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Familial adenomatous polyposis

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1285:"Colonoscopy every two to three years, beginning at age 18 to 20 years; esophagogastroduodenoscopy (EGD) beginning by age 25 years or prior to colectomy and repeated every 1–3 years; in some cases, endoscopic retrograde cholangiopancreatography (ERCP) may be necessary to evaluate for adenomas of the common bile duct; annual physical examination with palpation of the thyroid with consideration of follow-up ultrasound examination and fine-needle aspiration if thyroid nodules are present. Colectomy usually advised when more than 20 or 30 adenomas or multiple adenomas with advanced histology have developed." Sovaria states as at 1998 that "colonoscopy, as opposed to sigmoidoscopy, should be advised for endoscopic surveillance, because of the right-side location of colorectal adenomas; UGI endoscopic surveillance is warranted in an attempt to detect premalignant gastric or duodenal tumors; individuals affected with may require total colectomy with ileo-rectal anastomosis only when prophylactic colectomy is advised" 1173:"Full gene sequencing of all APC exons and intron-exon boundaries appears to be the most accurate clinical test available. Most APC mutations are nonsense or frameshift mutations that cause premature truncation of the APC protein.. The likelihood of detecting an APC mutation is highly dependent on the severity of colonic polyposis and on the family history.. ◦Approximately 20% of individuals with an apparent de novo APC mutation.. The markers used for linkage analysis of APC-associated polyposis conditions are highly informative and very tightly linked to the APC locus; thus, they can be used with greater than 98% accuracy in more than 95% of families with an APC-associated polyposis condition. Linkage testing is not possible for families with a single affected individual, a situation that often occurs when an individual has a de novo gene mutation and no affected offspring.. If no disease causing APC mutation is found, molecular genetic testing of MUTYH (see Differential Diagnosis) should be considered." 1282:"Sigmoidoscopy or colonoscopy every 1–2 years, beginning at age ten to 12 years; colonoscopy, once polyps are detected; annual colonoscopy, if colectomy is delayed more than a year after polyps emerge (Age ten to 20 years with certain milder symptoms, delay in colectomy may be considered); Esophagogastroduodenoscopy (EGD) by age 25 years or prior to colectomy and repeated every 1–3 years; in some cases, endoscopic retrograde cholangiopancreatography (ERCP) to evaluate for adenomas of the common bile duct; small-bowel imaging when duodenal adenomas are detected or prior to colectomy, repeated every 1–3 years depending on findings; screening for hepatoblastoma (optimal interval unknown, one paper recommends "at least every three months"); annual physical examination, including evaluation for extraintestinal manifestations, and palpation of the thyroid with consideration of follow-up ultrasound examination and fine-needle aspiration if thyroid nodules are present" 1296:"Early recognition may allow for timely intervention and improved final outcome; thus, surveillance of asymptomatic, at-risk children for early manifestations is appropriate; genetic testing is more cost effective than sigmoidoscopy in determining who in the family is affected; individuals diagnosed with APC-associated polyposis conditions as a result of having an affected relative have a significantly greater life expectancy than those individuals diagnosed on the basis of symptoms.. As colon monitoring for those at risk for classic FAP begins as early as age ten to 12 years, molecular genetic testing is generally offered to children at risk for classic FAP by age ten years. Genetic testing at birth may also be warranted, as some parents and pediatricians may consider hepatoblastoma screening from infancy to age five years in affected offspring.. No evidence points to an optimal age at which to begin screening." 414: 51: 763: 1219:: mutations in codons 1395–1493 has significantly higher rates of desmoid tumors, osteomas, and epidermoid cysts than those with mutations in codons 177–452; mutations in codons 1395–1493 have significantly higher rates of desmoid tumors and osteomas than those with mutations in codons 457–1309; no individuals with mutations in codons 177–452 developed osteomas or periampullary cancers; only individuals with mutations in codons 457–1309 developed hepatoblastoma and/or brain tumors. 281:(7% risk by age 21, rising to 87% by age 45 and 93% by age 50). These gene changes do not trigger cancer, but rather, they reduce the body's ability to prevent cells from becoming cancerous. Even with the gene change, it may still take time before a cell actually does develop that is cancerous as a result, and the gene may in some cases still partially operate to control tumours, therefore cancer from FAP takes many years to develop and is almost always an adult-onset disease. 664: 1227:: mutations 3' to codon 1399 were associated with desmoid tumor development with an odds ratio of 4.37; desmoid tumors in 20% of individuals with mutations 5' to codon 1444, 49% of individuals with mutations 3' to codon 1444, and 61% of individuals with mutations in codons 1445–1580; several families with severe desmoid tumors had mutations at the extreme 3' end; consistent association of desmoid tumors with mutations distal to codon 1444. 788:
It is important to note that visual examination, or monitoring, cannot 'clear' a person of risk. It can only say what their condition is at the time. If at any point in their life the person develops numerous polyps, this would tend to suggest a diagnosis of FAP. (Absence of polyps does not 'clear' a
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Most frequent APC mutation is at codon 1309 and lead to a high number of polyps at an early age (~20). Profuse polyposis (avg=5000) reported with mutations in codons 1250–1464. Most partial and whole APC deletions are associated with 100–2000 colonic adenomas, although attenuated FAP has been seen.
