Abstract
Prophylactic colectomy is generally recommended for patients with familial adenomatous polyposis (FAP) who are inevitably affected with large bowel cancer. After prophylactic colectomy has been performed, gastrointestinal malignancy is the leading cause of death. Duodenal adenomas are found in patients with FAP and the adenoma-carcinoma sequence exists in the FAP duodenum, suggesting that treatment of duodenal polyps might be beneficial. Several methods of treatment for duodenal lesions in patients with FAP have been reported, but the current treatment options are not ideal. The nonsteroid anti-inflammatory drugs, sulindac and aspirin, are used for chemoprevention, while recently developed cyclooxygenase-2 inhibitors may be of some use in the future. Endoscopic polypectomy has been attempted for duodenal polyps and open surgical polypectomy has proven to be effective for selected patients. Photodynamic therapy and Argon plasma coagulation may be suitable to treat carpeted polyposis. New methods of duodenal resection, such as pancreas-preserving duodenectomy and pyloruspreserving pancreaticoduodenectomy, might be considered for severe duodenal polyposis; however, because prophylactic duodenal surgery has been considered too aggressive, surveillance duodenoscopy is usually performed to detect duodenal cancer at an early stage.
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Bussey HJR (1975) Familial polyposis coli. Johns Hopkins Baltimore
Nugent KP, Spigelman AD, Phillips RKS (1993) Life expectancy after colectomy and ileorectal anastomosis for familial adenomatous polyposis. Dis Colon Rectum 36:1059–1062
Jagelman DG, DeCosse JJ, Bussey HJR (1988) Upper gastrointestinal cancer in familial adenomatous polyposis. Lancet i:1149–1151
Iwama T, Mishima Y, Utsunomiya J (1993) The impact of familial adenomatous polyposis on the tumorigenesis and mortality at several organs. Its rational treatment. Ann Surg 217:101–108
Spigelman AD, Talbot IC, Penna C, Nugent KP, Phillips RKS, Costello C, DeCosse JJ (1994) Evidence for adenoma-carcinoma sequence in the duodenum of patients with familial adenomatous polyposis. J Clin Pathol 47:709–710
Hauser G (1895) Uber polyposis intestinalis adenomatosa and deren Beziehungen zur Krebsentwicklung. Deutsche Arch Klim Med 55:429–438
Funkenstein O (1904) Uber polyposis intestinalis. Z Klin Med Berl 55:236–248
Cabot RC (1935) Case records of the Massachusetts General Hospital: case no. 21061. N Engl J Med 212:263–267
Murphy ES, Mireles M, Beltran A (1962) Familial polyposis of the colon and gastric carcinoma: concurrent conditions in a 16 year old boy. J Am Med Assoc 179:1026–1028
Utsunomiya J, Maki T, Iwama T, Matsunaga Y, Ichkawa T, Shimomura T, Hamaguchi E, Aoki N (1974) Gastric lesion of familial polyposis coli. Cancer 34:745–754
Yao T, Iida M, Ohsato K, Watanabe H, Omae T (1977) Duodenal lesions in familial polyposis of the colon. Gastroenterology 73:1086–1092
Spigelman AD, Williams CB, Talbot IC, Domizio P, Phillips RKS (1989) Upper gastrointestinal cancer in patients with familial adenomatous polyposis. Lancet ii:783–785
Lillemoe K, Imbembo A (1980) Malignant neoplasms of the duodenum. Surg Gynecol Obstet 150:822–826
Jones TR, Nance FC (1977) Periampullary malignancy in Gardner’s syndrome. Ann Surg 185:565–573
Iida M, Yao T, Itoh H, Watanabe H, Matsui T, Iwashita A, Fujishima M (1989) Natural history of duodenal lesions in Japanese patients with familial adenomatosis coli (Gardner’s syndrome). Gastroenterology 96:1301–1306
Bulow S, Alm T, Fausa O, Hultcrantz R, Jarvinen H, Vasen H, DAF Project Group (1995) Duodenal adenomatosis in familial adenomatous polyposis. DAF Project Group. Int J Colorectal Dis 10:43–46
Offerhaus GJA, Giardiello FM, Krush AJ, Booker SV, Tersmette AC, Kelly NC, Hamilton SR (1992) The risk of upper gastrointestinal cancer in familial adenomatous polyposis. Gastroenterology 102:1980–1982
