Classic familial adenomatous polyposis, called FAP or classic FAP, is a genetic condition. It is diagnosed when a person develops more than adenomatous colon polyps. The average age for polyps to develop in people with FAP is in the mid-teens. Most people with FAP will have multiple colon polyps by age If FAP is not recognized and treated, there is a very high likelihood that a person will develop colorectal cancer.

Individuals with FAP also have an increased chance of developing cancer in other organs, including the stomach , small intestine , and the pancreas and biliary tree. Risk for hepatoblastoma , a rare type of liver cancer, is increased in young children with FAP.

Risk for papillary thyroid cancer is also increased. Congenital hypertrophy of the retinal pigment epithelium CHRPE. This is an eye condition that is present at birth that does not affect vision, but it is a condition that an eye doctor may see during an examination with a special instrument called an ophthalmoscope.

Classic FAP is typically characterized by more than colorectal polyps as described above. Surgery to remove the colon is often the most effective way to manage the polyps and reduce the risk for colorectal cancer. Attenuated FAP AFAP is often associated with multiple adenomatous colorectal polyps, such as 20 to polyps.

People with AFAP usually continue to develop adenomatous colon polyps during their lifetime and have an increased risk of developing colorectal cancer if the polyps are not removed.

People with AFAP tend to develop polyps later in life than individuals with classic FAP; however, the experience of each person can vary among families, and careful surveillance is important.

It has not yet been determined if families with AFAP have the same risk for other types of cancer as families with classic FAP. Similarly, risks for some of the other, non-cancerous features associated with classic FAP have not been determined fully.

Gardner syndrome is a variant of FAP. Like in FAP, people with Gardner syndrome develop multiple adenomatous colon polyps, but in addition, they also develop other tumors outside the gastrointestinal organs, which may include:. Turcot syndrome is considered a variant of either FAP or Lynch syndrome , rather than a distinct genetic condition by itself.

People with Turcot syndrome tend to have multiple adenomatous colon polyps, an increased risk of colorectal cancer, and an increased risk of brain tumors. The type of brain tumor generally depends on whether the Turcot syndrome is more similar to Lynch syndrome or more similar to FAP. The 2 most common types of brain tumors in Turcot syndrome are:.

This type of brain tumor is a very aggressive form of astrocytoma that is commonly found in families who have features of Lynch syndrome. This type of brain tumor begins in the cerebellum, the back of the brain.

Medulloblastoma most often occurs in children and is commonly found in families who have features of FAP. FAP is passed from generation to generation in a family.

Genetic alterations in the APC gene that are present at birth are linked to FAP, AFAP, Gardner syndrome, and Turcot syndrome. This type of change to a gene can also be called a genetic mutation, gene alteration, pathogenic or likely pathogenic germline variant, or a disruptive gene change.

APC stands for adenomatous polyposis coli. Not all individuals with FAP have a family history of FAP. Up to 1 in 3 people with FAP develop a de novo meaning "new" germline mutation in APC. Not all individuals with colorectal polyposis have FAP. Some individuals are found to carry 2 alterations in the MUTYH gene, also called the MYH gene, that is associated with a condition called MUTYH-associated Polyposis MAP.

People who have FAP or AFAP can have a DNA test usually from blood or saliva to look for genetic alterations in the APC gene or the MUTYH gene. In order to evaluate for alterations in other genes which can be less common causes of polyposis, genetic testing is often performed with a multigene panel.

Normally, every cell has 2 copies of each gene: 1 inherited from the mother and 1 inherited from the father. FAP with an APC gene mutation or alteration that is known to disrupt gene function follows an autosomal dominant inheritance pattern.

In autosomal dominant inheritance, this alternation in only 1 copy of the gene is sufficient to develop the condition. This means that a parent with this alteration may pass along a copy of their normal gene or a copy of the gene with the disruptive change.

Options exist for people interested in having a child when a prospective parent carries the genetic change that increases the risk for a hereditary cancer syndrome. Preimplantation genetic diagnosis PGD is a medical procedure done in conjunction with in-vitro fertilization IVF.

It allows people who carry a specific known disruptive gene change to reduce the likelihood that their children will inherit the condition. Eggs are removed and fertilized in a laboratory. When the embryos reach a certain size, 1 cell is removed and is tested for the hereditary condition in question.

The parent s can then choose to transfer embryos which do not have the disruptive gene change. PGD has been in use for over 2 decades and has been used for several hereditary cancer predisposition syndromes.

