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家族性腺瘤性息肉病中十二指肠腺瘤病的监测与治疗

Surveillance and treatment of duodenal adenomatosis in familial adenomatous polyposis.

作者信息

Lepistö A, Kiviluoto T, Halttunen J, Järvinen H J

机构信息

Department of Surgery, Helsinki University Central Hospital, Finland.

出版信息

Endoscopy. 2009 Jun;41(6):504-9. doi: 10.1055/s-0029-1214719. Epub 2009 Jun 16.

Abstract

BACKGROUND AND STUDY AIMS

Patients with familial adenomatous polyposis (FAP) are at increased risk for duodenal cancer whereas colorectal cancer is largely prevented by prophylactic colectomy. We analyzed the results of endoscopic surveillance and different treatment modalities of duodenal adenomatosis in patients with FAP.

PATIENTS AND METHODS

Data on endoscopies, histopathological examinations, and surgical therapies were collected from the medical histories of 129 patients with FAP. The cumulative incidences of duodenal adenomatosis and severe dysplasia and cancer were calculated using Kaplan-Meier analysis.

RESULTS

By the age of 60 years, the cumulative incidence was 80% for any adenomatosis and 23% for severe dysplasia or cancer. Duodenal cancer was observed in six patients (4.7%). Fifteen endoscopic excisions in 14 patients, and 19 open duodenotomies in 17 patients were carried out. Later, pancreaticoduodenectomy was undertaken in six (35.3%) of these 17 patients. Altogether, 12 patients (9.3%) underwent pancreaticoduodenectomy. Except for one patient, the indication for surgery was based on follow-up endoscopies, and none of these patients died of duodenal cancer. No postoperative deaths occurred. Seven patients (58.3%) had major complications, four (33.3%) of which were surgical.

CONCLUSIONS

The high incidence of severe dysplasia and cancer in duodenal polyps suggests that endoscopic surveillance is essential. Endoscopic polypectomies under sedation anesthesia have partly replaced open duodenotomies. High-risk patients with Spigelman IV adenomatosis or adenomas with persisting severe dysplasia should undergo surgery with pylorus-preserving pancreaticoduodenectomy before invasive cancer develops.

摘要

背景与研究目的

家族性腺瘤性息肉病(FAP)患者患十二指肠癌的风险增加,而预防性结肠切除术在很大程度上可预防结直肠癌。我们分析了FAP患者十二指肠腺瘤病的内镜监测结果及不同治疗方式。

患者与方法

从129例FAP患者的病历中收集内镜检查、组织病理学检查及手术治疗的数据。采用Kaplan-Meier分析计算十二指肠腺瘤病、重度异型增生及癌症的累积发病率。

结果

到60岁时,任何腺瘤病的累积发病率为80%,重度异型增生或癌症的累积发病率为23%。观察到6例患者(4.7%)发生十二指肠癌。对14例患者进行了15次内镜下切除术,对17例患者进行了19次开放性十二指肠切开术。后来,这17例患者中有6例(35.3%)接受了胰十二指肠切除术。共有12例患者(9.3%)接受了胰十二指肠切除术。除1例患者外,手术指征均基于随访内镜检查,且这些患者均未死于十二指肠癌。无术后死亡病例。7例患者(58.3%)发生严重并发症,其中4例(33.3%)为手术相关并发症。

结论

十二指肠息肉中重度异型增生和癌症的高发病率表明内镜监测至关重要。镇静麻醉下的内镜息肉切除术已部分取代开放性十二指肠切开术。患有Spigelman IV级腺瘤病或存在持续重度异型增生腺瘤的高危患者应在发生浸润性癌之前接受保留幽门的胰十二指肠切除术。

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