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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
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has been performed, colonoscopic surveillance of the remaining colon is necessary as the individual still has a risk of developing colon cancer. However, if this happened, it would be a fresh incident from polyps developing anew in the unremoved part of the colon subsequent to surgery, rather than a
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Prior to reaching the advanced stages of colorectal cancer, the polyps are confined to the inner wall and thickness of the intestinal tract and do not metastasize or 'spread'. So provided FAP is detected and controlled either at the pre-cancerous stage or when any cancerous polyps are still internal
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NCBI states that "Although most individuals diagnosed with an APC-associated polyposis condition have an affected parent, the family history may appear to be negative because of failure to recognize the disorder in family members, early death of the parent before the onset of symptoms, or late onset
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is the usual diagnostic test of choice as it favours the common right-side location of polyps better than sigmoidoscopy if the mutation is attenuated FAP, and can confirm or allow (a) the actual clinical presentation and any change to the condition, of the 'at risk' individual, (b) quantification of
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Because of the genetic nature of FAP, polyposis registries have been developed around the world. The purpose of these registries is to increase knowledge about the transmissibility of FAP, but also to document, track, and notify family members of affected individuals. One study has shown that the
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Screening/monitoring programs involve visually examining the intestinal tract to check its healthy condition. It is undertaken as a routine matter every few years where there is cause for concern when either (a) a genetic test has confirmed the risk or (b) a genetic test has not been undertaken for
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gene exists that can determine whether it is present, and therefore can predict the possibility of FAP. Individuals at risk (due to family links or genetic testing) are usually offered routine monitoring of the intestinal tract every 1–3 years for life, from puberty for FAP and early adulthood for
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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).
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Because of the way familial polyposis develops, it is possible to have the genetic condition, and therefore be at risk, but have no polyps or issues so far. Therefore, an individual may be diagnosed "at risk of" FAP, and require routine monitoring, but not (yet) actually have FAP (i.e., carries a
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Attenuated FAP is associated with mutations (typically truncating) in the 5' part of the gene (codons 1–177), exon 9, and the distal 3' end of the gene; interstitial deletions of chromosome 5q22 that include APC; partial and whole-gene deletions; and somatic mosaicism for APC mutations that are
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Under 100 (0–470, typ. 30), sometimes flat rather than polypoid morphology, and more proximal to the splenic flexure. In a study of 120 individuals 37% (N=44) had <10 polyps; 3 of these 44 had colorectal cancer. Gastric fundic polyps and duodenal adenomas are also seen. Therefore, polyps and
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gene functional but slightly impaired. It is therefore somewhat able to operate as usual. Attenuated FAP still presents a high 70% lifetime risk of cancer (as estimated), but typically presents with far fewer polyps (typically 30) rather than the hundreds or thousands usually found in FAP, and
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allele with a stop codon at position 850. Heterozygosity for this mutation results in a fully penetrant phenotype on most genetic backgrounds, with mice on a sensitive background developing over 100 tumors in the intestinal tract. The number and location of the intestinal tumors are modified by
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is usually needed in families where FAP has been diagnosed. Testing may also aid in the diagnosis of borderline cases in families that are otherwise known to p34.3 and p32.1 (1p34.3–p32.1). Testing can only show if an individual is susceptible to FAP or rule it out (i.e., whether or not they
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In 2007, the "ApcPirc" rat model was isolated with a stop codon at position 1137. In contrast to the mouse models where >90% of tumors form in the small intestine, the Pirc rat forms tumors preferentially (>60%) in the large intestine, similar to the human clinical presentation.
