Materiały źródłowe

• #1 Familial Adenomatous Polyposis (FAP): Symptoms, Diagnosis, Risks

  https://my.clevelandclinic.org/health/diseases/16993-familial-adenomatous-polyposis-fap

  Familial adenomatous polyposis (FAP) is a hereditary syndrome that raises your risk of developing colorectal cancer to nearly 100%. Cancer develops from adenomatous colon polyps, which can appear in the hundreds to thousands. To manage this risk, most people with FAP will have their whole colon removed sometime in their early life. […] Many people get a few colon polyps at random as they get older. But if you have a hereditary polyposis syndrome like FAP, you’ll develop many colon polyps typically over a hundred starting from a young age. This significantly raises your lifetime risk that one of them will become cancerous. […] Without treatment, the risk of developing colorectal cancer with familial adenomatous polyposis is close to 100%. It also develops relatively earlier and faster with FAP than in those without.

• #2 Familial adenomatous polyposis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/familial-adenomatous-polyposis/symptoms-causes/syc-20372443

  The main sign of FAP is hundreds or even thousands of polyps growing in your colon and rectum, usually starting by your mid-teens. The polyps are nearly 100 percent certain to develop into colon cancer or rectal cancer by the time you’re in your 40s. […] Most people with familial adenomatous polyposis eventually need surgery to remove the large intestine to prevent cancer. The polyps in the duodenum also can develop cancer, but they can usually be managed by careful monitoring and by removing polyps regularly. […] Some people have a milder form of the condition, called attenuated familial adenomatous polyposis (AFAP). People with AFAP usually have fewer colon polyps (an average of 30) and develop cancer later in life.

• #3 Familial Adenomatous Polyposis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538233/

  Familial adenomatous polyposis is an autosomal dominant polyposis syndrome characterized by mutations in the adenomatous polyposis coli gene. Patients with familial adenomatous polyposis develop hundreds to thousands of polyps throughout the colon and rectum, significantly elevating their lifetime risk of colorectal cancer if left untreated. […] If left untreated, affected individuals develop hundreds to thousands of polyps throughout the colon and rectum, often manifesting in the early teenage years. This condition ultimately results in an almost 100% lifetime risk of colorectal cancer, typically occurring by age 40. Colectomy is recommended to significantly reduce the risk of developing colorectal cancer. Individuals with FAP face an elevated risk of other malignancies, such as gastric and duodenal adenocarcinoma, hepatoblastoma, and desmoid tumors.

• #4 Familial Adenomatous Polyposis (FAP): Symptoms, Diagnosis, Risks

  https://my.clevelandclinic.org/health/diseases/16993-familial-adenomatous-polyposis-fap

  Colon polyps in FAP begin to appear much earlier than in the general population, often during the teenage years. They usually won’t cause symptoms until they’ve grown large enough to be dangerous. But if you have a colonoscopy for screening purposes, your healthcare provider will find them. […] People with FAP often have hundreds or thousands of colon polyps. Those with AFAP have at least 20. The greater number and faster growth of polyps in FAP may make them more likely to cause symptoms than polyps usually are. Symptoms may include rectal bleeding, diarrhea or chronic abdominal pain. […] Colon polyps in FAP begin to appear at the average age of 16. […] If you’re diagnosed with FAP, you can expect a lifetime of medical screenings and possibly multiple surgeries to remove tumors. Tumors outside of your colon are less likely to become cancerous than colon polyps. Desmoid tumors, which aren’t cancerous, can be a minor irritation or a major one. […] You can expect to have a total colectomy sometime in your early life.

• #5 Familial Adenomatous Polyposis: Symptoms, Causes, Treatment

  https://www.healthline.com/health/colorectal-cancer/familial-adenomatous-polyposis

  Familial adenomatous polyposis (FAP) is a rare condition that predisposes you (makes you more susceptible) to precancerous colorectal adenomatous polyps. […] FAP usually requires the removal of part or all of your large intestines. If left treated, it almost always leads to colorectal cancer at a relatively young age. […] People with FAP develop hundreds to thousands of these polyps along their rectum and colon. […] Polyps develop between the ages of 7 to 36, and the average age of development is 16. If left unrecognized and untreated, they almost always turn into colorectal cancer by your 30s or 40s. […] The primary sign of FAP is the development of hundreds to thousands of polyps. Common symptoms can include abdominal pain, diarrhea, blood in stools, and dark, sticky feces. […] People with FAP almost always develop colon cancer in their 30s or 40s if they don’t receive surgery to remove part or all of their colon.

• #6 Familial Adenomatous Polyposis: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/175377-overview

  Familial adenomatous polyposis (FAP) is the most common adenomatous polyposis syndrome. It is an autosomal dominant inherited disorder characterized by the early onset of hundreds to thousands of adenomatous polyps throughout the colon. If left untreated, all patients with this syndrome will develop colon cancer by age 35-40 years. In addition, an increased risk exists for the development of other malignancies. […] The average age of onset of polyposis in FAP is 16 years, whereas the average age of onset for colorectal cancer is 39 years. […] Patients with untreated FAP have a median life expectancy of 42 years. Life expectancy is extended greatly in those treated with colectomy. […] The principal cause of mortality is colorectal cancer, which develops in all patients unless they are treated. The mean age at which colorectal cancer develops in patients with classic FAP is 39 years. Patients with adenomatous polyposis itself often are asymptomatic. […] Complications of FAP include the following: Colorectal cancer (100% in untreated patients).

• #7 Familial Adenomatous Polyposis – Gastrointestinal Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/gastrointestinal-disorders/tumors-of-the-gastrointestinal-tract/familial-adenomatous-polyposis

  Familial adenomatous polyposis is a hereditary disorder causing numerous colonic polyps and frequently results in colon carcinoma, often by age 40. Patients are usually asymptomatic but may have heme-positive stool. […] Many patients are asymptomatic, but rectal bleeding, typically occult, occurs. […] Polyps are present in 50% of patients by age 15 years, and 95% by age 35 years. Cancer develops before age 40 in nearly all untreated patients. […] Patients also can develop various extracolonic manifestations (previously termed Gardner syndrome), both benign and malignant. […] Patients have an increased risk of other cancers, particularly of the duodenum, and also the pancreas, thyroid, brain, and liver.

• #8 Familial adenomatous polyposis syndromes – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-gb/652

  Familial adenomatous polyposis (FAP) syndrome is a hereditary colorectal cancer syndrome characterised by the development of hundreds to thousands of colorectal adenomas. The attenuated form leads to the formation of fewer than 100 polyps. […] Median age of onset for polyps is 15 years. […] In the absence of total colectomy or endoscopic polyp clearance, colorectal cancer arises in close to 100% of patients with FAP by 40 years of age and in 80% of patients with attenuated FAP by 60 years of age. […] Patients with classical FAP have hundreds to thousands of colorectal adenomas and a nearly 100% risk for colorectal cancer by the age of 40 years if proctoprophylactic colectomy is not performed. […] Attenuated FAP is also caused by APC mutations and is characterised by fewer than 100 adenomas and a later age of colorectal cancer onset.

• #9 Familial Adenomatous Polyposis (FAP): Symptoms, Diagnosis, Risks

  https://my.clevelandclinic.org/health/diseases/16993-familial-adenomatous-polyposis-fap

  Colon polyps in FAP begin to appear much earlier than in the general population, often during the teenage years. They usually won’t cause symptoms until they’ve grown large enough to be dangerous. But if you have a colonoscopy for screening purposes, your healthcare provider will find them. […] People with FAP often have hundreds or thousands of colon polyps. Those with AFAP have at least 20. The greater number and faster growth of polyps in FAP may make them more likely to cause symptoms than polyps usually are. Symptoms may include rectal bleeding, diarrhea or chronic abdominal pain. […] Colon polyps in FAP begin to appear at the average age of 16. […] If you’re diagnosed with FAP, you can expect a lifetime of medical screenings and possibly multiple surgeries to remove tumors. Tumors outside of your colon are less likely to become cancerous than colon polyps. Desmoid tumors, which aren’t cancerous, can be a minor irritation or a major one. […] You can expect to have a total colectomy sometime in your early life.

• #10 Familial Adenomatous Polyposis (FAP) | MUSC Health | Charleston SC

  https://muschealth.org/medical-services/ddc/patients/digestive-diseases/colon-and-rectum/familial-adenomatous-polyposis

  Familial Adenomatous Polyposis is an inherited condition, primarily affecting the colon and rectum. People who inherit it develop numerous polyps on the inner lining of their colon and/or rectum. These polyps (also called adenomas) develop in the teenage years. If untreated, one or more of the polyps eventually will become cancerous in young adulthood. […] Most patients develop polyps without symptoms. However, the following symptoms may occur: blood in the stool, diarrhea, constipation, crampy abdominal pain, decrease in size of stool, weight loss. […] It should be emphasized that there is no safety in simply waiting for symptoms to develop.

• #11 Familial Adenomatous Polyposis | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/familial-adenomatous-polyposis

  Familial adenomatous polyposis (FAP) is a hereditary cancer predisposition syndrome characterized by the development of hundreds of gastrointestinal polyps in the small and large intestines. If left untreated, there is nearly a 100 percent chance a person with FAP will develop colorectal cancer likely before age 40. […] The most common symptoms of classic FAP are abdominal pain, cramping, and vomiting (with blood in the vomit) or bleeding with bowel movements secondary to the presence of 100 or more adenomatous polyps in the small and/or large intestines. However, some individuals with FAP may not have symptoms from the polyps. Individuals with FAP generally begin to develop polyps in their mid-teens; by age 35, more than 95 percent of individuals with FAP will have multiple colon polyps. […] Individuals with FAP will need long-term follow-up care for the rest of their lives. They have a nearly 100 percent chance of developing colorectal cancer if colon polyps are not removed and the condition is left untreated.

