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Prevention and Management of Periampullary Tumors in Familial Adenomatous Polyposis

DOI: 10.4236/oalib.1102338, PP. 1-5

Subject Areas: Gastroenterology & Hepatology

Keywords: Familial Adenomatous Polyposis (FAP), Duodenal Adenocarcinoma, Spigelman Classfication, Pancreas-Preserving Total Duodenectomy (PPTD)

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Abstract

Familial adenomatosis polyposis (FAP) is one of two well described forms of hereditary colorectal cancer. The primary cause of death from this symdrome is colorectal caner which develops usually by the fifth decade of life. The overall survival of FAP was significantly improved by screening by genetic testing and endoscopy with prophylactic surgery. Almost all patients with FAP develop duodenal polyposis most of which occurs in periampullary. The second leading cause of death in FAP is duodenal adenocarcinoma. Between 5% and 10% of FAP patients die from duodenal cancer, usually periampullary in origin. Therefore, I will discuss the clinicopathological features, management and prevention of duodenal neoplasma in patients with FAP.

Cite this paper

Hashimoto, N. (2016). Prevention and Management of Periampullary Tumors in Familial Adenomatous Polyposis. Open Access Library Journal, 3, e2338. doi: http://dx.doi.org/10.4236/oalib.1102338.

References

[1]  Alderlieste, Y.A., Rauws, E.A., Mathus-Vliegen, E.M., et al. (2013) Prospective Enteroscopic Evaluation of Jejunal Polyposis in Patients with Familial Adenomatous Polyposis and Advanced Duodenal Polyposis. Familial Cancer, 12, 51-56.
http://dx.doi.org/10.1007/s10689-012-9571-1
[2]  Trimboth, J.D. and Giardiello, F.M. (2002) Genetic Testing and Counseling for Hereditary Colorectal Cancer. Ali- mentary Pharmacology & Therapeutics, 16, 1843-1857.
http://dx.doi.org/10.1046/j.1365-2036.2002.01357.x
[3]  Basford, P.J. and Bhandari, P. (2012) Endoscopic Management of Nonampullary Duodenal Polyposis. Therapeutic Advances in Gastroenterology, 5, 127-138.
http://dx.doi.org/10.1177/1756283X11429590
[4]  Tonelli, F., Nordi, F., Bechi, P., et al. (1985) Extracolonic Polyps in Familial Polyposis Coli and Garder’s Syndrome. Diseases of the Colon & Rectum, 28, 664-668.
http://dx.doi.org/10.1007/BF02553447
[5]  Bulow, S., Bjork, J., Christensen, U., et al. (2004) Duodenal Adenomatosis in Familial Adenomatous Polyposis. Gut, 53, 381-386.
http://dx.doi.org/10.1136/gut.2003.027771
[6]  Heiskanen, I., Kellokumpu, I. and Jarvinen, H. (1999) Management of Duodenal Adenomas in 98 Patients with Familial Adenomatous Polyposis. Endoscopy, 31, 412-416.
http://dx.doi.org/10.1055/s-1999-41
[7]  Pauli, R.M., Pauli, M.E. and Hall, J.G. (1980) Gardner Syndrome and periampullary Malignancy. American Journal of Medical Genetics, 6, 205-219.
http://dx.doi.org/10.1002/ajmg.1320060305
[8]  Vasen, H.F., Bulow, S., Myrhoi, T., et al. (1997) Decision Analysis in the Management of Duodenal Adenomatosis in Familial Adenomatous Polyposis. Gut, 40, 716-719.
http://dx.doi.org/10.1136/gut.40.6.716
[9]  Bjork, J., Akerbrant, H., Iselius, L., et al. (2001) Periampullary Adenomas and Adenocarcinomas in Familial Adeno- matous Polyposis: Cumulative risks and APC Gene Mutations. Gastroenterology, 121, 1127-1135.
http://dx.doi.org/10.1053/gast.2001.28707
[10]  Nugent, K.P., Spigelman, A.D. and Phillips, R.K. (1993) Life Expectancy after Cololectomy and Ileorectal Ana- stomosis for Familial Adenomatous Polyposis. Diseases of the Colon & Rectum, 36, 1059-1062.
