Abstract
Duodenal cancer is a leading cause of death in patients with familial adenomatous polyposis (FAP). In patients with Spigelman’s classification (SC) stage IV duodenal polyposis (DP), careful endoscopic surveillance by specialists or surgical intervention is mandatory. We herein report the surgical and pathological outcomes of FAP patients with SC stage duodenal polyposis undergoing pancreas-sparing total duodenectomy (PSTD), which has been rarely reported but seems optimal in such patients. PSTD and distal gastrectomy with Billroth-I type reconstruction in ten consecutive FAP patients with SC stage IV DP are reported. The median duration of surgery was 396 min (range 314–571 min) and the median estimated blood loss was 480 mL (range 100–975 mL). Significant postoperative complications included wound infection in 1 patient, pancreatic fistula [International Study Group on Pancreatic Fistula definition (ISGPF) grade B] in 4 patients. Histopathologic examinations revealed a well-differentiated carcinoma in situ in 3 patients and others were all adenomas. Over a median follow-up period of 15 months (range 9–29 months), 1 patient developed a stomal ulcer which improved with medical treatment. There were no patients with a body weight loss of ≥10 % relative to the preoperative body weight. No recurrence were experienced during the follow up period. Patients were free from postoperative diabetes mellitus. PSTD is a feasible and acceptable procedure in FAP patients with SC stage IV DP, in terms of surgical, pathological and clinical outcome. However, accumulation of the patients and long-term follow up study is necessary.
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Arvanitis ML, Jagelman DG, Fazio VW et al (1990) Mortality in patients with familial adenomatous polyposis. Dis Colon Rectum 33:639–642
Belchetz LA, Berk T, Bapat BV et al (1996) Changing causes of mortality in patients with familial adenomatous polyposis. Dis Colon Rectum 39:384–387
Spigelman AD, Talbot IC, Penna C, Nugent KP, Phillips RK, Costello C et al (1994) Evidence for adenoma–carcinoma sequence in the duodenum of patients with familial adenomatous polyposis. The Leeds Castle Polyposis Group (Upper Gastrointestinal Committee). J Clin Pathol 47:709–710
Björk J, Akerbrant H, Iselius L et al (2001) Periampullary adenomas and adenocarcinomas in familial adenomatous polyposis: cumulative risks and APC gene mutations. Gastroenterology 121:1127–1135
Bülow S, Björk J, Christensen IJ et al (2004) Duodenal adenomatosis in familial adenomatous polyposis. Gut 53:381–386
de Vos tot Nederveen Cappel WH, Järvinen HJ, Björk J et al (2003) Worldwide survey among polyposis registries of surgical management of severe duodenal adenomatosis in familial adenomatous polyposis. Br J Surg 90:705–710
Spigelman AD, Williams CB, Talbot IC et al (1989) Upper gastrointestinal cancer in patients with familial adenomatous polyposis. Lancet 2:783–785
Groves CJ, Saunders BP, Spigelman AD et al (2002) Duodenal cancer in patients with familial adenomatous polyposis (FAP): results of a 10 year prospective study. Gut 50:636–641
Skipworth JR, Morkane C, Raptis DA et al (2011) Pancreaticoduodenectomy for advanced duodenal and ampullary adenomatosis in familial adenomatous polyposis. HPB (Oxford) 13:342–349
Gallagher MC, Shankar A, Groves CJ et al (2004) Pylorus-preserving pancreaticoduodenectomy for advanced duodenal disease in familial adenomatous polyposis. Br J Surg 91:1157–1164
Chung RS, Church JM, vanStolk R (1995) Pancreas-sparing duodenectomy: indications, surgical technique, and results. Surgery 117:254–259
van Heumen BW, Nieuwenhuis MH, van Goor H et al (2012) Surgical management for advanced duodenal adenomatosis and duodenal cancer in Dutch patients with familial adenomatous polyposis: a nationwide retrospective cohort study. Surgery 151:681–690
Ishikawa H, Mutoh M, Iwama T et al (2016) Endoscopic management of familial adenomatous polyposis in patioents refusing colectomy. Endoscopy 48:51–55
Bassi Claudio, Dervenis Christos, Butturini Giovanni et al (2005) Postoperative pancreatic fistula: an international study group (ISGPF) definition. Surgery 138:8–13
Alwmark A, Andersson A, Lasson A (1980) Primary carcinoma of the duodenum. Ann Surg 191:13–18
Iwama T, Mishima Y, Utsunomiya J (1993) The impact of familial adenomatous polyposis on the tumorigenesis and mortality at the several organs. Its rational treatment. Ann Surg 217:101–108
Yamaguchi T, Ishida H, Ueno H et al (2016) Upper gastrointestinal tumours in Japanese familial adenomatous polyposis patients. Jpn J Clin Oncol 46:310–315
Balmforth DC, Phillips RK, Clark SK (2012) Advanced duodenal disease in familial adenomatous polyposis: how frequently should patients be followed up after successful therapy? Fam Cancer 11:553–557
Samadder NJ, Neklason DW, Boucher KM et al (2016) Effect of Sulindac and Erlotinib on duodenal neoplasia in familial adenomatous polyposis: a randomized clinical trial. JAMA 315:1266–1275
von Roon AC, Will OC, Man RF et al (2011) Mucosectomy with handsewn anastomosis reduces the risk of adenoma formation in the anorectal segment after restorative proctocolectomy for familial adenomatous polyposis. Ann Surg 253:314–317