However, this is a complex procedure with financial, physical, and emotional factors to consider before starting. For more information, talk with an assisted reproduction specialist at a fertility clinic.

FAP and AFAP are uncommon. Specific estimates on how many people have FAP vary from 1 in 22, up to 1 in 7, Most colorectal cancer is sporadic, meaning it occurs by chance, and is not related to FAP or other inherited syndromes. Classic FAP is a clinical diagnosis.

This means that it is typically diagnosed when the doctor finds many colorectal polyps, rather than by the results of a laboratory test.

A person with more than adenomatous colon polyps is considered to have FAP. AFAP is suspected when a person has a history of more than 20, but fewer than , adenomatous colon polyps.

If an alteration is found in the APC gene, other family members may be diagnosed with FAP or its subtypes if they are tested and have the same gene mutation. The risks of cancer for people with Turcot syndrome depend on whether it appears to be more similar to Lynch syndrome or FAP.

Visit the Lynch syndrome section on this website for a summary of cancer risk and other features. ASCO recommends the following screening for people with FAP. It is important to discuss these options with your health care team, as each individual is different:.

Sigmoidoscopy or colonoscopy every 1 to 2 years starting at age 10 to 12 for people with FAP. Individuals with AFAP should undergo colonoscopy beginning at age 18 to Yearly colonoscopy once polyps are found until a colectomy is planned.

There are different types of colon surgery for individuals with FAP and AFAP. The Whipple procedure also called pancreaticoduodenectomy is used for tumours in the head of the pancreas.

It removes the head of the pancreas along with part of the duodenum the first part of the small intestine , the gallbladder, part of the common bile duct, the pylorus bottom part of the stomach that attaches to the duodenum and lymph nodes near the head of the pancreas. The information that the Canadian Cancer Society provides does not replace your relationship with your doctor.

The information is for your general use, so be sure to talk to a qualified healthcare professional before making medical decisions or if you have questions about your health.

We do our best to make sure that the information we provide is accurate and reliable but cannot guarantee that it is error-free or complete. The Canadian Cancer Society is not responsible for the quality of the information or services provided by other organizations and mentioned on cancer.

ca, nor do we endorse any service, product, treatment or therapy. Home Cancer information Cancer types Pancreatic What is pancreatic cancer Precancerous conditions Print. Precancerous conditions of the pancreas. See topics Pancreatic cancer What is pancreatic cancer Risks Signs and symptoms Diagnosis Grading Staging If cancer spreads Prognosis and survival Treatment Supportive care Statistics.

Pancreatic cancer What is pancreatic cancer Risks Signs and symptoms Diagnosis Grading Staging If cancer spreads Prognosis and survival Treatment Supportive care Statistics. The most common precancerous conditions of the pancreas are: Mucinous cystic neoplasm MCN is found in the body and tail of the pancreas.

References American Cancer Society. Pancreatic Cancer. Pancreatic cancer. American Society of Clinical Oncology ASCO. Alexandria, VA. Jankowiski J, Hawk E eds. Handbook of Gastrointestinal Cancer. Wiley-Blackwell; Khan, A. Pancreas, mucinous cystic neoplasm. Omaha: eMedicine, Inc; National Cancer Institute.

Pancreatic Cancer PDQ®: Treatment - Health Professionals. National Cancer Institute; Wexler, A. Unusual pancreatic tumors.

The ampulla, or ampulla of vater, is where the pancreatic duct and bile duct join together to drain into the duodenum, which is the first part of the small intestine. A polyp of the ampulla starts out benign but can turn into cancer of the ampulla as it grows.

Bile duct blockage, bacterial infection, and pancreatitis can occur. More importantly, ampullary polyps lead to ampullary cancer, which is treated in our Multidisciplinary Pancreas Clinic. Usually, there are no symptoms for ampullary polyps until they are blocking the exit of bile or pancreatic juices, which can cause attacks of pancreatitis inflammation of the pancreas or elevated liver enzymes.

Then symptoms can include:. To diagnose ampullary polyps, we begin with a comprehensive exam and collecting a thorough history. An upper endoscopy with biopsy is generally required. Upper endoscopy, also known as an esophagogastroduodenoscopy or EGD , uses an endoscope—a lighted, flexible tube, about the thickness of a finger—to examine the upper gastrointestinal tract, consisting of the esophagus, stomach, and duodenum, which is the first part of the small intestine.