1253:"2.29 to 3.2 per 100,000 individuals.. APC-associated polyposis conditions historically accounted for about 0.5% of all colorectal cancers; this figure is declining as more at-risk family members undergo successful treatment following early polyp detection and prophylactic colectomy." 1073:
Not settled as yet. (a) no family history of 100+ polyps before age 30 PLUS ONE OF 10–99 polyps / 100+ polyps and aged over 35 to 40 / colorectal cancer before age 60 and relatives with multiple adenomatous polyps, OR (b) Family history of 10 to 99 adenomas diagnosed after age 30
509:). A flaw in the APC gene means APC is not as effective as it should be, and over time it is likely that some cells that should have been controlled by APC will not be, and will instead continue to develop and become cancerous. In familiar polyposis they usually manifest as 901:
reconstruction. The decision to remove the rectum depends on the number of polyps in the rectum as well as the family history. If the rectum has few polyps, the colon is partly or fully removed and the small bowel (ileum) can be directly connected to the rectum instead
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APC-associated polyposis conditions are inherited in an autosomal dominant manner. Approximately 20–25% have the altered gene as the result of a de novo gene mutation. Little or no evidence of maternal/paternal bias, or effect related to advanced paternal age, in
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NCBI states that when an individual is identified as having FAP, or the mutations resulting in FAP: "It is appropriate to evaluate the parents of an affected individual (a) with molecular genetic testing of APC if the disease-causing mutation is known in the
402:, or in any combination of these. Therefore, an absence of polyps in, for example, the rectum, may not of itself be sufficient to confirm absence of polyps. It may be necessary to consider and visually examine other possible parts of the intestinal tract. 276:
that prevent development of tumours. The change allows numerous cells of the intestinal wall to develop into potentially cancerous polyps when they would usually reach the end of their life; inevitably one or more will eventually progress and give rise to
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Classic FAP: "Colectomy is recommended after adenomas emerge; colectomy may be delayed depending on the size and number of adenomatous polyps. Colectomy is usually advised when more than 20 or 30 adenomas or multiple adenomas with advanced histology have
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end. Phenotypic expression in these three groups of kindreds is variable but is definitely milder than that in classical FAP" and that rectal polyps are rare in attenuated FAP but not yet confirmed whether this also means rectal cancer risk is lower as
1159:"inherited in an autosomal dominant manner. Approximately 75%-80% of individuals with APC-associated polyposis conditions have an affected parent. Offspring of an affected individual are at a 50% risk of inheriting the disease-causing mutation" 625:
pattern, which means two copies of the gene must be altered for a person to be affected by the disorder. Most often, the parents of a child with an autosomal recessive disorder are not affected but are carriers of one copy of the altered gene.
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NCBI states that physicians must ensure they understand the "risks, benefits, and limitations" of any genetic test done since in 1997 "for almost one-third of individuals assessed for FAP, the physician misinterpreted the test results".
1145:"Small bowel carcinoma 4–12% Rare; Pancreas Adenocarcinoma ~1%; Papillary thyroid carcinoma 1–2%; CNS <1%; Liver hepatoblastoma 1.6%; Bile ducts adenocarcinoma Low but increased; Stomach adenocarcinoma <1% in Western cultures." 296:
arises at an age when FAP is usually no longer considered likely—typically between 40 and 70 years old (average 55) rather than the more usual 30s upward. Because it has far fewer polyps, options for management may be different.
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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.
1893:"Efficacy and safety of eflornithine (CPP-1X)/sulindac combination therapy versus each as monotherapy in patients with familial adenomatous polyposis (FAP): design and rationale of a randomized, double-blind, Phase III trial" 260:
gene on chromosome 1. Of the three, FAP itself is the most severe and most common; although for all three, the resulting colonic polyps and cancers are initially confined to the colon wall. Detection and removal before
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attenuated forms. Colon resection surgery is recommended if numerous colon polyps are found due to high risk of early death from colon cancer. International polyposis registries exist that track known cases of FAP or
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pattern, which means one copy of the altered gene is sufficient to cause the disorder. The incidence of malignancy in these cases approaches 100%. In most cases, an affected person has one parent with the condition.