• #12 Age and manifestation related symptoms in familial adenomatous polyposis | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/1471-2407-5-24

  FAP was diagnosed clinically on the basis of symptoms in 84% (120/143) of these patients. Most presented with intestinal symptoms such as colonic bleeding (68%) and diarrhea (42%). All but one of the patients between 20 and 40 years old had rectal polyps (98.7%, 75/76), whereas in those over 40 years old the prevalence was 76% (35/46). Non-specific symptoms such as abdominal pain, fatigue and bloating were less frequent and were mainly reported by patients older than 40. […] The commonest presenting features of FAP are alteration of bowel habit and rectal bleeding, but both are found in many other conditions. Patients with these findings need immediate endoscopy to allow prompt diagnosis and prophylactic surgery. […] The diagnosis of FAP was established clinically if more than 100 polyps were detected endoscopically in the colorectum.

• #13 Familial Adenomatous Polyposis | Columbia Surgery

  https://columbiasurgery.org/conditions-and-treatments/familial-adenomatous-polyposis

  Symptoms can vary from person to person, even when the condition exists within the same family, and range from mild to severe. For people with FAP, the development of polyps within their colon and rectum usually begins in their teens. Polyps arent typically painful, but they can bleed and lead to the appearance of blood in stool. Bleeding from polyps can also lead to the gradual development of anemia. […] The mutated APC gene that causes FAP can also lead to issues with other organs, like bones, the brain, the thyroid gland, and the retinas of the eye. Some of the most common other organs to develop polyps in FAP are the stomach and the duodenum (the first part of the small intestine). Rarely, the polyps in the stomach and the duodenum can develop into stomach cancer and duodenal cancer, respectively. […] If the polyps associated with FAP develop into malignant cancers, other symptoms may develop, including: Fatigue, Weight loss, Altered bowel habits.

• #14 Age and manifestation related symptoms in familial adenomatous polyposis | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/1471-2407-5-24

  FAP was diagnosed clinically on the basis of symptoms in 84% (120/143) of these patients. Most presented with intestinal symptoms such as colonic bleeding (68%) and diarrhea (42%). All but one of the patients between 20 and 40 years old had rectal polyps (98.7%, 75/76), whereas in those over 40 years old the prevalence was 76% (35/46). Non-specific symptoms such as abdominal pain, fatigue and bloating were less frequent and were mainly reported by patients older than 40. […] The commonest presenting features of FAP are alteration of bowel habit and rectal bleeding, but both are found in many other conditions. Patients with these findings need immediate endoscopy to allow prompt diagnosis and prophylactic surgery. […] The diagnosis of FAP was established clinically if more than 100 polyps were detected endoscopically in the colorectum.

• #15 Age and manifestation related symptoms in familial adenomatous polyposis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1079798/

  FAP was diagnosed clinically on the basis of symptoms in 84% (120/143) of these patients. Most presented with intestinal symptoms such as colonic bleeding (68%) and diarrhea (42%). All but one of the patients between 20 and 40 years old had rectal polyps (98.7%, 75/76), whereas in those over 40 years old the prevalence was 76% (35/46). Non-specific symptoms such as abdominal pain, fatigue and bloating were less frequent and were mainly reported by patients older than 40. […] The commonest presenting features of FAP are alteration of bowel habit and rectal bleeding, but both are found in many other conditions. Patients with these findings need immediate endoscopy to allow prompt diagnosis and prophylactic surgery. […] Untreated, the disease usually leads to the appearance of hundreds of adenomatous polyps in the colorectum between puberty and age 20 and to cancer by the early forties at the latest which is the most frequent reason for death in patients with FAP.

• #16 Age and manifestation related symptoms in familial adenomatous polyposis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1079798/

  The predominant symptoms of FAP were analyzed and documented retrospectively in all 143 cases. […] Colonic bleeding, anemia, mucous discharge, diarrhea, paradoxical diarrhea and constipation were categorized as bleeding and stool disorders. […] In 59% (84/143) tests for fecal occult blood were positive. […] Colonic bleeding as an early symptom was present in 52% (74/143). This was found in 68% (52/76) of patients between 20 and 40 years old and in 35% (16/46) of patients older than 40 years. […] Diarrhea was reported in 31% (45/143). 42% (32/76) of patients between 20 and 40 years but only in 24% (11/46) older than 40 years suffered from this symptom.

• #17 Age and manifestation related symptoms in familial adenomatous polyposis | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/1471-2407-5-24

  The predominant symptoms of FAP were analyzed and documented retrospectively in all 143 cases. […] Colonic bleeding as an early symptom was present in 52% (74/143). This was found in 68% (52/76) of patients between 20 and 40 years old and in 35% (16/46) of patients older than 40 years. […] Diarrhea was reported in 31% (45/143). 42% (32/76) of patients between 20 and 40 years but only in 24% (11/46) older than 40 years suffered from this symptom. […] The incidence of colonic carcinoma was 42-50% in this age group. We conclude that non-specific symptoms can imply a pronounced manifestation of FAP which usually affects patients beyond 40 years of age.

• #18 Age and manifestation related symptoms in familial adenomatous polyposis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1079798/

  The predominant symptoms of FAP were analyzed and documented retrospectively in all 143 cases. […] Colonic bleeding, anemia, mucous discharge, diarrhea, paradoxical diarrhea and constipation were categorized as bleeding and stool disorders. […] In 59% (84/143) tests for fecal occult blood were positive. […] Colonic bleeding as an early symptom was present in 52% (74/143). This was found in 68% (52/76) of patients between 20 and 40 years old and in 35% (16/46) of patients older than 40 years. […] Diarrhea was reported in 31% (45/143). 42% (32/76) of patients between 20 and 40 years but only in 24% (11/46) older than 40 years suffered from this symptom.

• #19 Age and manifestation related symptoms in familial adenomatous polyposis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1079798/

  The predominant symptoms of FAP were analyzed and documented retrospectively in all 143 cases. […] Colonic bleeding, anemia, mucous discharge, diarrhea, paradoxical diarrhea and constipation were categorized as bleeding and stool disorders. […] In 59% (84/143) tests for fecal occult blood were positive. […] Colonic bleeding as an early symptom was present in 52% (74/143). This was found in 68% (52/76) of patients between 20 and 40 years old and in 35% (16/46) of patients older than 40 years. […] Diarrhea was reported in 31% (45/143). 42% (32/76) of patients between 20 and 40 years but only in 24% (11/46) older than 40 years suffered from this symptom.

• #20 Age and manifestation related symptoms in familial adenomatous polyposis | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/1471-2407-5-24

  The predominant symptoms of FAP were analyzed and documented retrospectively in all 143 cases. […] Colonic bleeding as an early symptom was present in 52% (74/143). This was found in 68% (52/76) of patients between 20 and 40 years old and in 35% (16/46) of patients older than 40 years. […] Diarrhea was reported in 31% (45/143). 42% (32/76) of patients between 20 and 40 years but only in 24% (11/46) older than 40 years suffered from this symptom. […] The incidence of colonic carcinoma was 42-50% in this age group. We conclude that non-specific symptoms can imply a pronounced manifestation of FAP which usually affects patients beyond 40 years of age.

• #21 Age and manifestation related symptoms in familial adenomatous polyposis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1079798/

  The predominant symptoms of FAP were analyzed and documented retrospectively in all 143 cases. […] Colonic bleeding, anemia, mucous discharge, diarrhea, paradoxical diarrhea and constipation were categorized as bleeding and stool disorders. […] In 59% (84/143) tests for fecal occult blood were positive. […] Colonic bleeding as an early symptom was present in 52% (74/143). This was found in 68% (52/76) of patients between 20 and 40 years old and in 35% (16/46) of patients older than 40 years. […] Diarrhea was reported in 31% (45/143). 42% (32/76) of patients between 20 and 40 years but only in 24% (11/46) older than 40 years suffered from this symptom.

• #22 Age and manifestation related symptoms in familial adenomatous polyposis | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/1471-2407-5-24

  FAP was diagnosed clinically on the basis of symptoms in 84% (120/143) of these patients. Most presented with intestinal symptoms such as colonic bleeding (68%) and diarrhea (42%). All but one of the patients between 20 and 40 years old had rectal polyps (98.7%, 75/76), whereas in those over 40 years old the prevalence was 76% (35/46). Non-specific symptoms such as abdominal pain, fatigue and bloating were less frequent and were mainly reported by patients older than 40. […] The commonest presenting features of FAP are alteration of bowel habit and rectal bleeding, but both are found in many other conditions. Patients with these findings need immediate endoscopy to allow prompt diagnosis and prophylactic surgery. […] The diagnosis of FAP was established clinically if more than 100 polyps were detected endoscopically in the colorectum.

• #23 Age and manifestation related symptoms in familial adenomatous polyposis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1079798/

  FAP was diagnosed clinically on the basis of symptoms in 84% (120/143) of these patients. Most presented with intestinal symptoms such as colonic bleeding (68%) and diarrhea (42%). All but one of the patients between 20 and 40 years old had rectal polyps (98.7%, 75/76), whereas in those over 40 years old the prevalence was 76% (35/46). Non-specific symptoms such as abdominal pain, fatigue and bloating were less frequent and were mainly reported by patients older than 40. […] The commonest presenting features of FAP are alteration of bowel habit and rectal bleeding, but both are found in many other conditions. Patients with these findings need immediate endoscopy to allow prompt diagnosis and prophylactic surgery. […] Untreated, the disease usually leads to the appearance of hundreds of adenomatous polyps in the colorectum between puberty and age 20 and to cancer by the early forties at the latest which is the most frequent reason for death in patients with FAP.