http://dx.doi.org/10.1007/BF02047300
[11]  Spigelman, A.D., Williams, C.B., Talbot, I.C., et al. (1989) Upper Gastrointestinal Cancer in Patients with Familial Adenomatous Polyposis. The Lancet, 2, 783-785.
http://dx.doi.org/10.1016/S0140-6736(89)90840-4
[12]  Tonelli, F., Nardi, F., Bechi, P., et al. (1985) Extracolonic Polyps in Familial Polyposis Coli and Gardner’s Syndrome. Diseases of the Colon & Rectum, 28, 664-668.
http://dx.doi.org/10.1007/BF02553447
[13]  Davies, D.R., et al. (1995) Sever Gardner Syndrome in Families with Mutations Restricted to a Specific Region of the APC Gene. Am J of Hum Genet, 57, 1151-1158.
[14]  Evans, D.G.R., Guy, S.P. and Thakker, N. (1993) Non Peneterance and Late Appearance of Polyps in Families with Familial Adenomatous Polyposis. Gut, 34, 1389-1393.
http://dx.doi.org/10.1136/gut.34.10.1389
[15]  Morpurgo, E., Vitale, G.C., Galandiuk, S., et al. (2004) Clincal Characteiietics of Familial Adenomatous Polyposis and Management of Duodenal Adenomas. Journal of Gastrointestinal Surgery, 8, 559-564.
[16]  Bertario, L., Russo, A., Sala, P., et al. (2003) Hereditary Colorectal Tumor Registry. Multiple Approach to the Exploration of Genotype-Phenotype Correlations in Familial Adenomatous Polyposis. Journal of Clinical Oncology, 21, 1698-1707.
[17]  Phillips, R.K., Wallace, M.H., Lynch, P.M., et al. (2002) A Randomised, Double Blind, Placebo Controlled Study of Celecoxib, a Selective Cyclooxygenase 2 Inhibitor, on Duodenal Polyposis in Familial Adenomatous Polyposis. Gut, 50, 857-860.
http://dx.doi.org/10.1136/gut.50.6.857
[18]  Winde, G., Schmid, K.W., Brandt, B., et al. (1997) Clinical and Genomic Influence of Sulindac on Rectal Mucosa in Familial Adenomatous Polyposis. Diseases of the Colon & Rectum, 40, 1156-1168.
http://dx.doi.org/10.1007/BF02055161
[19]  Mahmoud, N.N., Dannenberg, A.J., Bilinski, R.T., et al. (1999) Administration of Anunconjugated Bile Acid Increases Duodenal Tumors in a Murine Model of Familial Adenomatous Polyposis. Carcinogenesis, 20, 299-303.
http://dx.doi.org/10.1093/carcin/20.2.299
[20]  Morpurgo, E., Vitale, G.C., Galandiuk, S., et al. (2004) Clinical Characteristics of Familial Adenomatous Polyposis and Management of Duodenal Adenomas. Journal of Gastrointestinal Surgery, 8, 559-564.
http://dx.doi.org/10.1016/j.gassur.2004.03.001
[21]  Alarcon, F.J., Burke, C.A., Church, J.M., et al. (1999) Familial Adenomatous Polyposis: Efficacy of Endoscopic and Surgical Treatment for Advanced Duodenal Adenomas. Diseases of the Colon & Rectum, 42, 1533-1536.
[22]  Penna, C., Phillips, R.K., Tiret, F., et al. (1993) Surgical Polypectomy of Duodenal Adenomas in Familial Adenomatous Polyposis. Experience of Two European Centers. British Journal of Surgery, 80, 1027-1029.
http://dx.doi.org/10.1002/bjs.1800800833
[23]  Clary, B.M., Tyler, D.S., Dematos, P., et al. (2000) Local Ampullary Resection with Careful Intraoperative Frozen Section Evaluation for Presumed Benign Ampullary Neoplasma. Surgery, 127, 628-633.
http://dx.doi.org/10.1067/msy.2000.106532
[24]  Penna, C., Bataille, N., Balladur, P., et al. (1998) Surgical Treatment of Sever Duodenal Polyposis in Familial Adenomatous Polyposis. British Journal of Surgery, 85, 665-668.
http://dx.doi.org/10.1046/j.1365-2168.1998.00684.x
[25]  Chung, R.S., Church, J.M. and Van Stolk, R. (1995) Pancreas-Sparing Duodenectomy: Indications, Surgical Technique, and Results. Surgery, 117, 254-259.
http://dx.doi.org/10.1016/S0039-6060(05)80198-9

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