Tonelli F, Nardi F, Bechi P, Taddei G, Gozzo P, Romagnoli P (1985) Extracolonic polyps in familial polyposis coli and Gardner’s syndrome. Dis Colon Rectum 28:664–668
Bulow S, Lauritsen KB, Johansen A, Svendsen LB, Sondergaard JO (1985) Gastroduodenal polyps in familial polyposis coli. Dis Colon Rectum 28:90–93
Kurtz R, Sternberg SS, Miller HH, Decosse JJ (1987) Upper gastrointestinal neoplasia in familial polyposis. Dig Dis Sci 32:459–465
Burt RW, Berenson MM, Lee RG, Tolman KG, Freston JW, Gardner EJ (1984) Upper gastrointestinal polyps in Gardner’s syndrome. Gastroenterology 86:295–301
Alexander JR, Andrews JM, Buchi KN, Lee RG, Becker JM, Burt RW (1989) High prevalence of adenomatous polyps of the duodenal papilla in familial adenomatous polyposis. Dig Dis Sci 34:167–170
Sarre RG, Frost AG, Jagelman DG, Petras RE, Sivak MV, McGannon E (1987) Gastric and duodenal polyps in familial adenomatous polyposis: a prospective study of the nature and prevalence of upper gastrointestinal polyps. Gut 28:306–314
Ranzi T, Castagnone D, Velio P, Bianchi P, Polli EE (1981) Gastric and duodenal polyps in familial polyposis coli. Gut 22:363–367
Jarvinen H, Nyberg M, Peltokallio P (1983) Upper gastrointestinal tract polyps in familial adenomatosis coli. Gut 24:333–339
Sawada T, Muto T (1995) Familial adenomatous polyposis: should patients undergo surveillance of the upper gastrointestinal tract? Endoscopy 27:6–11
Marcello PW, Asbun HJ, Veidenheimer MC, Rossi RL, Robert PL, Fine SN, Coller JA, Murry JJ, Schoetz DJ (1996) Gastroduodenal polyps in familial adenomatous polyposis. Surg Endosc 10:418–424
Iida M, Aoyagi K, Fujishima Y, Matsumoto T, Hizawa K, Nakamura S (1996) Nonpolypoid adenomas of the duodenum in patients with familial adenomatous polyposis (Gardner’s syndrome). Gastrointest Endosc 44:305–308
Bertoni G, Sassatelli R, Nigrisoli E, Pennazio M, Tansini P, Arrigoni A, Ponz de Leon M, Rossini FP, Bedogni G (1996) High prevalence of adenomas and microadenomas of the duodenal papilla and periampullary region in patients with familial adenomatous polyposis. Eur J Gastroenterol Hepatol 8:1201–1206
Domizio P, Talbot IC, Spigelman AD, Williams CB, Phillips RKS (1990) Upper gastrointestinal pathology in familial adenomatous polyposis: results from a prospective study of 102 patients. J Clin Pathol 43:738–743
Spigelman AD, Phillips RKS (1994) The upper gastrointestinal tract. In: Phillips RKS, Spigelman AD, Thomson JPS (eds) Familial adenomatous polyposis and other polyposis syndromes. Edward Arnold, London, pp 106–127
Park JG, Park KJ, Ahn YO, Song IS, Choi KW, Moon HY, Choo SY, Kim JP (1992) Risk of gastric cancer among Korean familial adenomatous polyposis patients: report of three cases. Dis Colon Rectum 35:996–998
Talbot IC (1994) Pathology. In: Phillips RKS, Spigelman AD, Thomson JPS (eds) Familial adenomatous polyposis and other polyposis syndromes. Edward Arnold, London, pp 15–25
Debinski HS, Spigelman AD, Hatfield A, Williams CB, Phillips RKS (1995) Upper intestinal surveillance in familial adenomatous polyposis. Eur J Cancer 31a:1149–1153
Zwick A, Munir M, Ryan CK, Gian J, Burt RW, Leppert M, Spirio L, Chey WY (1997) Gastric adenocarcinoma and dysplasia in fundic gland polyps of a patients with attenuated adenomatous polyposis coli. Gastroenterology 113:659–663
Iida M, Yao T, Itoh H, Watanabe H, Matsui T, Iwashita A, Fujishima M (1988) Natural history of gastric adenomas in patients with familial adnomatosis coli/Gardner’s syndrome. Cancer 61:605–611
Spigelman AD, Scates DK, Venitt S, Phillips RKS (1991) DNA adducts, detected by32P-postlabelling, in the foregut of patients with familial adenomatous polyposis and in unaffected controls. Carcinogenesis 12:1727–1732
Scates DK, Spigelman AD, Phillips RKS, Venitt S (1992) DNA adducts detected by32P-postlabelling, in the intestine of rats given bile from patients with familial adenomatous polyposis and from unaffected controls. Carcinogenesis 13:731–735