1239:: revealed 89 submicroscopic APC deletions (42 partial and 47 whole-gene deletions). Extracolonic findings were seen in 36% of cases, with no significant differences in those with partial vs. whole-gene deletions. 1767:
Lynch, Patrick M; Burke, Carol A; Phillips, Robin; Morris, Jeffrey S; Slack, Rebecca; Wang, Xuemei; Liu, Jun; Patterson, Sherri; Sinicrope, Frank A; Rodriguez-Bigas, Miguel A; Half, Elizabeth (February 2016).
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See FAP. Also "Colon screening for those with attenuated FAP begins at age 18 to 20 years; thus, molecular genetic testing should be offered to those at risk for attenuated FAP at approximately age 18 years."
933:(NSAIDs). NSAIDS have been shown to significantly decrease the number of polyps but do not usually alter management since there are still too many polyps to be followed and treated endoscopically. The drug 678:
Making the diagnosis of FAP before the development of colon cancer is important not just for the individual, but also for the sake of other family members who may be affected. Two diagnostic methods exist:
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and lead to tumor formation. When MYH glycosylase does not function correctly, DNA errors may accrue to initiate tumorigenesis with a clinical presentation similar to that in patients with APC mutations.
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Burke, Carol A.; Dekker, Evelien; Lynch, Patrick; Samadder, N. Jewel; Balaguer, Francesc; HĂĽneburg, Robert; Burn, John; Castells, Antoni; Gallinger, Steven; Lim, Ramona; Stoffel, Elena M. (2020-09-10).
881:. Most individuals with the APC mutation will develop colon cancer by the age of 40, although the less-common attenuated version typically manifests later in life (40–70). Accordingly, in many cases, 885:
surgery may be recommended before the age of 25, or upon detection if actively monitored. There are several surgical options that involve the removal of either the colon or both the colon and rectum.
1131:"polyps of the gastric fundus and duodenum, osteomas, dental anomalies, congenital hypertrophy of the retinal pigment epithelium (CHRPE), soft tissue tumors, desmoid tumors, and associated cancers" 1271:"Colectomy may be necessary, but in approximately one third of individuals the colonic polyps are limited enough in number that surveillance with periodic colonoscopic polypectomy is sufficient" 4089: 1235:: In 24 individuals, the majority of mutations identified were 5' to codon 1220 ; 9 of 12 individuals had APC mutations identified proximal to the mutation cluster region (codons 1286–1513). 4363: 2002:
Neklason, Deborah W.; Stevens, Jeffery; Boucher, Kenneth M.; Kerber, Richard A.; Matsunami, Nori; Barlow, Jahn; Mineau, Geraldine; Leppert, Mark F.; Burt, Randall W. (January 2008).
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to the intestinal tract, surgery has a very high success rate of preventing or removing cancer, without recurrence, since the locations giving rise to cancer are physically removed
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is possible if a disease-causing mutation is identified in an affected family member; however, prenatal testing for typically adult-onset disorders is uncommon and requires careful
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gene defects, for research and clinical purposes. Mutation of APC also occurs commonly in incident cases of colorectal carcinoma, emphasizing its importance in this form of cancer.
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and the membrane. The canonical tumor-suppressor function of APC is suppression of β-catenin, but other tumor-suppressor functions of APC may be related to cell adherence and
3725: 1323:, therefore genetic testing should be offered. Offspring each have a 50% chance of inheritance. Other family members are at risk if their parents share the same mutation. 322:, or in the stomach or duodenum) where they show no symptoms until cancer is present and greatly advanced. APC mutations have been linked to certain other cancers such as 3922: 648: 926:
Treatment for the two milder forms of FAP may be substantially different from the more usual variant, as the number of polyps is far fewer, allowing more options.
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Depending on the nature of the defect in the APC gene, and whether it is the full or attenuated form, familial polyposis may manifest as polyps in colon or in the
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Diagnosis (confirming whether they have FAP)—this can be done either by genetic testing, which is definitive or by visually checking the intestinal tract itself.
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diagnosis (cell/cancer type detection) and (d) where polyps exist, it can suggest whether outpatient excision (removal) is viable or surgery is recommended.
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Treatment, typically surgery of some kind, is involved if polyposis has led to a large number of polyps, or a significant risk of cancer, or actual cancer.
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is indicated if more than a hundred polyps are present, if there are severely dysplastic polyps, or if multiple polyps larger than 1 cm are present.
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Familial adenomatous polyposis can have different inheritance patterns and different genetic causes. When this condition results from mutations in the
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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.
