RSS-Feed abonnieren
DOI: 10.1055/s-0029-1214719
© Georg Thieme Verlag KG Stuttgart · New York
Surveillance and treatment of duodenal adenomatosis in familial adenomatous polyposis
Publikationsverlauf
submitted 31 October 2008
accepted after revision 17 March 2009
Publikationsdatum:
16. Juni 2009 (online)
![](https://www.thieme-connect.de/media/endoscopy/200906/lookinside/thumbnails/10.1055-s-0029-1214719-1.jpg)
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.
References
- 1 Funkenstein O. Über polyposis intestinalis. Z Klin Med, Berlin. 1904; 55 236-248
- 2 Cabot R C. Case records of the Massachusetts General Hospital case 21 061. N Engl J Med. 1935; 212 363-368
- 3 Järvinen H J, Nyberg M, Peltokallio P. Upper gastrointestinal tract polyps in familial adenomatosis coli. Gut. 1983; 24 333-339
- 4 Heiskanen I, Kellokumpu I, Järvinen H. Management of duodenal adenomas in 98 patients with familial adenomatous polyposis. Endoscopy. 1999; 31 412-416
- 5 Bülow S, Björk J, Christensen I J. et al . Duodenal adenomatosis in familial adenomatous polyposis. Gut. 2004; 53 381-386
- 6 Vasen H FA, Myrhoj T, Mathus-Vliegen L. et al . Decision analysis in the management of duodenal adenomatosis in familial adenomatous polyposis. Gut. 1997; 40 716-719
- 7 Wallace M H, Phillips R KS. Upper gastrointestinal disease in patients with familial adenomatous polyposis. Br J Surg. 1998; 85 742-750
- 8 Björk J, Åkerbrandt H, Iselius L. et al . Periampullary adenomas and adenocarcinomas in familial adenomatous polyposis: Cumulative risks and APC gene mutations. Gastroenterology. 2001; 121 1127-1135
- 9 Jagelman D G, DeCosse J J, Bussey H J. Upper gastrointestinal cancer in familial adenomatous polyposis. Lancet. 1988; 1 1149-1151
- 10 Bülow S. Results of national registration of familial adenomatous polyposis. Gut. 2003; 52 742-746
- 11 Groves C J, Saunders B P, Spigelman A D, Phillips R KS. Duodenal cancer in patients with familial adenomatous polyposis (FAP): results of a 10 year prospective study. Gut. 2002; 50 636-641
- 12 Brosens L AA, Keller J J, Offerhaus G JA. et al . Prevention and management of duodenal polyps in familial adenomatous polyposis. Gut. 2005; 54 1034-1043
- 13 Spigelman A D, Williams C B, Talbot I C. et al . Upper intestinal cancer in patients with familial adenomatous polyposis. Lancet. 1989; ii 783-785
- 14 Whipple A O, Parsons W B, Mullins C R. Treatment of carcinoma of the ampulla of Vater. Ann Surg. 1935; 102 763
- 15 Grace P A, Pitt H A, Longmire W P. Pancreaticoduodenectomy with pylorus preservation for adenocarcinoma of the head of the pancreas. Br J Surg. 1986; 73 647
- 16 Bresalier R S, Sandler R S, Quan H. et al . Adenomatous Polyp Prevention on Vioxx (APPROVe) Trial Investigators. Cardiovascular events associated with rofecoxib in a colorectal adenoma chemoprevention trial. N Engl J Med. 2005; 352 1092-1102
- 17 Alarcon F J, Burke C A, Church J M, van Stolk R U. Familial adenomatous polyposis: efficacy of endoscopic and surgical treatment for advanced duodenal adenomas. Dis Colon Rectum. 1999; 42 1533-1536
- 18 Penna C, Bataille N, Balladur P. et al . Surgical treatment of severe duodenal polyposis in familial adenomatous polyposis. Br J Surg. 1998; 85 665-668
- 19 Balladur P, Penna C, Tiret E. et al . Pancreatco-duodenectomy for cancer and precancer in familial adenomatous polyposis. Int J Colorectal Dis. 1993; 8 151-153
- 20 Sarmiento J M, Thompson G B, Nagorney D M. et al . Pancreas-sparing duodenectomy for duodenal polyposis. Arch Surg. 2002; 137 557-563
- 21 de Vos tot Nederveen Cappel W H, Järvinen H J, Björk J. et al . Worldwide survey among polyposis registries of surgical management of severe duodenal adenomatosis in familial adenomatous polyposis. Br J Surg. 2003; 90 705-710
- 22 Gallagher M C, Shankar A, Groves C J. et al . Pylorus-preserving pancreaticoduodenectomy for advanced duodenal disease in familial adenomatous polyposis. Br J Surg. 2004; 91 1157-1164
- 23 Ruo L, Coit D G, Brennan M F. et al . Long-term follow-up of patients with familial adenomatous polyposis undergoing pancreaticoduodenal surgery. J Gastrointest Surg. 2002; 6 671-675
-
24 Lillemore K D, Cameron J L.
Postoperative results following pancreaticoduodenectomy. In: Zinner MJ, Schwartz SI, Ellis H, eds Maingot’s Abdominal operations, 10th edn. USA; Appleton & Lange, A Simon & Schuster Company 1997: 1991-1992
A. LepistöMD, PhD
Department of Surgery
Helsinki University Central Hospital
P.O. Box 340
Helsinki
00029 HUS
Finland
Fax: +358-9-74609
eMail: anna.lepisto@hus.fi