A special instrument is passed through the tube to take a small piece of tissue a biopsy for examination in the laboratory. If there is no evidence of ampullary cancer and the polyp is not too large, the treatment is a procedure called endoscopic retrograde cholangiopancreatography ERCP with placement of a temporary pancreatic duct stent which looks like a plastic straw.

hello ezra. Our Blog. Our Tech Blog. Our Mission. Our Advisors. Our Care Team. Full Body MRI Scan. Conditions We Scan For. Prostate AI. Privacy Policy. Telehealth Authorization. Follow Us. For Providers. For Employers. This type of brain tumor is a very aggressive form of astrocytoma that is commonly found in families who have features of Lynch syndrome.

This type of brain tumor begins in the cerebellum, the back of the brain. Medulloblastoma most often occurs in children and is commonly found in families who have features of FAP. FAP is passed from generation to generation in a family. Genetic alterations in the APC gene that are present at birth are linked to FAP, AFAP, Gardner syndrome, and Turcot syndrome.

This type of change to a gene can also be called a genetic mutation, gene alteration, pathogenic or likely pathogenic germline variant, or a disruptive gene change. APC stands for adenomatous polyposis coli.

Not all individuals with FAP have a family history of FAP. Up to 1 in 3 people with FAP develop a de novo meaning "new" germline mutation in APC. Not all individuals with colorectal polyposis have FAP.

Some individuals are found to carry 2 alterations in the MUTYH gene, also called the MYH gene, that is associated with a condition called MUTYH-associated Polyposis MAP.

People who have FAP or AFAP can have a DNA test usually from blood or saliva to look for genetic alterations in the APC gene or the MUTYH gene. In order to evaluate for alterations in other genes which can be less common causes of polyposis, genetic testing is often performed with a multigene panel.

Normally, every cell has 2 copies of each gene: 1 inherited from the mother and 1 inherited from the father. FAP with an APC gene mutation or alteration that is known to disrupt gene function follows an autosomal dominant inheritance pattern. In autosomal dominant inheritance, this alternation in only 1 copy of the gene is sufficient to develop the condition.

This means that a parent with this alteration may pass along a copy of their normal gene or a copy of the gene with the disruptive change. Options exist for people interested in having a child when a prospective parent carries the genetic change that increases the risk for a hereditary cancer syndrome.

Preimplantation genetic diagnosis PGD is a medical procedure done in conjunction with in-vitro fertilization IVF. It allows people who carry a specific known disruptive gene change to reduce the likelihood that their children will inherit the condition.

Eggs are removed and fertilized in a laboratory. When the embryos reach a certain size, 1 cell is removed and is tested for the hereditary condition in question. The parent s can then choose to transfer embryos which do not have the disruptive gene change.

PGD has been in use for over 2 decades and has been used for several hereditary cancer predisposition syndromes. However, this is a complex procedure with financial, physical, and emotional factors to consider before starting.

For more information, talk with an assisted reproduction specialist at a fertility clinic. FAP and AFAP are uncommon. Specific estimates on how many people have FAP vary from 1 in 22, up to 1 in 7, Most colorectal cancer is sporadic, meaning it occurs by chance, and is not related to FAP or other inherited syndromes.

Classic FAP is a clinical diagnosis. This means that it is typically diagnosed when the doctor finds many colorectal polyps, rather than by the results of a laboratory test.

A person with more than adenomatous colon polyps is considered to have FAP. AFAP is suspected when a person has a history of more than 20, but fewer than , adenomatous colon polyps.

If an alteration is found in the APC gene, other family members may be diagnosed with FAP or its subtypes if they are tested and have the same gene mutation.

The risks of cancer for people with Turcot syndrome depend on whether it appears to be more similar to Lynch syndrome or FAP. Visit the Lynch syndrome section on this website for a summary of cancer risk and other features.

ASCO recommends the following screening for people with FAP. It is important to discuss these options with your health care team, as each individual is different:.

Sigmoidoscopy or colonoscopy every 1 to 2 years starting at age 10 to 12 for people with FAP. Individuals with AFAP should undergo colonoscopy beginning at age 18 to Yearly colonoscopy once polyps are found until a colectomy is planned. There are different types of colon surgery for individuals with FAP and AFAP.

People with classic FAP may require a total colectomy, due to a high number of polyps and the high risk of colorectal cancer.