853:), typically once every 1–3 years, and/or a genetic blood test to definitively confirm or deny susceptibility. A small number of polyps can often be 4049: 2932: 4039: 4031: 3420: 809:
any reason so the actual risk is unknown. Screening and monitoring allow polyposis to be detected visually before it can become life-threatening.
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inherited the defective APC gene). It cannot determine the actual condition of a patient; this can only be found by direct physical examination.
3280: 1770:"An international randomised trial of celecoxib versus celecoxib plus difluoromethylornithine in patients with familial adenomatous polyposis" 505:, a protein that plays a crucial role in cell communication, signalling, growth, and controlled destruction, but which left uncontrolled also 4330: 1195:
generally associated with classic FAP. Sovaria states attenuated FAP is "caused by mutations in three distinct regions of the APC gene—the 5
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defective gene but as yet appears not to have any actual medical issue as a result of this). Clinical management can cover several areas:
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From early adolescence, patients with this condition gradually (and much of the time asymptomatically) develop hundreds to thousands of
501:(APC), a large multifunction tumour-suppressing protein which acts as a "gatekeeper" to prevent development of tumours. (APC regulates 3495: 2347: 2296: 4155: 3032: 2117: 413: 1191:
Sample typical onset ages: between codon 168 and 1580 (excluding 1309) = 30 years, 5' of codon 168 and 3' of codon 1580 = 52 years.
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Desmoid tumors, with their infiltrative nature and potential proximity to vital structures, are the second highest cause of death.
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person, as polyps can develop later in life; also a few polyps over time are not that uncommon in people without FAP. However a
371:. These may bleed, leading to blood in the stool. If the blood is not visible, it is still possible for the patient to develop 3959: 2832: 2665: 387:
or elsewhere. FAP can also develop 'silently' in some individuals, giving few or no signs until it has developed into advanced
1256:"Likely underdiagnosed, given the lower number of colonic polyps and lower risk for colorectal cancer compared to classic FAP" 4377: 4212: 4044: 4034: 3430: 3233: 4059: 3698: 3141: 421:
The genetic determinant in familial polyposis may also predispose carriers to other malignancies, e.g., of the duodenum and
1354: 1106:"Lower.. less well known.. estimated 70% by age 80". Sovaria states as at 1998, "average age at CRC diagnosis is ~58 years" 4207: 4176: 4127: 4004: 3974: 3878: 3828: 3798: 3488: 2770: 2289: 643:
unlinked genes. Many other models have since appeared, including a model of attenuated FAP (the 1638N model) and several
3594: 3848: 3782: 3459: 3133: 3046: 2956: 2559: 4407: 3668: 3645: 3622: 3584: 3546: 3399: 3175: 3151: 2817: 2734: 2367: 3137: 1681:"Phenotypic variability of familial adenomatous polyposis in 11 unrelated families with identical APC gene mutation" 4247: 4228: 4145: 3415: 3156: 3010: 2874: 2856: 2766: 2717: 1581:"A genetic and clinical study of intestinal polyposis, a predisposing factor for carcinoma of the colon and rectum" 846: 567: 558:
also occurs commonly in incident cases of colorectal carcinoma, emphasizing its importance in this form of cancer.
498: 326:. As the mutation causing FAP is autosomal dominant, it can be inherited directly from either parent to a child. A 250: 153: 2137: 4397: 3323: 3252: 2948: 2851: 2774: 2611: 2437: 2400: 778:
Identifying those individuals who could be at risk of FAP: usually from family medical history or genetic testing
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Prominent extracolonic manifestations often correlate (though not completely) with more distal APC mutations.
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Various medications are being investigated for slowing malignant degeneration of polyps, most prominently the
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Hes FJ, Nielsen M, Bik EC, Konvalinka D, Wijnen JT, Bakker E, Vasen HF, Breuning MH, Tops CM (January 2008).
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Soravia, C.; Berk, T.; Madlensky, L.; Mitri, A.; Cheng, H.; Gallinger, S.; Cohen, Z.; Bapat, B. (June 1998).
