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Table 3. Absolute Risks of Colorectal Cancer for Mutation Carriers in Hereditary Colorectal Cancer Syndromes
Syndrome
|
Absolute Risk in Mutation Carriers
|
FAP |
90% by age 45 y [1] |
Attenuated FAP |
69% by age 80 y [2]
|
Lynch |
80% by age 75 ya [3] |
MYH-associated neoplasia |
Not established |
Peutz-Jeghers |
39% by age 70 y [4] |
Juvenile polyposis |
17% to 68% by age 60 y [5,6] |
FAP = familial adenomatous polyposis.
|
aSee text on Lynch syndrome for a full discussion of risk.
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References
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Bussey HJ: Familial Polyposis Coli: Family Studies, Histopathology, Differential Diagnosis, and Results of Treatment. Baltimore, Md: The Johns Hopkins University Press, 1975.
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Burt RW, Leppert MF, Slattery ML, et al.: Genetic testing and phenotype in a large kindred with attenuated familial adenomatous polyposis. Gastroenterology 127 (2): 444-51, 2004.
[PUBMED Abstract]
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Vasen HF, Wijnen JT, Menko FH, et al.: Cancer risk in families with hereditary nonpolyposis colorectal cancer diagnosed by mutation analysis. Gastroenterology 110 (4): 1020-7, 1996.
[PUBMED Abstract]
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Hearle N, Schumacher V, Menko FH, et al.: Frequency and spectrum of cancers in the Peutz-Jeghers syndrome. Clin Cancer Res 12 (10): 3209-15, 2006.
[PUBMED Abstract]
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Coburn MC, Pricolo VE, DeLuca FG, et al.: Malignant potential in intestinal juvenile polyposis syndromes. Ann Surg Oncol 2 (5): 386-91, 1995.
[PUBMED Abstract]
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Desai DC, Neale KF, Talbot IC, et al.: Juvenile polyposis. Br J Surg 82 (1): 14-7, 1995.
[PUBMED Abstract]
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