Features of Duodenal Cancer in Patients With Familial Adenomatous Polyposis
Features of Duodenal Cancer in Patients With Familial Adenomatous Polyposis
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DOI：
10.1016/j.cgh.2009.02.028
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发表时间：
2009-06-01
影响因子：
12.6
通讯作者：
Clark, Susan K.
中科院分区：
文献类型：
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作者：
Latchford, Andrew R.;Neale, Kay F.;Clark, Susan K.
背景和目标：大多数家族性腺瘤性息肉病（FAP）患者发生十二指肠腺瘤;十二指肠癌是该患者组死亡的主要原因。我们回顾了FAP患者的十二指肠癌病例，以确定决定长期癌症风险的因素。方法：从注册数据库检索中确定了20例FAP患者（12例配偶）。对来自登记处和医疗记录以及内窥镜和组织病理学报告的数据进行了评价。结果：11例壶腹癌，9例十二指肠癌。癌症诊断的中位年龄为53岁。17例患者死亡（死亡时的中位年龄为57岁;诊断后的中位生存期为11个月）; 14例患者的死亡原因为转移性或十二指肠/壶腹癌。15例患者出现复发（包括监测期间的3例间期癌症）。2例在监测时诊断，3例在手术期间诊断，用于晚期良性疾病的内窥镜特征。与壶腹癌相比，十二指肠癌的平均结肠息肉计数显著较低（496 +/- 282 vs 1322 +/- 735; P = 0.025）;这种癌症似乎有家族聚集性。当内窥镜数据可用时（n = 11/20），所有壶腹癌均来自大于1 cm的壶腹。Spigelman分期不能预测壶腹癌的风险，但可以预测十二指肠癌（十二指肠癌的中位2期与4期）。结论：FAP患者一旦发生癌变，预后差，应将预防作为主要目标。监测间隔应反映Spigelman分期和壶腹疾病。
Background & Aims: most patients with familial adenomatous polyposis (FAP) develop duodenal adenomas; duodenal cancer is a major cause of mortality in this patient group. We reviewed cases of duodenal cancer in patients with FAP to identify factors that determine long-term cancer risk. Methods: Twenty FAP patients (12 mate) were identified from a registry database search. Data from registry and medical notes and endoscopic and histopathologic reports were evaluated. Results: Of the cancers that developed in these patients, 11 were ampullary and 9 were duodenal. The median age at cancer diagnosis was 53 years. Seventeen patients died (median age at death, 57 y; median survival from diagnosis, 11 mo); the cause of death was metastatic or duodenal/ampullary cancer in 14 patients. Fifteen patients presented symptomatically (including 3 interval cancers while on surveillance). Two were diagnosed at surveillance and 3 were diagnosed during surgery performed for endoscopic features of advanced benign disease. Duodenal cancers were associated with a significantly lower mean colonic polyp count than ampullary cancers (496 +/- 282 vs 1322 +/- 735; P = .025); there appeared to be familial clustering of this cancer. When endoscopic data were available (n = 11 of 20), all ampullary cancers arose from ampullas greater than 1 cm. The Spigelman stage did not predict risk of ampullary cancer but did predict duodenal cancer (median stage 2 vs stage 4 for duodenal cancer). Conclusions: Once cancer arises in patients with FAP, prognosis is poor, so cancer prevention should be the main goal. Surveillance intervals should reflect both Spigelman staging and ampullary disease.