It is a rare, genetic condition caused by a mutation in the gene responsible for it. Most people inherit the gene from one parent. However, the gene mutation occurs on its own without warning in 25% to 30% of people with the condition.
This condition causes excess tissue (polyps) to develop in your large intestine (colon) and rectum. Polyps may also appear in the upper part of your intestinal tract, particularly at the beginning of your small intestine (duodenum). If left untreated, these colon and rectal polyps become cancerous by the time you reach your 40s.
Most people with this condition eventually require surgical removal of their large intestine to prevent cancer. Duodenal polyps can also become cancerous, but this can be prevented with regular screening and prompt polyp removal.
Familial adenomatous polyposis (FAPP) occurs as a result of a defective gene inherited from one parent. However, some people develop the defective gene, causing the condition.
Risk Factors
Your risk of developing FAPP is increased if you have a parent, child, brother, or sister with the condition.
Surgery does not cure familial adenomatous polyposis (FAP). Polyps may continue to form in the remaining or resected portions of the colon, stomach, and small intestine. Depending on the number and size of polyps, laparoscopic removal may not be enough to reduce the risk of cancer. You may need additional surgery.
You will need regular screening—and treatment, if necessary—for complications of FAP that may arise after colorectal surgery. Depending on your history and the type of surgery you underwent, screening may include:
1. Sigmoidoscopy or colonoscopy
2. Upper endoscopy
3. Thyroid ultrasound
4. Computed tomography (CT) or magnetic resonance imaging (MRI) to examine desmoid tumors
Depending on the screening results, your doctor may add additional treatments for the following problems:
1. Duodenal polyps and periampullary polyps.
Your doctor may recommend surgery to remove the upper part of your small intestine (duodenum and ampulla) because these types of benign tumors can develop into cancer.
2_Desseromas.
You may be given a combination of medications, including nonsteroidal anti-inflammatory drugs, antiestrogens, and chemotherapy. Sometimes, surgery may be required.
3_Bone tumors.
Doctors may remove noncancerous bone tumors to relieve pain or for cosmetic reasons.
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Depending on your condition, you may have one of the following types of surgery to remove all or part of your colon:
1. Subtotal colectomy with ileorectal anastomosis, in which the rectum remains in place.
2. Total proctocolectomy with a segregated ileostomy, in which the colon and rectum are removed and an opening (ileostomy) is created, usually on the right side of the abdomen.
3. Total proctocolectomy with ileoanal anastomosis (also called J-pouch surgery), in which the colon and rectum are removed and a portion of the small intestine is attached to the rectum.
If you have familial adenomatous polyposis (FAP), you will need regular imaging tests followed by surgery if necessary.
Surgery can help prevent the development of rectal and colon cancer or other complications.