• #24 Age and manifestation related symptoms in familial adenomatous polyposis | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/1471-2407-5-24

  FAP was diagnosed clinically on the basis of symptoms in 84% (120/143) of these patients. Most presented with intestinal symptoms such as colonic bleeding (68%) and diarrhea (42%). All but one of the patients between 20 and 40 years old had rectal polyps (98.7%, 75/76), whereas in those over 40 years old the prevalence was 76% (35/46). Non-specific symptoms such as abdominal pain, fatigue and bloating were less frequent and were mainly reported by patients older than 40. […] The commonest presenting features of FAP are alteration of bowel habit and rectal bleeding, but both are found in many other conditions. Patients with these findings need immediate endoscopy to allow prompt diagnosis and prophylactic surgery. […] The diagnosis of FAP was established clinically if more than 100 polyps were detected endoscopically in the colorectum.

• #25 Age and manifestation related symptoms in familial adenomatous polyposis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1079798/

  FAP was diagnosed clinically on the basis of symptoms in 84% (120/143) of these patients. Most presented with intestinal symptoms such as colonic bleeding (68%) and diarrhea (42%). All but one of the patients between 20 and 40 years old had rectal polyps (98.7%, 75/76), whereas in those over 40 years old the prevalence was 76% (35/46). Non-specific symptoms such as abdominal pain, fatigue and bloating were less frequent and were mainly reported by patients older than 40. […] The commonest presenting features of FAP are alteration of bowel habit and rectal bleeding, but both are found in many other conditions. Patients with these findings need immediate endoscopy to allow prompt diagnosis and prophylactic surgery. […] Untreated, the disease usually leads to the appearance of hundreds of adenomatous polyps in the colorectum between puberty and age 20 and to cancer by the early forties at the latest which is the most frequent reason for death in patients with FAP.

• #26 Age and manifestation related symptoms in familial adenomatous polyposis | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/1471-2407-5-24

  The predominant symptoms of FAP were analyzed and documented retrospectively in all 143 cases. […] Colonic bleeding as an early symptom was present in 52% (74/143). This was found in 68% (52/76) of patients between 20 and 40 years old and in 35% (16/46) of patients older than 40 years. […] Diarrhea was reported in 31% (45/143). 42% (32/76) of patients between 20 and 40 years but only in 24% (11/46) older than 40 years suffered from this symptom. […] The incidence of colonic carcinoma was 42-50% in this age group. We conclude that non-specific symptoms can imply a pronounced manifestation of FAP which usually affects patients beyond 40 years of age.

• #27 Familial adenomatous polyposis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/familial-adenomatous-polyposis/symptoms-causes/syc-20372443

  The main sign of FAP is hundreds or even thousands of polyps growing in your colon and rectum, usually starting by your mid-teens. The polyps are nearly 100 percent certain to develop into colon cancer or rectal cancer by the time you’re in your 40s. […] Most people with familial adenomatous polyposis eventually need surgery to remove the large intestine to prevent cancer. The polyps in the duodenum also can develop cancer, but they can usually be managed by careful monitoring and by removing polyps regularly. […] Some people have a milder form of the condition, called attenuated familial adenomatous polyposis (AFAP). People with AFAP usually have fewer colon polyps (an average of 30) and develop cancer later in life.

• #28 Familial adenomatous polyposis: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/familial-adenomatous-polyposis/

  A milder type of familial adenomatous polyposis, called autosomal recessive familial adenomatous polyposis, has also been identified. People with the autosomal recessive type of this disorder have fewer polyps than those with the classic type. Fewer than 100 polyps typically develop, rather than hundreds or thousands. The autosomal recessive type of this disorder is caused by mutations in a different gene than the classic and attenuated types of familial adenomatous polyposis.

• #29 Familial adenomatous polyposis – Wikipedia

  https://en.wikipedia.org/wiki/Familial_adenomatous_polyposis

  Because familial polyposis develops very gradually over years, and can also manifest in an 'attenuated’ form even more gradually, polyps resulting from FAP can lead to cancer developing at any point from adolescence to old age. […] The incidence of the mutation is between 1 in 10,000 and 1 in 15,000 births. By age 35 years, 95% of individuals with FAP (100 adenomas) have polyps. Without colectomy, colon cancer is virtually inevitable. The mean age of colon cancer in untreated individuals is 39 years (range 34-43 years). […] Attenuated FAP arises when APC is defective but still somewhat functional. As a result, it retains part of its ability to suppress polyps. Therefore, attenuated FAP manifests as colorectal cancer unusually late (age 40-70, average=55), and typically with few, or at least far fewer polyps (typically 30), than the more usual version of FAP, at an age when FAP is no longer considered much of a likelihood or risk according to usual FAP epidemiology.

• #30 Gastrointestinal Polyposis Syndromes > Fact Sheets > Yale Medicine

  https://www.yalemedicine.org/conditions/gastrointestinal-polyposis-syndromes

  Symptoms include rectal bleeding, blood in stool, weight loss, nausea and/or vomiting, abdominal pain […] The polyposis syndromes often cause symptoms in other parts of the body, as noted above. […] Classic FAP is characterized by the presence of numerousoften hundreds or thousandsof polyps in the colon and rectum. The polyps typically begin to appear in childhood or adolescence, usually by age 16, and more polyps develop as people age. Without surgical treatment, the likelihood of developing colorectal cancer is nearly 100% for people with classic FAP. On average, people with classic FAP who remain untreated are diagnosed with colorectal cancer at age 39. […] In AFAP, the lifetime risk of developing colorectal cancer is lower (though still high, at around 80%), and people tend to have fewer colorectal polyps (10 to 100) that typically develop at a later age (mid-to-late 20s). If colorectal cancer develops, it is usually diagnosed at a later ageat age 58, on averagethan in those with classic FAP.

• #31 Familial Adenomatous Polyposis | Cancer Institute NSW

  https://www.cancer.nsw.gov.au/what-we-do/supporting-cancer-care/hereditary-cancers/familial-adenomatous-polyposis

  Following surgery, a sigmoidoscopy (a procedure in which a long flexible tube is used to view the remaining colon and or/rectum) should be performed every 12 months. […] If a close relative is diagnosed with Familial Adenomatous Polyposis you may be at risk of developing FAP. People at risk of FAP should have a colonoscopy every year starting from age 10-15 years. If no polyps have been found by age 15, then screening may be reduced to every 2 years. […] Attenuated FAP involves fewer polyps generally less than 100 and people with attenuated FAP will typically develop polyps and/or cancer later than people with classic FAP. If you are diagnosed with this condition, you are still strongly predisposed to colorectal cancer.

• #32 Familial adenomatous polyposis syndromes – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-gb/652

  Familial adenomatous polyposis (FAP) syndrome is a hereditary colorectal cancer syndrome characterised by the development of hundreds to thousands of colorectal adenomas. The attenuated form leads to the formation of fewer than 100 polyps. […] Median age of onset for polyps is 15 years. […] In the absence of total colectomy or endoscopic polyp clearance, colorectal cancer arises in close to 100% of patients with FAP by 40 years of age and in 80% of patients with attenuated FAP by 60 years of age. […] Patients with classical FAP have hundreds to thousands of colorectal adenomas and a nearly 100% risk for colorectal cancer by the age of 40 years if proctoprophylactic colectomy is not performed. […] Attenuated FAP is also caused by APC mutations and is characterised by fewer than 100 adenomas and a later age of colorectal cancer onset.

• #33 Gastrointestinal Polyposis Syndromes > Fact Sheets > Yale Medicine

  https://www.yalemedicine.org/conditions/gastrointestinal-polyposis-syndromes

  Symptoms include rectal bleeding, blood in stool, weight loss, nausea and/or vomiting, abdominal pain […] The polyposis syndromes often cause symptoms in other parts of the body, as noted above. […] Classic FAP is characterized by the presence of numerousoften hundreds or thousandsof polyps in the colon and rectum. The polyps typically begin to appear in childhood or adolescence, usually by age 16, and more polyps develop as people age. Without surgical treatment, the likelihood of developing colorectal cancer is nearly 100% for people with classic FAP. On average, people with classic FAP who remain untreated are diagnosed with colorectal cancer at age 39. […] In AFAP, the lifetime risk of developing colorectal cancer is lower (though still high, at around 80%), and people tend to have fewer colorectal polyps (10 to 100) that typically develop at a later age (mid-to-late 20s). If colorectal cancer develops, it is usually diagnosed at a later ageat age 58, on averagethan in those with classic FAP.

• #34 Pathology Outlines – Familial adenomatous polyposis, attenuated

  https://www.pathologyoutlines.com/topic/colontumorafap.html

  Subtype of familial adenomatous polyposis (FAP) characterized by fewer than 100 adenomatous colorectal polyps […] Colonic adenomatous polyps have high risk for progression to colorectal adenocarcinoma (69% cumulative risk by age 80) (Gastroenterology 2004;127:444) […] FAP variant characterized by markedly fewer polyps […] Patients might not require colectomy […] Cancers usually develop at age 50 – 55 years, 15 years later than classic FAP […] Patients may develop gastric fundic gland polyps, gastric or small bowel flat adenomas and gastric / duodenal carcinomas (Cancer 1993;71:2709) […] Classically reserved for patients with fewer than 100 colonic adenomatous polyps but exact diagnostic criteria have not been firmly established […] Patients with fewer than 20 – 30 polyps may not require total colectomy if they have frequent surveillance colonscopies with polypectomies […] Polyps are usually more proximal (i.e. right sided) than in classic FAP […] Adenomatous polyps are microscopically similar to sporadic type adenomas […] Associated with pathogenic variants in the 5′ and distal 3′ end of APC, as well as interstitial deletions of 5q22, which include the APC gene.