Iida M, Yao T, Itoh H, Watanabe H, Kohrogi N, Shigematsu A, Iwashita A, Fujishima M (1985) Natural history of fundic gland polyposis in patients with familial adenomatosis coli/Gardner’s syndrome Gastroenterology 89:1021–1025
Nugent KP, Spigelman AD, Williams CB, Talbot IC, Phillips RKS (1994) Surveillance of duodenal polyps in familial adenomatous polyposis: progress report. J R Soc Med 87:704–706
Kashiwagi H, Spigelman AD, Debinski HS, Talbot IC, Phillips RKS (1994) Surveillance of ampullary adenomas in familial adenomatous polyposis. Lancet 344:1582
Noda Y, Watanabe H, Iida M, Narisawa R, Kurosaki I, Iwafuchi M, Satoh M, Ajioka Y (1992) Histologic follow-up of ampullary adenomas in patients with familial adenomatosis coli. Cancer 70:1847–1856
Nakatsubo N, Kashiwagi H, Okumura M, Kamoshida T, Takahashi A, Spigelman AD (1998) Malignant change in a duodenal adenoma in familial adenomatous polyposis. Report of a case. Am J Gastroenterol 96:1566–1568
Miyaki M, Seki M, Okamoto M, Yamanaka A, Maeda Y, Tanaka K, Kikuchi R, Iwama T, Ikeuchi T, Tonomura A, Nakamura Y, White R, Miki Y, Utsunomiya J, Mori T (1990) Genetic changes and histopathological types in colorectal tumors from patients with familial adenomatous polyposis. Cancer Res 50:7166–7173
Miyaki M, Konishi M, Kikuchi-Yanoshita R, Enomoto M, Igari T, Tanaka K, Muraoka M, Takahashi H, Amada Y, Fukayama M, Maeda Y, Iwama T, Mishima Y, Mori T, Koike M (1994) Characteristics of somatic mutation of the adenomatous polyposis coli gene in colorectal tumors. Cancer Res 54:3011–3020
Ando M, Takemura K, Maruyama M, Endo M, Iwama T, Yuasa Y (1992) Mutations in c-K-ras 2 gene codon 12 during colorectal tumorigenesis in familial adenomatous polyposis. Gastroenterology 103:1725–1731
Kikuchi-Yanoshita R, Konoshi M, Ito S, Seki M, Tanaka K, Maeda Y, Iino H, Fukayama M, Koike M, Mori T, Sakuraba H, Fukunari H, Iwama T, Miyaki M (1992) Genetic changes of both p53 alleles associated with the conversion from colorectal adenoma to early carcinoma in familial adenomatous polyposis and non-familial adenomatous polyposis patients. Cancer Res 52:3965–3971
Shirasawa S, Urabe K, Yanagawa Y, Toshitani K, Iwama T, Sasazuki T (1991) P53 gene mutations in colorectal tumors from patients with familial polyposis coli. Cancer Res 51:2874–2878
Toyooka M, Konishi M, Kikuchi-Yanoshita R, Iwama T, Miyaki M (1995) Somatic mutations of the adenomatous polyposis coli gene in gastroduodenal tumor from patients with familial adenomatous polyposis. Cancer Res 55:3165–3170
Gallinger S, Vivona AA, Odze RD, Mitri A, O’Beirne CP, Berk TC, Bapat BV (1995) Somatic APC and K-ras codon 12 mutations in periampullary adenomas and carcinomas from familial adenomatous polyposis patients. Oncogene 10:1875–1878
Kashiwagi H, Spigelman AD, Talbot IC, Phillips RKS (1996) P53 overexpression in duodenal tumours in patients with familial adenomatous polyposis. Br J Surg 83:225–228
Kashiwagi H, Spigelman AD, Talbot IC, Debinski HS, McKie AB, Lemoine NR, Phillips RKS (1997) P53 and K-ras status in duodenal adenomas in familial adenomatous polyposis. Br J Surg 84:856–859
Sanabria JR, Croxford R, Berk TC, Cohen Z, Bapat BV, Gallinger S (1996) Familial segregation in the occurrence and severity of periampullary neoplasms in familial adenomatous polyposis. Am J Surg 171:136–141
Soravia C, Berk T, Madlensky L, Mitri A, Cheng H, Gallinger S, Cohen Z, Bapat B (1998) Genotype-phenotype correlations in attenuated adenomatous polyposis coli. Am J Hum Genet 62:1290–1301
Lynch HT, Smyrk T, McGinn T, Lanspa S, Cavalieri J, Lynch J, Slominski-Castor S, Cayouette MC, Priluck I, Luce MC (1995) Attenuated familial adenomatous polyposis (AFAP): a phenotypically and genotypically distinctive variant of FAP. Cancer 76:2427–2433