1176:"Fewer than 30% of individuals with attenuated phenotypes are expected to have an identifiable APC mutation" 4341: 4283: 3888: 3464: 3213: 2982: 2357: 938: 903: 739: 86: 3994: 3952: 3551: 3305: 3093: 3055: 2965: 2740: 2564: 2504: 494: 273: 144: 1349:
use of a registry to notify family members (call-ups) significantly reduced mortality when compared with
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Rectum not involved: the portion of the colon manifesting polyps can be removed and the ends 'rejoined' (
644: 3741: 3599: 3556: 2902: 2626: 2269: 4253: 3165: 1103:"inevitable.. virtually 100%": 7% by age 21, 87% by age 45, 93% by age 50. Typical ages: 34–43 (avg.39) 455: 3310: 2730: 2589: 2526: 2362: 2342: 1891:
Burke, Carol A.; Dekker, Evelien; Samadder, N. Jewel; Stoffel, Elena; Cohen, Alfred (December 2016).
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mutation, and of those with an apparent de novo APC mutation (i.e. no known family history) 20% have
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The normal function of the APC gene product is still being investigated; it is present in both the
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Rectum involved: the rectum and part or all of the colon are removed. The patient may require an
857:(removed) during the procedure if found, but if there are more severe signs or numbers, inpatient 425:(particularly ampullary adenocarcinoma). Other signs that may point to FAP are the development of 4222: 3898: 3868: 3838: 3818: 3787: 3370: 3352: 3292: 3121: 3117: 2699: 2531: 2494: 2372: 2112: 1950: 1873: 1815: 1749: 1385: 1324: 733: 706: 521: 475: 201: 197: 2390: 1721: 830:. Asymptomatic individuals (and therefore asymptomatic family members) are also known to exist. 1622:"A target-selected Apc-mutant rat kindred enhances the modeling of familial human colon cancer" 647:
that allow for tissue-specific or temporal ablation of gene function. For more information see
3963: 3650: 3627: 3579: 3561: 3437: 3389: 3380: 3103: 3005: 2974: 2843: 2229: 2108:— full clinical summary of FAP and attenuated FAP, including lifetime risks, epidemiology etc. 2099: 2068: 2033: 1932: 1914: 1865: 1857: 1807: 1799: 1741: 1702: 1661: 1602: 1561: 1459: 1370: 827: 671: 433:(benign skin tumors that may be apparent before other signs of FAP), pigmented lesions of the 388: 224: 130: 91: 70: 17: 536:) to APC-mutated cells are much more likely to lead to cancer than they would in non-mutated 4190: 3238: 3112: 3027: 3015: 2804: 2722: 2536: 2448: 2060: 2023: 2015: 1922: 1904: 1847: 1836:"Eflornithine plus Sulindac for Prevention of Progression in Familial Adenomatous Polyposis" 1789: 1781: 1769: 1733: 1692: 1651: 1641: 1592: 1551: 1543: 1532:"Soft Tissue Special Issue: Fibroblastic and Myofibroblastic Neoplasms of the Head and Neck" 1449: 1441: 1390: 1375: 1327:
has been documented in asymptomatic cases. Prenatal testing is possible via fetal extracted
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Giardiello FM, Krush AJ, Petersen GM, Booker SV, Kerr M, Tong LL, Hamilton SR (June 1994).
945:, is being investigated as a potential preventive medication in combination with the NSAID 913:), a surgery that has a substantial healing time but leaves quality of life largely intact. 375:
due to gradually developing iron deficiency. If malignancy develops, this may present with
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for this, as it provides better observation of the common right-side location of polyps.
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mutations. Siblings have classic 50% risk of sharing the condition if inherited and not
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outside the colon can greatly reduce and in many cases eliminate the spread of cancer.
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is mutated or deleted, accelerating generation of polyps. Further mutations (e.g., in
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is associated with: mutations between codons 311 and 1444; whole APC gene deletions.
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As for FAP but "CHRPE and desmoid tumors are rare" and also thyroid cancer is added.
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has already taken place: the inherited APC mutation. Often, the remaining "normal"
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In some cases FAP can manifest higher in the colon than usual (for example, the
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occurs when they are left untreated. Three variants are known to exist, FAP and
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of the disease in the affected parent." In addition around 20% of cases are a
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Inheritance and implications of confirmed diagnosis for other close relatives
699:(a form of medical imaging) can also be used to suggest the diagnosis of FAP. 4278: 4171: 3834: 3824: 3814: 3804: 3772: 3760: 3688: 3347: 3270: 3088: 2658: 2594: 2334: 2330: 2253: 2178: 2174: 2138:
National Cancer Institute: Genetics of Colorectal Cancer information summary
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or (b) for clinical manifestations of APC-associated polyposis conditions".