• #35 Colorectal Cancer Risk Factors | Hereditary Colorectal Risk Factors | American Cancer Society

  https://www.cancer.org/cancer/types/colon-rectal-cancer/causes-risks-prevention/risk-factors.html

  FAP is caused by changes (mutations) in the APC gene that a person inherits from their parents. About 1% of all colorectal cancers are caused by FAP. […] In the most common type of FAP, hundreds or thousands of polyps develop in a persons colon and rectum, often starting at ages 10 to 12. Cancer usually develops in 1 or more of these polyps as early as age 20. By age 40, almost all people with FAP will have colon cancer if their colon hasnt been removed to prevent it. People with FAP also have an increased risk for cancers of the stomach, small intestines, pancreas, liver, and some other organs. […] There are 3 sub-types of FAP: In attenuated FAP or AFAP, patients have fewer polyps (less than 100), and colorectal cancer tends to occur at a later age (40s and 50s). Gardner syndrome is a type of FAP that also causes noncancerous tumors of the skin, soft tissue, and bones. In Turcot syndrome, people who have APC gene mutation are at a high risk of having many adenomatous polyps and colorectal cancer, but also a specific type of brain cancer called medulloblastoma.

• #36 Familial Adenomatous Polyposis (FAP): Symptoms, Diagnosis, Risks

  https://my.clevelandclinic.org/health/diseases/16993-familial-adenomatous-polyposis-fap

  Familial adenomatous polyposis (FAP) is a hereditary syndrome that raises your risk of developing colorectal cancer to nearly 100%. Cancer develops from adenomatous colon polyps, which can appear in the hundreds to thousands. To manage this risk, most people with FAP will have their whole colon removed sometime in their early life. […] Many people get a few colon polyps at random as they get older. But if you have a hereditary polyposis syndrome like FAP, you’ll develop many colon polyps typically over a hundred starting from a young age. This significantly raises your lifetime risk that one of them will become cancerous. […] Without treatment, the risk of developing colorectal cancer with familial adenomatous polyposis is close to 100%. It also develops relatively earlier and faster with FAP than in those without.

• #37 Familial Adenomatous Polyposis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538233/

  Familial adenomatous polyposis is an autosomal dominant polyposis syndrome characterized by mutations in the adenomatous polyposis coli gene. Patients with familial adenomatous polyposis develop hundreds to thousands of polyps throughout the colon and rectum, significantly elevating their lifetime risk of colorectal cancer if left untreated. […] If left untreated, affected individuals develop hundreds to thousands of polyps throughout the colon and rectum, often manifesting in the early teenage years. This condition ultimately results in an almost 100% lifetime risk of colorectal cancer, typically occurring by age 40. Colectomy is recommended to significantly reduce the risk of developing colorectal cancer. Individuals with FAP face an elevated risk of other malignancies, such as gastric and duodenal adenocarcinoma, hepatoblastoma, and desmoid tumors.

• #38 Age and manifestation related symptoms in familial adenomatous polyposis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1079798/

  FAP was diagnosed clinically on the basis of symptoms in 84% (120/143) of these patients. Most presented with intestinal symptoms such as colonic bleeding (68%) and diarrhea (42%). All but one of the patients between 20 and 40 years old had rectal polyps (98.7%, 75/76), whereas in those over 40 years old the prevalence was 76% (35/46). Non-specific symptoms such as abdominal pain, fatigue and bloating were less frequent and were mainly reported by patients older than 40. […] The commonest presenting features of FAP are alteration of bowel habit and rectal bleeding, but both are found in many other conditions. Patients with these findings need immediate endoscopy to allow prompt diagnosis and prophylactic surgery. […] Untreated, the disease usually leads to the appearance of hundreds of adenomatous polyps in the colorectum between puberty and age 20 and to cancer by the early forties at the latest which is the most frequent reason for death in patients with FAP.

• #39 Familial Adenomatous Polyposis: Symptoms, Causes, Treatment

  https://www.healthline.com/health/colorectal-cancer/familial-adenomatous-polyposis

  Familial adenomatous polyposis (FAP) is a rare condition that predisposes you (makes you more susceptible) to precancerous colorectal adenomatous polyps. […] FAP usually requires the removal of part or all of your large intestines. If left treated, it almost always leads to colorectal cancer at a relatively young age. […] People with FAP develop hundreds to thousands of these polyps along their rectum and colon. […] Polyps develop between the ages of 7 to 36, and the average age of development is 16. If left unrecognized and untreated, they almost always turn into colorectal cancer by your 30s or 40s. […] The primary sign of FAP is the development of hundreds to thousands of polyps. Common symptoms can include abdominal pain, diarrhea, blood in stools, and dark, sticky feces. […] People with FAP almost always develop colon cancer in their 30s or 40s if they don’t receive surgery to remove part or all of their colon.

• #40 Familial Adenomatous Polyposis: Symptoms, Causes, Treatment

  https://www.healthline.com/health/colorectal-cancer/familial-adenomatous-polyposis

  Without a colectomy, people with FAP usually develop colorectal cancer in their 30s or 40s, and the average age of development is 39 years. The average age of death in people without surgery is 42 years. […] Life expectancy is significantly higher in people who receive a colectomy. Regular screening is also associated with better outcomes.

• #41 Familial Adenomatous Polyposis: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/175377-overview

  Familial adenomatous polyposis (FAP) is the most common adenomatous polyposis syndrome. It is an autosomal dominant inherited disorder characterized by the early onset of hundreds to thousands of adenomatous polyps throughout the colon. If left untreated, all patients with this syndrome will develop colon cancer by age 35-40 years. In addition, an increased risk exists for the development of other malignancies. […] The average age of onset of polyposis in FAP is 16 years, whereas the average age of onset for colorectal cancer is 39 years. […] Patients with untreated FAP have a median life expectancy of 42 years. Life expectancy is extended greatly in those treated with colectomy. […] The principal cause of mortality is colorectal cancer, which develops in all patients unless they are treated. The mean age at which colorectal cancer develops in patients with classic FAP is 39 years. Patients with adenomatous polyposis itself often are asymptomatic. […] Complications of FAP include the following: Colorectal cancer (100% in untreated patients).

• #42 Familial Adenomatous Polyposis (FAP) | Cancer Genetics Web

  http://www.cancerindex.org/geneweb/FAP.html

  Familial Adenomatous Polyposis (FAP) is a genetic condition characterized by multiple (100) adenomatous polyps in the colon and rectum typically developing after the first decade of life. […] Individuals with FAP have an increased risk of colorectal cancer and other tumours. […] People who inherit a mutant APC gene have over 90% likelihood of developing colonic adenomas. Age at onset of adenomas in the colon varies: 15% by age 10, 75% by age 20, and 90% by 30. Without intervention, most individuals with FAP will develop colon or rectal cancer by the fourth decade of life. […] The rate of polyposis progression in young patients with FAP varies with a median of about 25 new polyps per year. Progression is associated with distinct factors, which can be used in discussion with patients regarding the need for and timing of prophylactic colorectal surgery.

• #43 Bowel (Colonic) Polyps | Doctor

  https://patient.info/doctor/bowel-colonic-polyps-pro

  Patients are often asymptomatic but may present with rectal bleeding, diarrhoea, abdominal pain and mucous discharge. […] Obstruction may cause constipation, vomiting and peritonitis. […] The mean age of colon cancer diagnosis in untreated individuals is 35-40 years. […] Patients with more than 1,000 polyps have been proven to have 2.3 times the cancer risk compared to patients with fewer than 1,000 polyps. […] However, colorectal cancer will develop in all individuals with familial polyposis unless prophylactic colectomy is performed. After total colectomy with ileo-rectal anastomosis, the recurrence rate is 30% after 20 years and 45% after 30 years.

• #44 Familial Adenomatous Polyposis: Symptoms, Causes, Treatment

  https://www.healthline.com/health/colorectal-cancer/familial-adenomatous-polyposis

  Without a colectomy, people with FAP usually develop colorectal cancer in their 30s or 40s, and the average age of development is 39 years. The average age of death in people without surgery is 42 years. […] Life expectancy is significantly higher in people who receive a colectomy. Regular screening is also associated with better outcomes.

• #45 Familial Adenomatous Polyposis: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/175377-overview

  Familial adenomatous polyposis (FAP) is the most common adenomatous polyposis syndrome. It is an autosomal dominant inherited disorder characterized by the early onset of hundreds to thousands of adenomatous polyps throughout the colon. If left untreated, all patients with this syndrome will develop colon cancer by age 35-40 years. In addition, an increased risk exists for the development of other malignancies. […] The average age of onset of polyposis in FAP is 16 years, whereas the average age of onset for colorectal cancer is 39 years. […] Patients with untreated FAP have a median life expectancy of 42 years. Life expectancy is extended greatly in those treated with colectomy. […] The principal cause of mortality is colorectal cancer, which develops in all patients unless they are treated. The mean age at which colorectal cancer develops in patients with classic FAP is 39 years. Patients with adenomatous polyposis itself often are asymptomatic. […] Complications of FAP include the following: Colorectal cancer (100% in untreated patients).

