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Genetics of Colorectal Cancer (PDQ®)

  • Updated: 02/28/2013

Table 5. Extracolonic Tumor Risks in Familial Adenomatous Polyposis

Malignancy  Relative Risk  Absolute Lifetime Risk (%)  
Desmoid852.015.0
Duodenum330.85.0–12.0
Thyroid7.62.0
Brain7.02.0
Ampullary123.71.7
Pancreas4.51.7
Hepatoblastoma847.01.6
Gastric—0.6a

Adapted from Giardiello et al.,[12] Jagelman et al.,[13] Sturt et al.,[14] Lynch et al.,[15] Bülow et al.,[16] Burt et al.,[17] and Galiatsatos et al.[18]
aThe Leeds Castle Polyposis Group.

References

  1. Giardiello FM, Offerhaus JG: Phenotype and cancer risk of various polyposis syndromes. Eur J Cancer 31A (7-8): 1085-7, 1995 Jul-Aug.  [PUBMED Abstract]

  2. Jagelman DG, DeCosse JJ, Bussey HJ: Upper gastrointestinal cancer in familial adenomatous polyposis. Lancet 1 (8595): 1149-51, 1988.  [PUBMED Abstract]

  3. Sturt NJ, Gallagher MC, Bassett P, et al.: Evidence for genetic predisposition to desmoid tumours in familial adenomatous polyposis independent of the germline APC mutation. Gut 53 (12): 1832-6, 2004.  [PUBMED Abstract]

  4. Lynch HT, Fitzgibbons R Jr: Surgery, desmoid tumors, and familial adenomatous polyposis: case report and literature review. Am J Gastroenterol 91 (12): 2598-601, 1996.  [PUBMED Abstract]

  5. Bülow S, Björk J, Christensen IJ, et al.: Duodenal adenomatosis in familial adenomatous polyposis. Gut 53 (3): 381-6, 2004.  [PUBMED Abstract]

  6. Burt RW: Colon cancer screening. Gastroenterology 119 (3): 837-53, 2000.  [PUBMED Abstract]

  7. Galiatsatos P, Foulkes WD: Familial adenomatous polyposis. Am J Gastroenterol 101 (2): 385-98, 2006.  [PUBMED Abstract]