Leggett BA, Young JP, Biden K, Buttenshaw RL, Knight N, Cowen AE (1997) Severe upper gastrointestinal polyposis associated with sparse colonic polyposis in a familial adenomatous polyposis family with an APC mutation at codon 1520. Gut 41:518–521
Dobbie Z, Heinimann K, Bishop D, Muller H, Scott R (1997) Identification of a modifier gene locus on chromosome 1p35-36 in familial adenomatous polyposis. Hum Genet 99:653–657
Tomlinson IPM, Neal K, Talbot IC, Spigelman AD, Williams CB, Phillips RKS, Bodmer WF (1996) A modifying locus for familial adenomatous polyposis may be present on chromosome 1p35-p36. J Med Genet 33:268–273
Spigelman AD, Farmer KCR, Oliver S, Nugent KP, Bennett PN, Notarianni LJ, Dobrocky P, Phillips RKS (1995) Caffeine phenotyping of cytochrome P4501A2, N-acetyltransferase, and xanthine oxidase in patients with familial adenomatous polyposis. Gut 36:251–254
Nugent KP, Northover J (1994) Total colectomy and ileorectal anastomosis. In: Phillips RKS, Spigelman AD, Thomson JPS (eds) Familial adenomatous polyposis and other polyposis syndromes. Edward Arnold, London, pp 79–91
Bleau BL, Gostout CJ (1996) Endoscopic treatment of ampullary adnomas in familial adenomatous polyposis. J Clin Gastroenterol 22:237–241
Binmoeller KF, Boaventura S, Ramsperger K, Soehendra N (1993) Endoscopic snare excision of benign adenomas of the papilla of Vater. Gastrointest Endosc 39:127–131
Iwama T, Tomita H, Kawachi Y, Yoshinaga K, Kume S, Maruyama H, Mishima Y (1994) Indications for local excision of ampullary lesions associated with familial adenomatous polyposis. J Am Coll Surg 179:462–464
Penna C, Phillips RK, Tiret E, Spigelman AD (1993) Surgical polypectomy of duodenal adenomas in familal adenomatous polyposis; experience of two European centers. Br J Surg 80:1027–1029
Midwinter MJ, Beveridge CJ, Wilsdon JB, Bennett MK, Baudouin CJ, Charnley RM (1999) Correlation between spiral computed tomography, endoscopic ultrasonography and findings at operation in pancreatic and ampullary tumours. Br J Surg 86:189–193
Chung RS, Church JM, van Stock R (1995) Pancreas-sparing duodenectomy; indications, surgical technique, and results. Surgery 117:254–259
Penna C, Bataille N, Balladur P, Tiret E, Parc R (1998) Surgical treatment of severe duodenal polyposis in familial adenomatous polyposis. Br J Surg 85:665–668
Mlkvy P, Messman H, Debinski H, Regula J, Conio M, MacRobert A, Spigelman AD, Phillips RKS, Bown SG (1995) Photodynamic therapy for polyps in familial adenomatous polyposis — a pilot study. Eur J Cancer 31a:1160–1165
Nugent KP, Farmer KCR, Spigelman AD, Williams CB, Phillips RKS (1993) Randomized controlled trial of the effect of sulindac on duodenal and rectal polyposis and cell proliferation in patients with familial adenomatous polyposis. Br J Surg 80:1618–1619
Smith WL (1993) Prostanoid biosynthesis and mechanism of action. Am J Physiol 263:F181-F191
Oshima M, Dinchuk JE, Kargman SL, Oshima H, Hancock B, Kwong E, Traskos JM, Evans JF, Taketo MM (1996) Suppression of intestinal polyposis inApc 716 knockout mice by inhibition of cyclooxygenase 2 (COX-2). Cell 87:803–809
Soew-Chen F, Ho JM, Wong J, Goh HS (1992) Gross and histological abnormalities of the foregut in familial adenomatous polyposis: a study from a South East Asian Registry. Int J Colorect Dis 7:177–183
Church JM, McGannon E, Hull-Boiner S, Sivak MV, van Stolk R, Jagelman DG, Fazio VW, Oakley JR, Lavery IC, Milson JW (1992) Gastroduodenal polyps in patients with familial adenomatous polyposis. Dis Colon Rectum 35:1170–1173
Goedde TA, Rodriguez-Bigas MA, Herrera L, Petrelli NJ (1992) Gastroduodenal polyps in familial adenomatous polyposis. Surg Oncol 1:357–361
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Kashiwagi, H., Spigelman, A.D. Gastroduodenal lesions in familial adenomatous polyposis. Surg Today 30, 675–682 (2000). https://doi.org/10.1007/s005950070077
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DOI: https://doi.org/10.1007/s005950070077