Galle T S, Juel K, Bülow S
The Danish Polyposis Register, Hvidovre Hospital.
Scand J Gastroenterol. 1999 Aug;34(8):808-12. doi: 10.1080/003655299750025741.
The prognosis in familial adenomatous polyposis (FAP) has improved over the past decades owing to a reduction in the prevalence of colorectal cancer, resulting from effective early screening. During the same period several polyposis registers have recorded an increasing number of deaths due to duodenal/periampullary cancer and desmoid tumours. The aim of this study was to examine the causes of death with special emphasis on duodenal/periampullary cancer.
The material consisted of 328 patients (144 females and 184 males) registered from I January 1943 to 31 December 1992 in the Danish Polyposis Register. The standard mortality rate (SMR) was calculated for known major causes of death, using the entire Danish population as background population. The attributable risk was also calculated for selected death causes.
One hundred and thirty-three patients had died, SMR being 4.98 (95% confidence limits, 4.17-5.90). There were significantly lower SMRs in the call-up group than in the proband group. The late cohort (1943-1992) had lower SMRs than the early group (1889-1942). SMR was significantly increased for death due to colorectal cancer (145), duodenal cancer (214), and ovarian cancer (30). No deaths due to desmoids were observed in the examination period. The attributable risk for colorectal cancer was 29% and for duodenal cancer only 0.6%.
Colorectal cancer is the most frequent cause of death in polyposis patients, followed by duodenal/periampullary cancer, but the latter is still a rare cause of death in FAP.
在过去几十年中，由于有效的早期筛查降低了结直肠癌的患病率，家族性腺瘤性息肉病（FAP）的预后有所改善。在此期间，多个息肉病登记处记录了十二指肠/壶腹周围癌和硬纤维瘤导致的死亡人数不断增加。本研究的目的是检查死亡原因，特别关注十二指肠/壶腹周围癌。
研究材料包括1943年1月1日至1992年12月31日在丹麦息肉病登记处登记的328例患者（144例女性和184例男性）。以整个丹麦人口为背景人群，计算已知主要死亡原因的标准化死亡率（SMR）。还计算了选定死亡原因的归因风险。
133例患者死亡，SMR为4.98（95%置信区间，4.17 - 5.90）。征召组的SMR显著低于先证者组。晚期队列（1943 - 1992年）的SMR低于早期队列（1889 - 1942年）。因结直肠癌（145）、十二指肠癌（214）和卵巢癌（30）死亡的SMR显著升高。在检查期间未观察到因硬纤维瘤导致的死亡。结直肠癌的归因风险为29%，十二指肠癌仅为0.6%。
结直肠癌是息肉病患者最常见的死亡原因,其次是十二指肠/壶腹周围癌，但后者在FAP中仍然是罕见的死亡原因。
