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Familial Polyposis


Overview :

Familial polyposis (FP) is known by many synonyms, most include some combination of words which reflect what is known about the disease. As the disease is inherited, the word, family, is often included. Because these mushroom-like growths are the most obvious manifestation of the disorder, the word, polyp, is usually in the term as well. Adenoma refers to the particular kind of polyp that is typically discovered. Some of the names found in medical texts and journals include polyposis coli, familial colonic polyposis, multiple familial polyposis, familial adenomatous colon polyposis, adenomatosis of the colon and rectum (ACR), and familial adenomatous polyposis (FAP). The last term and its abbreviation have been commonly used since the early 1990s. It will be used in this discussion.

Familial polyposis or familial adenomatous polyposis (FAP) is a premalignant disease. This means that a person with FAP, if left untreated, will invariably develop cancer. Individuals with this disorder grow hundreds of polyps throughout their large intestines. The polyps, which may also be called adenomas, commonly develop just after puberty. Approximately half of all FAP patients will have polyps by age 14. Ninety percent will have detectable polyps by age 25. Usually by age 35-40, one or more of these polyps will become cancerous.

FAP is a rare disease. One in 8,000 people in the United States have FAP. However, it may be very common in affected families. FAP is inherited in an autosomal dominant pattern. This means that a person with FAP has a 50% chance of passing the condition down to each of their children. FAP can also develop in someone with no family history of the disorder, due to a new genetic mutation in that individual. It is thought that approximately one percent of all colorectal cancers in the United States can be attributed to FAP.




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