• #46 Familial Adenomatous Polyposis Syndrome – DoveMed

  https://www.dovemed.com/diseases-conditions/familial-adenomatous-polyposis-syndrome

  The greater the number of polyps in the large intestine, the greater is the risk for developing colon cancer later in life. […] Adenomatous polyps can develop into colon cancer with additional complications. […] The prognosis of Familial Adenomatous Polyposis Syndrome depends on how early it is diagnosed and medical interventions instituted. […] With no medical intervention, the median life expectancy of an individual with Familial Adenomatous Polyposis Syndrome is about 42 years. […] Prophylactic colectomy is known to increase the life expectancy.

• #47 Familial Adenomatous Polyposis: Alarming Symptoms, Causes, Types, Diagnosis and Treatment – OncoDaily

  https://oncodaily.com/oncolibrary/cancer-types/familial-adenomatous-polyposis

  If left untreated, nearly all cases of FAP progress to colorectal cancer, making early detection and management critical. […] Without intervention, FAP almost inevitably progresses to cancer, making timely management essential. […] According to recent studies, familial adenomatous polyposis (FAP) patients who reach this stage without surgical intervention have a 75-90% chance of developing colorectal cancer. […] If left untreated, FAP has a nearly 100% risk of progressing to colorectal cancer by the age of 35-40. Untreated polyps can become malignant over time, leading to life-threatening complications.

• #48 Familial Adenomatous Polyposis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538233/

  The majority of patients typically present with nonspecific symptoms such as diarrhea, abdominal discomfort, and rectal bleeding. Increasingly, patients are identified through screening colonoscopy. Colonoscopic examinations reveal numerous colonic polyps, often exceeding 100, although the number may be lower when screenings start in childhood and are distributed throughout the colon. Attenuated FAP typically involves a reduced number of polyps emerging later in life, predominantly impacting the right colon. This variant normally features approximately 30 polyps and manifests at an average age of over 50 years. Symptoms and signs are related to the degree of polyposis and cancer stage. […] If left untreated, FAP progresses to colorectal cancer in nearly 100% of cases, leading to a shortened life expectancy, with most patients dying by their fourth decade of life. However, survival rates are significantly improved with enhanced screening, surveillance, and prophylactic colectomy.

• #49 Familial Adenomatous Polyposis Syndrome – DoveMed

  https://www.dovemed.com/diseases-conditions/familial-adenomatous-polyposis-syndrome

  The greater the number of polyps in the large intestine, the greater is the risk for developing colon cancer later in life. […] Adenomatous polyps can develop into colon cancer with additional complications. […] The prognosis of Familial Adenomatous Polyposis Syndrome depends on how early it is diagnosed and medical interventions instituted. […] With no medical intervention, the median life expectancy of an individual with Familial Adenomatous Polyposis Syndrome is about 42 years. […] Prophylactic colectomy is known to increase the life expectancy.

• #50 Familial Adenomatous Polyposis: Symptoms, Causes, Treatment

  https://www.healthline.com/health/colorectal-cancer/familial-adenomatous-polyposis

  Without a colectomy, people with FAP usually develop colorectal cancer in their 30s or 40s, and the average age of development is 39 years. The average age of death in people without surgery is 42 years. […] Life expectancy is significantly higher in people who receive a colectomy. Regular screening is also associated with better outcomes.

• #51 Familial Adenomatous Polyposis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538233/

  The majority of patients typically present with nonspecific symptoms such as diarrhea, abdominal discomfort, and rectal bleeding. Increasingly, patients are identified through screening colonoscopy. Colonoscopic examinations reveal numerous colonic polyps, often exceeding 100, although the number may be lower when screenings start in childhood and are distributed throughout the colon. Attenuated FAP typically involves a reduced number of polyps emerging later in life, predominantly impacting the right colon. This variant normally features approximately 30 polyps and manifests at an average age of over 50 years. Symptoms and signs are related to the degree of polyposis and cancer stage. […] If left untreated, FAP progresses to colorectal cancer in nearly 100% of cases, leading to a shortened life expectancy, with most patients dying by their fourth decade of life. However, survival rates are significantly improved with enhanced screening, surveillance, and prophylactic colectomy.

• #52 Familial adenomatous polyposis: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/familial-adenomatous-polyposis/

  Familial adenomatous polyposis (FAP) is an inherited disorder characterized by cancer of the large intestine (colon) and rectum. People with the classic type of familial adenomatous polyposis may begin to develop multiple noncancerous (benign) growths (polyps) in the colon as early as their teenage years. Unless the colon is removed, these polyps will become malignant (cancerous). The average age at which an individual develops colon cancer in classic familial adenomatous polyposis is 39 years. Some people have a variant of the disorder, called attenuated familial adenomatous polyposis, in which polyp growth is delayed. The average age of colorectal cancer onset for attenuated familial adenomatous polyposis is 55 years. […] In people with classic familial adenomatous polyposis, the number of polyps increases with age, and hundreds to thousands of polyps can develop in the colon. Also of particular significance are noncancerous growths called desmoid tumors. These fibrous tumors usually occur in the tissue covering the intestines and may be provoked by surgery to remove the colon. Desmoid tumors tend to recur after they are surgically removed. In both classic familial adenomatous polyposis and its attenuated variant, benign and malignant tumors are sometimes found in other places in the body, including the duodenum (a section of the small intestine), stomach, bones, skin, and other tissues. People who have colon polyps as well as growths outside the colon are sometimes described as having Gardner syndrome.

• #53 Familial Adenomatous Polyposis (FAP) | UCSF Department of Surgery

  https://colorectalsurgery.ucsf.edu/condition/familial-adenomatous-polyposis-fap

  In both classic familial adenomatous polyposis and its attenuated variant, benign and malignant tumors are sometimes found in other places in the body, including the duodenum (a section of the small intestine), stomach, bones, skin, and other tissues. […] A milder type of familial adenomatous polyposis, called autosomal recessive familial adenomatous polyposis, has also been identified. People with the autosomal recessive type of this disorder have fewer polyps than those with the classic type. Fewer than 100 polyps typically develop, rather than hundreds or thousands.

• #54 Familial Adenomatous Polyposis – MD Searchlight

  https://mdsearchlight.com/genetic-disorders/familial-adenomatous-polyposis/

  The majority of FAP patients, 90%, show gastric polyps, which usually do not become cancerous. Non-fundic gland polyps, however, are more frequently cancerous and require endoscopic management. High-grade dysplasia in any gastric polyp necessitates endoscopic or surgical resection based on guidelines. […] Periampullary and duodenal cancers are the leading causes of death in FAP patients after colon cancer. The lifetime risk of duodenal cancer is about 5% by age 60. Guidelines recommend starting surveillance at age 20 to 25 and repeating it every 2 to 3 years. Management depends on the polyps pathology and location. […] If Familial Adenomatous Polyposis (FAP) is not treated, it can almost always progress to colorectal cancer, which is a serious type of cancer affecting the large intestine. This can result in shorter lifespans, with the majority of patients not living past their 40s. However, with proper screening and preventive measures, the survival rates can be significantly improved. One of these preventive measures might be a colectomy, a surgery to remove part or all of your colon.

• #55 Managing familial adenomatous polyposis (FAP): new approaches | MD Anderson Cancer Center

  https://www.mdanderson.org/cancerwise/new-approaches-to-managing-familial-adenomatous-polyposis–fap.h00-159537378.html

  If FAP is confirmed, the patient will need a colonoscopy every year or sometimes more frequently. Patients may also receive an upper endoscopy to check for polyps in the upper gastrointestinal tract as well as imaging to check for desmoid tumors in the abdomen. […] As a person with FAP ages, the number of polyps increases. By age 30, if not sooner, the colon should be removed through a surgery known as a colectomy. […] With the colon removed, the risk of colorectal cancer becomes almost none. This effectively prevents FAP patients from dying of colorectal cancer, Vilar-Sanchez says. […] Even when the colon is removed, polyps can still form in the remaining gastrointestinal tissue. Many patients later develop cancer in their duodenum, which is the first part of the small intestine. […] The problem is that its a lifelong intervention, and it stops working after some time, Vilar-Sanchez says. […] Along with his team, hes investigating if obeticholic acid can serve as a chemoprevention therapy to slow or halt the development of polyps in the duodenum of patients with FAP who have received a colectomy.

• #56 Familial Adenomatous Polyposis – MD Searchlight

  https://mdsearchlight.com/genetic-disorders/familial-adenomatous-polyposis/

  The majority of FAP patients, 90%, show gastric polyps, which usually do not become cancerous. Non-fundic gland polyps, however, are more frequently cancerous and require endoscopic management. High-grade dysplasia in any gastric polyp necessitates endoscopic or surgical resection based on guidelines. […] Periampullary and duodenal cancers are the leading causes of death in FAP patients after colon cancer. The lifetime risk of duodenal cancer is about 5% by age 60. Guidelines recommend starting surveillance at age 20 to 25 and repeating it every 2 to 3 years. Management depends on the polyps pathology and location. […] If Familial Adenomatous Polyposis (FAP) is not treated, it can almost always progress to colorectal cancer, which is a serious type of cancer affecting the large intestine. This can result in shorter lifespans, with the majority of patients not living past their 40s. However, with proper screening and preventive measures, the survival rates can be significantly improved. One of these preventive measures might be a colectomy, a surgery to remove part or all of your colon.

• #57 Familial adenomatous polyposis (FAP) – Treatment abroad

  https://www.gsdinternational.com/conditions/familial-adenomatous-polyposis-fap

  Familial Adenomatous Polyposis (FAP) is an autosomal dominant disease characterized by hundreds of colorectal adenomatous polyps. These initially benign polyps may progress to one or more carcinomas over 7-12 years. […] The main feature is the development of hundreds of colorectal polyps. Equally characteristic is polyposis of the duodenum and sometimes the stomach, also with malignant potential. […] The risk of colorectal cancer is nearly 100% before age 50. […] The risk of colorectal cancer is almost 70%. […] At the duodenal level, adenomas have an incidence of more than 90% and a median age of presentation of 52 years.

