-three percent of cases had no SS IV history. SS components positively associated with cancer included duodenal polyp size (77% vs 47%, P = .015), and high-grade dysplasia (HGD; 29% vs 6%, P = .003) but not polyp number or histology. In the papilla, the frequency of tubulovillous or villous histology (80% vs 22%, P < .001) and HGD (30% vs 4%, P = .010) was greater in cases than control subjects.

Conclusions

SS IV polyposis was absent in half of FAP patients with duodenal cancer. Only 2 of 4 SS components (large duodenal polyp size and HGD) were positively associated with duodenal cancer. Advanced pathology of the papilla appears to be an important feature. Revision of SS to emphasize these findings should be considered to better estimate cancer risk.

中文翻译：

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在家族性腺瘤性息肉病患者中，Spigelman IV期十二指肠息肉病并不早于大多数十二指肠癌病例

背景和目标

家族性腺瘤性息肉病（FAP）中十二指肠癌的最大已知危险因素是Spigelman分期（SS）IV十二指肠息肉。SS 0至IV的FAP患者建议进行内镜监测，SS IV应考虑预防性十二指肠切除术。没有SS IV息肉病的患者会发生癌症。我们评估了FAP中SS和其他因素与十二指肠癌的关系。

方法

我们对18名十二指肠癌FAP患者和85名年龄特征相似的FAP对照受试者进行了病例对照研究。使用单变量和逻辑回归分析比较人口统计学，临床和内镜特征，以评估与十二指肠癌相关的因素。

结果

53％的病例没有SS IV病史。与癌症正相关的SS成分包括十二指肠息肉大小（77％vs 47％，P  = .015）和高度不典型增生（HGD； 29％vs 6％，P  = 0.003），但息肉数目或组织学则不包括在内。在乳头中，与对照组相比，病例中的肾小管或绒毛组织学频率（80％vs 22％，P  <.001）和HGD（30％vs 4％，P  = .010）更大。

结论

FAP十二指肠癌患者中不存在SS IV息肉病。4种SS成分中只有2种（十二指肠息肉大和HGD大）与十二指肠癌呈正相关。乳头状的高级病理似乎是一个重要特征。应该考虑修订SS以强调这些发现，以更好地估计癌症风险。