Familial adenomatous polyposis natural history, complications and prognosis
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Familial adenomatous polyposis is a rare disease that usually starts from childhood. Patients with familial adenomatous polyposis develop numerous colon polyps, which may vary from hundreds to thousands, by early twenties. If patients with FAP left untreated, almost all of the colon polyps develop colorectal cancer around 40 years of age. Patients with FAP may have polyps in different organs including the upper gastrointestinal tract. However, the progression of these extra colonic polyps, to malignancy is less than colon polyps. Patients with FAP might develop duodenal and ampullary cancer which are major causes of death following polypectomy. The prognosis of familial adenomatous polyposis is excellent with treatment.
Following are a few important facts about the natural history of familial adenomatous polyposis:
- Familial adenomatous polyposis is a rare disease that usually starts during teenage years.
- Patients with FAP usually develop hundreds to thousands colon polyps by the age of early twenties.
- Patients with FAP might be asymptomatic till third decade of their lives or whenever they have malignant transformation to colorectal cancer.
- If left untreated, almost all of the patients with FAP develop colorectal cancer around 40 years of age.
- Patients with FAP might have polyps in different organs including upper gastrointestinal tract. However, their progression to malignancy is less than colon polyps.
- Colon cancer which has two different pathways according to defect on APC gene:
- 1- Molecular pathogenesis of sporadic colon cancer is as follows:
- 2- Molecular pathogenesis of colitis-associated colon cancer is as follows:
- Duodenal and ampullary cancer:
- They are major causes of death following polypectomy.
- End-viewing and side-viewing duodenoscopy are conducted for surveillance.
- The prognosis of familial adenomatous polyposis is excellent with treatment.
- Without treatment, all patients with familial adenomatous polyposis develop colon cancer at age of 40-50.
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