• #58 Familial Adenomatous Polyposis – MD Searchlight

  https://mdsearchlight.com/genetic-disorders/familial-adenomatous-polyposis/

  The majority of FAP patients, 90%, show gastric polyps, which usually do not become cancerous. Non-fundic gland polyps, however, are more frequently cancerous and require endoscopic management. High-grade dysplasia in any gastric polyp necessitates endoscopic or surgical resection based on guidelines. […] Periampullary and duodenal cancers are the leading causes of death in FAP patients after colon cancer. The lifetime risk of duodenal cancer is about 5% by age 60. Guidelines recommend starting surveillance at age 20 to 25 and repeating it every 2 to 3 years. Management depends on the polyps pathology and location. […] If Familial Adenomatous Polyposis (FAP) is not treated, it can almost always progress to colorectal cancer, which is a serious type of cancer affecting the large intestine. This can result in shorter lifespans, with the majority of patients not living past their 40s. However, with proper screening and preventive measures, the survival rates can be significantly improved. One of these preventive measures might be a colectomy, a surgery to remove part or all of your colon.

• #59 Familial Adenomatous Polyposis – MD Searchlight

  https://mdsearchlight.com/genetic-disorders/familial-adenomatous-polyposis/

  FAP may also affect other parts of the body. Specific to FAP, the retinas can thicken and result in localized, pigmented retinal lesions usually without symptoms. An eye exam may reveal these changes. FAP may also cause dental abnormalities, such as supernumerary or impacted teeth, odontomas, and cysts. Lastly, bone growths known as osteomas can occur in areas like the jaw or skull. […] Another manifestation of FAP is the development of desmoid tumors. These benign growths are often large and do not spread to other areas but can grow aggressively and invade local structures. They occur in about 10-15% of FAP patients, most commonly in the abdomen. If there is a family history of these tumors, the risk shoots up to 25%. The likelihood of developing these tumors is about twice as high in women than men and can be related to surgical trauma. Therefore, surgery for FAP is often delayed in young patients. Monitoring these tumors typically involves annual abdominal exams or imaging studies. If necessary, these tumors can be treated with medication or removed surgically.

• #60 Familial Adenomatous Polyposis – MD Searchlight

  https://mdsearchlight.com/genetic-disorders/familial-adenomatous-polyposis/

  FAP may also affect other parts of the body. Specific to FAP, the retinas can thicken and result in localized, pigmented retinal lesions usually without symptoms. An eye exam may reveal these changes. FAP may also cause dental abnormalities, such as supernumerary or impacted teeth, odontomas, and cysts. Lastly, bone growths known as osteomas can occur in areas like the jaw or skull. […] Another manifestation of FAP is the development of desmoid tumors. These benign growths are often large and do not spread to other areas but can grow aggressively and invade local structures. They occur in about 10-15% of FAP patients, most commonly in the abdomen. If there is a family history of these tumors, the risk shoots up to 25%. The likelihood of developing these tumors is about twice as high in women than men and can be related to surgical trauma. Therefore, surgery for FAP is often delayed in young patients. Monitoring these tumors typically involves annual abdominal exams or imaging studies. If necessary, these tumors can be treated with medication or removed surgically.

• #61 Familial adenomatous polyposis: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/familial-adenomatous-polyposis/

  Familial adenomatous polyposis (FAP) is an inherited disorder characterized by cancer of the large intestine (colon) and rectum. People with the classic type of familial adenomatous polyposis may begin to develop multiple noncancerous (benign) growths (polyps) in the colon as early as their teenage years. Unless the colon is removed, these polyps will become malignant (cancerous). The average age at which an individual develops colon cancer in classic familial adenomatous polyposis is 39 years. Some people have a variant of the disorder, called attenuated familial adenomatous polyposis, in which polyp growth is delayed. The average age of colorectal cancer onset for attenuated familial adenomatous polyposis is 55 years. […] In people with classic familial adenomatous polyposis, the number of polyps increases with age, and hundreds to thousands of polyps can develop in the colon. Also of particular significance are noncancerous growths called desmoid tumors. These fibrous tumors usually occur in the tissue covering the intestines and may be provoked by surgery to remove the colon. Desmoid tumors tend to recur after they are surgically removed. In both classic familial adenomatous polyposis and its attenuated variant, benign and malignant tumors are sometimes found in other places in the body, including the duodenum (a section of the small intestine), stomach, bones, skin, and other tissues. People who have colon polyps as well as growths outside the colon are sometimes described as having Gardner syndrome.

• #62 Familial Adenomatous Polyposis | St. Jude Care & Treatment

  https://www.stjude.org/care-treatment/treatment/genetic-syndromes/familial-adenomatous-polyposis.html

  Up to 1 in 8 people with classic FAP, Gardner syndrome, and Turcot syndrome are also at risk for small intestine cancer. Small intestine cancer usually occurs after age 17. The average age of diagnosis is 45-52 years. […] People with attenuated FAP have a slightly lower risk of colon cancer compared to classic FAP. For those with this subtype who have not had their colon removed, the average age of colon cancer diagnosis is 50-55. […] Other features of FAP include: Polyps (growths) in the colon and rectum, Blood in stool, Teeth that are extra, missing or do not emerge from the gums, Dark patches on retina of the eye present from birth, Skin cysts or tags, Bony growths on the legs, arms, or head, Non-cancerous soft tissue tumors in the abdomen, Non-cancerous tumors of the adrenal glands, Rarely, a type of liver cancer called hepatoblastoma.

• #63 Familial Adenomatous Polyposis – MD Searchlight

  https://mdsearchlight.com/genetic-disorders/familial-adenomatous-polyposis/

  FAP may also affect other parts of the body. Specific to FAP, the retinas can thicken and result in localized, pigmented retinal lesions usually without symptoms. An eye exam may reveal these changes. FAP may also cause dental abnormalities, such as supernumerary or impacted teeth, odontomas, and cysts. Lastly, bone growths known as osteomas can occur in areas like the jaw or skull. […] Another manifestation of FAP is the development of desmoid tumors. These benign growths are often large and do not spread to other areas but can grow aggressively and invade local structures. They occur in about 10-15% of FAP patients, most commonly in the abdomen. If there is a family history of these tumors, the risk shoots up to 25%. The likelihood of developing these tumors is about twice as high in women than men and can be related to surgical trauma. Therefore, surgery for FAP is often delayed in young patients. Monitoring these tumors typically involves annual abdominal exams or imaging studies. If necessary, these tumors can be treated with medication or removed surgically.

• #64 Familial adenomatous polyposis syndromes – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-gb/652

  The median age of onset for polyps is 15 years. […] Individuals with FAP also have an increased lifetime risk for other cancers, including duodenal/periampullary cancer (1% to 10%), thyroid cancer (1.2% to 12%), gastric cancer (0.1% to 7.1%), and hepatoblastoma (0.4% to 2.5%, usually by age 5 years).

• #65 Familial Adenomatous Polyposis – MD Searchlight

  https://mdsearchlight.com/genetic-disorders/familial-adenomatous-polyposis/

  Some complications linked with known FAP conditions are: All patients (100%) develop colon and rectum cancer. About 10% of patients develop cancers involving the beginning of the small intestine or the ampulla of Vater (a small opening that drains pancreatic and bile ducts into the small intestine). Up to 20% of patients develop benign but potentially life-threatening tumors called desmoid tumors. Some patients might also develop additional cancer types like liver, brain, and thyroid cancers.

• #66 Familial adenomatous polyposis syndromes – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-gb/652

  The median age of onset for polyps is 15 years. […] Individuals with FAP also have an increased lifetime risk for other cancers, including duodenal/periampullary cancer (1% to 10%), thyroid cancer (1.2% to 12%), gastric cancer (0.1% to 7.1%), and hepatoblastoma (0.4% to 2.5%, usually by age 5 years).

• #67 Familial Adenomatous Polyposis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538233/

  Familial adenomatous polyposis is an autosomal dominant polyposis syndrome characterized by mutations in the adenomatous polyposis coli gene. Patients with familial adenomatous polyposis develop hundreds to thousands of polyps throughout the colon and rectum, significantly elevating their lifetime risk of colorectal cancer if left untreated. […] If left untreated, affected individuals develop hundreds to thousands of polyps throughout the colon and rectum, often manifesting in the early teenage years. This condition ultimately results in an almost 100% lifetime risk of colorectal cancer, typically occurring by age 40. Colectomy is recommended to significantly reduce the risk of developing colorectal cancer. Individuals with FAP face an elevated risk of other malignancies, such as gastric and duodenal adenocarcinoma, hepatoblastoma, and desmoid tumors.

• #68 Familial Adenomatous Polyposis (FAP): Symptoms, Diagnosis, Risks

  https://my.clevelandclinic.org/health/diseases/16993-familial-adenomatous-polyposis-fap

  Colon polyps in FAP begin to appear much earlier than in the general population, often during the teenage years. They usually won’t cause symptoms until they’ve grown large enough to be dangerous. But if you have a colonoscopy for screening purposes, your healthcare provider will find them. […] People with FAP often have hundreds or thousands of colon polyps. Those with AFAP have at least 20. The greater number and faster growth of polyps in FAP may make them more likely to cause symptoms than polyps usually are. Symptoms may include rectal bleeding, diarrhea or chronic abdominal pain. […] Colon polyps in FAP begin to appear at the average age of 16. […] If you’re diagnosed with FAP, you can expect a lifetime of medical screenings and possibly multiple surgeries to remove tumors. Tumors outside of your colon are less likely to become cancerous than colon polyps. Desmoid tumors, which aren’t cancerous, can be a minor irritation or a major one. […] You can expect to have a total colectomy sometime in your early life.

