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DOI: 10.1055/s-0045-1802333
Familial Polyposis and Colon Cancer
Funding None.

Abstract
Familial adenomatous polyposis is an important hereditary risk factor for colon cancer. Such patients and families need special attention for prevention, early detection, and optimal treatment. Molecular testing is key to identify the specific mutation in the proband and can then make it easier to identify other family members at risk. Aggressive surveillance and colonoscopy will be indicated in most patients. Both colonic and extra-colonic manifestations are important. Chemoprevention is worth considering. Almost all patients will ultimately need colectomy. These details will be discussed in this review.
Publikationsverlauf
Artikel online veröffentlicht:
28. Januar 2025
© 2025. MedIntel Services Pvt Ltd. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)
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References
- 1 IARC. GLOBOCAN 2020. Accessed on February 12, 2022 at: http://globocan.iarc.fr/Default.aspx
- 2 Medina Pabón MA, Babiker HM. A Review of Hereditary Colorectal Cancers. Treasure Island, FL:: StatPearls Publishing;; 2021
- 3 Yang J, Wen Z, Li W. et al. Immune microenvironment: new insight for familial adenomatous polyposis. Front Oncol 2021; 11: 570241
- 4 Torrezan GT, da Silva FC, Santos EM. et al. Mutational spectrum of the APC and MUTYH genes and genotype-phenotype correlations in Brazilian FAP, AFAP, and MAP patients. Orphanet J Rare Dis 2013; 8: 54
- 5 Bisgaard ML, Bülow S. Familial adenomatous polyposis (FAP): genotype correlation to FAP phenotype with osteomas and sebaceous cysts. Am J Med Genet A 2006; 140 (03) 200-204
- 6 Turcot J, Despres JP, St Pierre F. Malignant tumors of the central nervous system associated with familial polyposis of the colon: report of two cases. Dis Colon Rectum 1959; 2: 465-468
- 7 NCCN. NCCN Guidelines Colon Cancer. Accessed February 14, 2022 at: https://www.nccn.org/professionals/physician_gls/pdf/genetics_colon.pdf
- 8 Cruz-Correa M, Hylind LM, Romans KE, Booker SV, Giardiello FM. Long-term treatment with sulindac in familial adenomatous polyposis: a prospective cohort study. Gastroenterology 2002; 122 (03) 641-645
- 9 Samadder NJ, Kuwada SK, Boucher KM. et al. Association of sulindac and erlotinib vs placebo with colorectal neoplasia in familial adenomatous polyposis: secondary analysis of a randomized clinical trial. JAMA Oncol 2018; 4 (05) 671-677
- 10 Vasen HF, Möslein G, Alonso A. et al. Guidelines for the clinical management of familial adenomatous polyposis (FAP). Gut 2008; 57 (05) 704-713
- 11 Syngal S, Brand RE, Church JM, Giardiello FM, Hampel HL, Burt RW. American College of Gastroenterology. ACG clinical guideline: genetic testing and management of hereditary gastrointestinal cancer syndromes. Am J Gastroenterol 2015; 110 (02) 223-262 , quiz 263
- 12 Friederich P, de Jong AE, Mathus-Vliegen LM. et al. Risk of developing adenomas and carcinomas in the ileal pouch in patients with familial adenomatous polyposis. Clin Gastroenterol Hepatol 2008; 6 (11) 1237-1242