van Duijvendijk P, Vasen HF, Bertario L et al (1999) Cumulative risk of developing polyps or malignancy at the ileal pouch-anal anastomosis in patients with familial adenomatous polyposis. J Gastrointest Surg 3:325–330
Penninga L, Svendsen LB (2011) Pancreas-preserving total duodenectomy: a 10-year experience. J Hepatobiliary Pancreat Sci 18:717–723
Zuidema MF, Dekker W (1989) A patient with a metastasizing jejunal carcinoma 17 years after colectomy for familial polyposis coli. Neth J Med 34:317–321
Murakami Y, Uemura K, Sasaki M (2005) Duodenal cancer arising from the remaining duodenum after pylorus-preserving pancreatoduodenectomy for ampullary cancer in familial adenomatous polyposis. J Gastrointest Surg 9:389–392
Jagelman DG, DeCosseJJ Bussey HJ (1988) Upper gastrointestinal cancer in familial adenomatous polyposis. Lancet 21:1149–1151
Ishida H, Kumamoto K, Amano K et al (2013) Identification of APC gene mutations in jejunal carcinomas from a patient with familial adenomatous polyposis. Jpn J Clin Oncol 43:929–934
Ruys AT, Alderlieste YA, Gouma DJ et al (2010) Jejunal cancer in patients with familial adenomatous polyposis. Clin Gastroenterol Hepatol 8:731–733
Iwama T, Tamura K, Morita T et al (2004) A clinical overview of familial adenomatous polyposis derived from the database of the Polyposis Registry of Japan. Int J Clin Oncol 9:308–316
Matsumoto T, Esaki M, Yanaru-Fujisawa R et al (2008) Small-intestinal involvement in familial adenomatous polyposis: evaluation by double-balloon endoscopy and intraoperative enteroscopy. Gastrointest Endosc 68:911–919
Alderlieste YA, Bastiaansen BA, Mathus-Vliegen EM et al (2013) High rate of recurrent adenomatosis during endoscopic surveillance after duodenectomy in patients with familial adenomatous polyposis. Fam Cancer 12:699–706
de Castro SM, van Eijck CH, Rutten JP et al (2008) Pancreas-preserving total duodenectomy versus standard pancreatoduodenectomy for patients with familial adenomatous polyposis and polyps in the duodenum. Br J Surg 95:1380–1386
Burke CA, Beck GJ, Church JM et al (1999) The natural history of untreated duodenal and ampullary adenomas in patients with familial adenomatous polyposis followed in an endoscopic surveillance program. Gastrointest Endosc 49:358–364
Ma T, Jang EJ, Zukerberg LR et al (2014) Recurrences are common after endoscopic ampullectomy for adenoma in the familial adenomatous polyposis (FAP) syndrome. Surg Endosc 28:2349–2356
Irani S, Arai A, Ayub K et al (2009) Papillectomy for ampullary neoplasm: results of a single referral center over a 10-year period. Gastrointest Endosc 70:923–932
Catalano MF, Linder JD, Chak A et al (2004) Endoscopic management of adenoma of the major duodenal papilla. Gastrointest Endosc 59:225–232
Gluck N, Strul H, Rozner G et al (2015) Endoscopy and EUS are key for effective surveillance and management of duodenal adenomas in familial adenomatous polyposis. Gastrointest Endosc 81:960–966
Iwama T, Tomita H, Kawachi Y et al (1994) Indications for local excision of ampullary lesions associated with familial adenomatous polyposis. J Am Coll Surg 179:462–464
Camp ER, Hochwald SN, Liu C et al (2004) FAP with concurrent duodenal adenomatous polyposis and carcinoid tumor. Surg Oncol 87:187–190
July LV, Northcott KA, Yoshida EM et al (1999) Coexisting carcinoid tumors in familial adenomatous polyposis-associated upper intestinal adenomas. Am J Gastroenterol 94:1091–1094
Tsiotos GG, Sarr MG (1998) Pancreas-preserving total duodenectomy. Dig Surg 15:398–403
Sarmiento JM, Thompson GB, Nagorney DM et al (2002) Pancreas-sparing duodenectomy for duodenal polyposis. Arch Surg 137:557–563
Lundell L, Hyltander A, Liedman B (2002) Pancreas-sparing duodenectomy: technique and indications. Eur J Surg 168:74–77
Kalady MF, Clary BM, Tyler DS et al (2002) Pancreas-preserving duodenectomy in the management of duodenal familial adenomatous polyposis. J Gastrointest Surg 6:82–87
Imamura M, Komoto I, Doi R et al (2005) New pancreas-preserving total duodenectomy technique. World J Surg 29:203–207
Mackey R, Walsh RM, Chung R et al (2005) Pancreas-sparing duodenectomy is effective management for familial adenomatous polyposis. J Gastrointest Surg 9:1088–1093
Mullar MW, Dahmen R, Koninger J et al (2008) Is there an advantage in performing a pancreas-preserving total duodenectomy in duodenal adenomatosis? Am J Surg 195:741–748
Al-Sarireh B, Ghaneh P, Gardner-Thorpe J et al (2008) Complications and follow-up after pancreas-preserving total duodenectomy for duodenal polyps. Br J Surg 95:1506–1511
Benetatos N, Ammori MB, Ammori BJ (2011) Laparoscopic pancreas-preserving total duodenectomy for familial adenomatous polyposis. Surg Laparosc Endosc Percutan Tech 21:e332–e335
Stauffer JA, Adkisson CD, Riegert-Johnson DL et al (2012) Pancreas-sparing total duodenectomy for ampullary duodenal neoplasms. World J Surg 36:2461–2472
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Watanabe, Y., Ishida, H., Baba, H. et al. Pancreas-sparing total duodenectomy for Spigelman stage IV duodenal polyposis associated with familial adenomatous polyposis: experience of 10 cases at a single institution. Familial Cancer 16, 91–98 (2017). https://doi.org/10.1007/s10689-016-9932-2
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DOI: https://doi.org/10.1007/s10689-016-9932-2