• #69 Familial adenomatous polyposis (FAP) – diagnosis, surgery | Macmillan Cancer Support

  https://www.macmillan.org.uk/cancer-information-and-support/worried-about-cancer/causes-and-risk-factors/familial-adenomatous-polyposis-fap

  FAP causes hundreds or thousands of small growths in the large bowel. These are called polyps or adenomas. They usually start to appear when a person is in their teens. […] If the polyps are not treated, 1 or more of them will almost certainly develop into cancer. This usually happens by the age of 40. To prevent this, many people affected by FAP decide to have surgery to remove the large bowel. […] Without surgery to remove the large bowel, most people affected by FAP will develop bowel cancer. Your doctor will talk to you about your risk and the best age for you to have surgery. […] The thought of surgery may be stressful or difficult to cope with. Although surgery is very effective at preventing bowel cancer, there are risks and side effects. […] After the operation, you will be able to go to the toilet normally, but will need to go more often. There is still a risk that polyps may develop in the rectum. You will need a test to check this every 6 to 12 months, depending on how many polyps are growing in the rectum.

• #70 Managing familial adenomatous polyposis (FAP): new approaches | MD Anderson Cancer Center

  https://www.mdanderson.org/cancerwise/new-approaches-to-managing-familial-adenomatous-polyposis–fap.h00-159537378.html

  If FAP is confirmed, the patient will need a colonoscopy every year or sometimes more frequently. Patients may also receive an upper endoscopy to check for polyps in the upper gastrointestinal tract as well as imaging to check for desmoid tumors in the abdomen. […] As a person with FAP ages, the number of polyps increases. By age 30, if not sooner, the colon should be removed through a surgery known as a colectomy. […] With the colon removed, the risk of colorectal cancer becomes almost none. This effectively prevents FAP patients from dying of colorectal cancer, Vilar-Sanchez says. […] Even when the colon is removed, polyps can still form in the remaining gastrointestinal tissue. Many patients later develop cancer in their duodenum, which is the first part of the small intestine. […] The problem is that its a lifelong intervention, and it stops working after some time, Vilar-Sanchez says. […] Along with his team, hes investigating if obeticholic acid can serve as a chemoprevention therapy to slow or halt the development of polyps in the duodenum of patients with FAP who have received a colectomy.

• #71 Familial Adenomatous Polyposis: Diagnosis and More

  https://www.verywellhealth.com/familial-adenomatous-polyposis-overview-4582457

  The polyps in FAP are referred to as „adenomatous.” Adenomatous polyps start out as benign but eventually change into being cancerous (malignant). For this reason, they are often referred to as pre-cancerous. These polyps start developing in the person at around 16 years of age. […] If the polyps are not treated by removing part or all of the colon (a procedure called a colectomy), the development of cancer when a person is in their 30s or early 40s is almost inevitable. […] More symptoms of FAP include: Rectal bleeding, or basically any blood that passes through the anus (usually in the form of blood in the stool) […] Surgery doesnt cure the FAP and polyps may still continue to grow. Having FAP means youll have to go for regular medical screenings for the rest of your life. […] The purpose of these regular screenings is to check for new polyps and tumors that may develop into cancerous ones if not monitored and subsequently removed.

• #72 Familial Adenomatous Polyposis | St. Jude Care & Treatment

  https://www.stjude.org/care-treatment/treatment/genetic-syndromes/familial-adenomatous-polyposis.html

  To manage FAP, it is important to get regular screening for polyps. If polyps are found, they are removed. If there are many polyps, the colon may eventually be removed. The goal of screening is to find and treat growths such as polyps or other tumors early for the best outcome. […] People with FAP should watch for signs or symptoms that could signal cancer, such as: Unexplained weight loss, Appetite loss, Pain in the stomach or abdomen, Blood in the stool or changes in bowel habits, Aches, pains, lumps, or swelling for no clear reason, Headaches or changes in vision or nerve function that do not go away. It is important to seek medical help if anything unusual appears.

• #73 Familial Adenomatous Polyposis | Cancer Institute NSW

  https://www.cancer.nsw.gov.au/what-we-do/supporting-cancer-care/hereditary-cancers/familial-adenomatous-polyposis

  Familial Adenomatous Polyposis (FAP) is an inherited bowel cancer syndrome. People with FAP are at high risk of developing bowel cancer much earlier than the general population. […] People with Familial Adenomatous Polyposis develop 100s to 1000s of polyps in the large bowel (intestine) polyps are small mushroom-like growths with stalks that vary in size from a tiny pinhead to 2 cm or more. Most people with Familial Adenomatous Polyposis develop bowel polyps by their late teens, although polyps may develop at any age. Some polyps are likely to become cancerous if left untreated, while others remain benign. […] People diagnosed with Familial Adenomatous Polyposis will require regular check-ups to help reduce the risk of developing bowel cancer. […] For most people with FAP, a colectomy (surgical removal of all or part of the colon) is recommended by the age of 20. If you have not yet had a colectomy, you should have a colonoscopy every year to check for polyps.

• #74 Syndromes

  https://www.nzfgcs.co.nz/Syndromes/Familial-Adenomatous-Polyposis-FAP

  FAP is a rare condition associated with multiple bowel polyps or outgrowths from the lining of the large bowel. […] People with FAP usually develop multiple large bowel polyps before the age of 20 years. […] Without treatment, individuals with FAP will inevitably develop bowel cancer in mid-life. […] Almost all individuals with FAP will develop bowel cancers by their late thirties and forties. […] In FAP, polyps usually start to develop throughout the colon, before the age of 20 years. […] The major concern is that the polyps will become cancerous. […] Most individuals with FAP develop polyps in the first part of the small bowel (duodenum) by the time they are in their seventies but only a small percentage (approximately 5%) develop duodenal cancer. […] Once large bowel polyps are found, individuals with FAP will be advised to have surgery to remove the large bowel. […] Following pouch surgery, regular endoscope checks for polyps are required.

• #75 Managing familial adenomatous polyposis (FAP): new approaches | MD Anderson Cancer Center

  https://www.mdanderson.org/cancerwise/new-approaches-to-managing-familial-adenomatous-polyposis–fap.h00-159537378.html

  If FAP is confirmed, the patient will need a colonoscopy every year or sometimes more frequently. Patients may also receive an upper endoscopy to check for polyps in the upper gastrointestinal tract as well as imaging to check for desmoid tumors in the abdomen. […] As a person with FAP ages, the number of polyps increases. By age 30, if not sooner, the colon should be removed through a surgery known as a colectomy. […] With the colon removed, the risk of colorectal cancer becomes almost none. This effectively prevents FAP patients from dying of colorectal cancer, Vilar-Sanchez says. […] Even when the colon is removed, polyps can still form in the remaining gastrointestinal tissue. Many patients later develop cancer in their duodenum, which is the first part of the small intestine. […] The problem is that its a lifelong intervention, and it stops working after some time, Vilar-Sanchez says. […] Along with his team, hes investigating if obeticholic acid can serve as a chemoprevention therapy to slow or halt the development of polyps in the duodenum of patients with FAP who have received a colectomy.

• #76 Familial Adenomatous Polyposis – MD Searchlight

  https://mdsearchlight.com/genetic-disorders/familial-adenomatous-polyposis/

  The majority of FAP patients, 90%, show gastric polyps, which usually do not become cancerous. Non-fundic gland polyps, however, are more frequently cancerous and require endoscopic management. High-grade dysplasia in any gastric polyp necessitates endoscopic or surgical resection based on guidelines. […] Periampullary and duodenal cancers are the leading causes of death in FAP patients after colon cancer. The lifetime risk of duodenal cancer is about 5% by age 60. Guidelines recommend starting surveillance at age 20 to 25 and repeating it every 2 to 3 years. Management depends on the polyps pathology and location. […] If Familial Adenomatous Polyposis (FAP) is not treated, it can almost always progress to colorectal cancer, which is a serious type of cancer affecting the large intestine. This can result in shorter lifespans, with the majority of patients not living past their 40s. However, with proper screening and preventive measures, the survival rates can be significantly improved. One of these preventive measures might be a colectomy, a surgery to remove part or all of your colon.

• #77 Familial Adenomatous Polyposis | St. Jude Care & Treatment

  https://www.stjude.org/care-treatment/treatment/genetic-syndromes/familial-adenomatous-polyposis.html

  To manage FAP, it is important to get regular screening for polyps. If polyps are found, they are removed. If there are many polyps, the colon may eventually be removed. The goal of screening is to find and treat growths such as polyps or other tumors early for the best outcome. […] People with FAP should watch for signs or symptoms that could signal cancer, such as: Unexplained weight loss, Appetite loss, Pain in the stomach or abdomen, Blood in the stool or changes in bowel habits, Aches, pains, lumps, or swelling for no clear reason, Headaches or changes in vision or nerve function that do not go away. It is important to seek medical help if anything unusual appears.

• #78 Familial Adenomatous Polyposis | St. Jude Care & Treatment

  https://www.stjude.org/care-treatment/treatment/genetic-syndromes/familial-adenomatous-polyposis.html

  To manage FAP, it is important to get regular screening for polyps. If polyps are found, they are removed. If there are many polyps, the colon may eventually be removed. The goal of screening is to find and treat growths such as polyps or other tumors early for the best outcome. […] People with FAP should watch for signs or symptoms that could signal cancer, such as: Unexplained weight loss, Appetite loss, Pain in the stomach or abdomen, Blood in the stool or changes in bowel habits, Aches, pains, lumps, or swelling for no clear reason, Headaches or changes in vision or nerve function that do not go away. It is important to seek medical help if anything unusual appears.

• #79 Familial adenomatous polyposis (FAP) – diagnosis, surgery | Macmillan Cancer Support

  https://www.macmillan.org.uk/cancer-information-and-support/worried-about-cancer/causes-and-risk-factors/familial-adenomatous-polyposis-fap

  If you have any questions or worries, talk to your surgeon, nurse or FAP specialist. Sometimes it helps to talk to other people in the same situation. […] If you know your body and what is normal for you, it will help you be aware of any changes. If you notice a change in how you feel or how your body works, it is better to be safe and get it checked. Always see your GP or your FAP specialist if you have symptoms that are ongoing or unusual for you.

• #80 Familial Adenomatous Polyposis | St. Jude Care & Treatment

  https://www.stjude.org/care-treatment/treatment/genetic-syndromes/familial-adenomatous-polyposis.html

  To manage FAP, it is important to get regular screening for polyps. If polyps are found, they are removed. If there are many polyps, the colon may eventually be removed. The goal of screening is to find and treat growths such as polyps or other tumors early for the best outcome. […] People with FAP should watch for signs or symptoms that could signal cancer, such as: Unexplained weight loss, Appetite loss, Pain in the stomach or abdomen, Blood in the stool or changes in bowel habits, Aches, pains, lumps, or swelling for no clear reason, Headaches or changes in vision or nerve function that do not go away. It is important to seek medical help if anything unusual appears.

• #81 Colon polyps – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/colon-polyps/symptoms-causes/syc-20352875

  Most people with colon polyps do not have any symptoms. Colon polyps are often found as a part of routine colorectal cancer screening. […] Symptoms that should prompt an appointment with a healthcare professional include: […] Change in bowel habits. Constipation or diarrhea that lasts longer than a week may mean the presence of a larger colon polyp or cancer. […] Change in stool color. Blood can show up as red streaks in the stool or make stool appear black. […] Iron deficiency anemia. Bleeding from polyps can happen slowly over time, without visible blood in the stool. Chronic bleeding may lead to iron deficiency anemia, which can cause tiredness and shortness of breath. […] Pain. A large colon polyp or cancer can block part of the bowel, leading to cramping and belly pain. […] Rectal bleeding. This can be a sign of colon polyps or cancer or other conditions, such as hemorrhoids or minor tears of the anus.

• #82 Familial Adenomatous Polyposis (FAP): Symptoms, Diagnosis, Risks

  https://my.clevelandclinic.org/health/diseases/16993-familial-adenomatous-polyposis-fap

  Familial adenomatous polyposis (FAP) is a hereditary syndrome that raises your risk of developing colorectal cancer to nearly 100%. Cancer develops from adenomatous colon polyps, which can appear in the hundreds to thousands. To manage this risk, most people with FAP will have their whole colon removed sometime in their early life. […] Many people get a few colon polyps at random as they get older. But if you have a hereditary polyposis syndrome like FAP, you’ll develop many colon polyps typically over a hundred starting from a young age. This significantly raises your lifetime risk that one of them will become cancerous. […] Without treatment, the risk of developing colorectal cancer with familial adenomatous polyposis is close to 100%. It also develops relatively earlier and faster with FAP than in those without.

• #83 Familial Adenomatous Polyposis: Alarming Symptoms, Causes, Types, Diagnosis and Treatment – OncoDaily

  https://oncodaily.com/oncolibrary/cancer-types/familial-adenomatous-polyposis

  Common symptoms of FAP typically begin to appear in adolescence, although they can manifest at any age. Key symptoms include: Rectal bleeding: Often the first noticeable sign, occurring due to bleeding from polyps in the colon. Abdominal pain: This is caused by the presence of multiple polyps and potential blockages in the colon. Diarrhea: Frequent or chronic diarrhea can result from numerous polyps affecting the function of the colon. According to research, nearly all individuals with untreated FAP will experience these symptoms at some point, making it crucial to recognize early signs and pursue timely intervention. Rectal bleeding is present in approximately 75% of patients with FAP. […] The early signs of FAP are subtle and may be overlooked in the initial stages. These can include: Mild gastrointestinal discomfort, Small amounts of blood in stool, Unexplained weight loss, Fatigue due to anemia from chronic bleeding.

• #84 Age and manifestation related symptoms in familial adenomatous polyposis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1079798/

  FAP was diagnosed clinically on the basis of symptoms in 84% (120/143) of these patients. Most presented with intestinal symptoms such as colonic bleeding (68%) and diarrhea (42%). All but one of the patients between 20 and 40 years old had rectal polyps (98.7%, 75/76), whereas in those over 40 years old the prevalence was 76% (35/46). Non-specific symptoms such as abdominal pain, fatigue and bloating were less frequent and were mainly reported by patients older than 40. […] The commonest presenting features of FAP are alteration of bowel habit and rectal bleeding, but both are found in many other conditions. Patients with these findings need immediate endoscopy to allow prompt diagnosis and prophylactic surgery. […] Untreated, the disease usually leads to the appearance of hundreds of adenomatous polyps in the colorectum between puberty and age 20 and to cancer by the early forties at the latest which is the most frequent reason for death in patients with FAP.

• #85 Familial Adenomatous Polyposis: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/175377-overview

  Familial adenomatous polyposis (FAP) is the most common adenomatous polyposis syndrome. It is an autosomal dominant inherited disorder characterized by the early onset of hundreds to thousands of adenomatous polyps throughout the colon. If left untreated, all patients with this syndrome will develop colon cancer by age 35-40 years. In addition, an increased risk exists for the development of other malignancies. […] The average age of onset of polyposis in FAP is 16 years, whereas the average age of onset for colorectal cancer is 39 years. […] Patients with untreated FAP have a median life expectancy of 42 years. Life expectancy is extended greatly in those treated with colectomy. […] The principal cause of mortality is colorectal cancer, which develops in all patients unless they are treated. The mean age at which colorectal cancer develops in patients with classic FAP is 39 years. Patients with adenomatous polyposis itself often are asymptomatic. […] Complications of FAP include the following: Colorectal cancer (100% in untreated patients).

• #86 Familial adenomatous polyposis syndromes – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-gb/652

  Familial adenomatous polyposis (FAP) syndrome is a hereditary colorectal cancer syndrome characterised by the development of hundreds to thousands of colorectal adenomas. The attenuated form leads to the formation of fewer than 100 polyps. […] Median age of onset for polyps is 15 years. […] In the absence of total colectomy or endoscopic polyp clearance, colorectal cancer arises in close to 100% of patients with FAP by 40 years of age and in 80% of patients with attenuated FAP by 60 years of age. […] Patients with classical FAP have hundreds to thousands of colorectal adenomas and a nearly 100% risk for colorectal cancer by the age of 40 years if proctoprophylactic colectomy is not performed. […] Attenuated FAP is also caused by APC mutations and is characterised by fewer than 100 adenomas and a later age of colorectal cancer onset.

• #87 Familial Adenomatous Polyposis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538233/

  The majority of patients typically present with nonspecific symptoms such as diarrhea, abdominal discomfort, and rectal bleeding. Increasingly, patients are identified through screening colonoscopy. Colonoscopic examinations reveal numerous colonic polyps, often exceeding 100, although the number may be lower when screenings start in childhood and are distributed throughout the colon. Attenuated FAP typically involves a reduced number of polyps emerging later in life, predominantly impacting the right colon. This variant normally features approximately 30 polyps and manifests at an average age of over 50 years. Symptoms and signs are related to the degree of polyposis and cancer stage. […] If left untreated, FAP progresses to colorectal cancer in nearly 100% of cases, leading to a shortened life expectancy, with most patients dying by their fourth decade of life. However, survival rates are significantly improved with enhanced screening, surveillance, and prophylactic colectomy.

• #88 Familial Adenomatous Polyposis Syndrome – DoveMed

  https://www.dovemed.com/diseases-conditions/familial-adenomatous-polyposis-syndrome

  The greater the number of polyps in the large intestine, the greater is the risk for developing colon cancer later in life. […] Adenomatous polyps can develop into colon cancer with additional complications. […] The prognosis of Familial Adenomatous Polyposis Syndrome depends on how early it is diagnosed and medical interventions instituted. […] With no medical intervention, the median life expectancy of an individual with Familial Adenomatous Polyposis Syndrome is about 42 years. […] Prophylactic colectomy is known to increase the life expectancy.

• #89 Familial Adenomatous Polyposis: Causes, Symptoms, Treatments, and More

  https://resources.healthgrades.com/right-care/colon-cancer/familial-adenomatous-polyposis

  FAP is currently not curable. The condition requires lifelong screening, even after surgery. […] FAP is a rare inherited condition characterized by the formation of colorectal polyps that are likely to become cancerous. While many people are asymptomatic, symptoms may include abdominal discomfort, rectal bleeding, and diarrhea.

• #90 Gardner syndrome: What is it, causes, and symptoms

  https://www.medicalnewstoday.com/articles/gardner-syndrome

  People with Gardner syndrome need to schedule regular checkups with a healthcare professional to monitor the growth and manage symptoms. […] Some people may not experience symptoms, but others may experience gastrointestinal symptoms. […] There is no cure for Gardner syndrome. However, regular screening and treatment help manage the symptoms. People with Gardner syndrome have a higher risk of developing cancer earlier in life. Regularly following up with a healthcare professional and following treatment plans may help decrease the severity of symptoms and cancer risk.

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