766: 688: 173: 2103: 2072: 2037: 1936: 1869: 1811: 1794: 1745: 1665: 1606: 1565: 1852: 1835: 1706: 1463: 1430:"Genotype-phenotype correlations in attenuated adenomatous polyposis coli" 4198: 3614: 3410: 2264: 2090:
Jasperson, Kory W.; Patel, Swati G.; Ahnen, Dennis J. (2 February 2017).
2004:"American Founder Mutation for Attenuated Familial Adenomatous Polyposis" 950: 878: 606: 598: 468: 399: 238: 79: 2157: 4321: 4238: 4218: 4114: 3705: 3480: 2616: 2423: 2395: 1380: 1350: 866: 858: 854: 594: 586: 446: 442: 422: 1223:: Fourfold increased risk with mutations between codons 976 and 1067. 4137: 3943: 3571: 2893: 2686: 2480: 2313: 2169: 2064: 1070:(a) 100+ polyps and age under 40, OR (b) polyps and FAP in a relative 590: 525: 434: 372: 368: 278: 216: 2051:
Reyes Moreno J, Ginard Vicens D, Vanrell M, et al. (2007). "".
1445: 1199:
end in the region spanning exons 4 and 5, exon 9, and the extreme 3
3912: 3908: 3904: 3660: 3538: 2414: 1722:"Somatic APC mosaicism: an underestimated cause of polyposis coli" 894: 797:
of polyps would generally tend to suggest a diagnosis of FAP, and
761: 662: 573: 412: 384: 256: 520:
Although the polyps are inherently benign, the first step of the
4358: 3407: 2212: 417:
CHRPE - Congenital hypertrophy of the retinal pigment epithelium
3484: 2921: 2285: 1328: 610: 529: 445:(benign bone tumors). The combination of polyposis, osteomas, 1279:
Surveillance (monitoring) activities once risk is established
1357:
is the oldest in the world, started in 1924, and many other
4090:
Hydrops-ectopic calcification-moth-eaten skeletal dysplasia
769:
specimen showing numerous polyps throughout the large bowel
1117:
Inter- and intrafamilial phenotypic variability are common
4364:
Microcephalic osteodysplastic primordial dwarfism type II
2094:. In Adam MP; Ardinger HH; Pagon RA; Wallace SE (eds.). 1620:
Amos-Landgraf J, Kwong LN, Dove WF, et al. (2007).
1165:
Different—recessive (requires 2 parents to be carriers)
1059:
gastrointestinal tract rather than the usual locations.
949:
for treatment of FAP. Another investigational agent is
897:
where stool goes into a bag on the abdomen) or have an
1505:"Familial adenomatous polyposis: MedlinePlus Genetics" 1217:
General study of FAP plus extracolonic symptoms showed
801:
to determine whether or not any polyps are cancerous.)
4326:
Epidermolysis bullosa simplex with muscular dystrophy
1319:
and a "low" but slightly higher risk than general if
307:, is also milder and, as its name suggests, requires 2147: 4293: 4237: 4189: 4164: 4136: 4113: 4104: 4013: 3983: 3936: 3749: 3740: 3681: 3659: 3636: 3613: 3570: 3537: 3528: 3519: 3450: 3379: 3337: 3260: 3251: 3226: 3204: 3102: 3079: 3054: 3045: 2996: 2973: 2964: 2955: 2892: 2865: 2842: 2803: 2792: 2756: 2708: 2685: 2674: 2639: 2582: 2545: 2517: 2436: 2422: 2413: 2381: 2329: 2320: 2244: 2151: 1002:This table compares the different subtypes of FAP: 638:" mouse model was described in 1990 and carries an 179: 159: 143: 129: 121: 113: 105: 97: 85: 69: 40: 35: 3726:Terminal osseous dysplasia with pigmentary defects 301:autosomal recessive familial adenomatous polyposis 1237:General review of the literature (to August 2006) 705:provides the ultimate diagnosis in 95% of cases; 649:mouse models of colorectal and intestinal cancer 1489: 978:of any cancer removed by the original surgery. 3859:Meesmann juvenile epithelial corneal dystrophy 3809:Meesmann juvenile epithelial corneal dystrophy 65:of patient with familial adenomatous polyposis 3496: 2933: 2297: 1085:7–36 (typ. 16), rapidly increasing thereafter 8: 4347:Arrhythmogenic right ventricular dysplasia 9 4315:Arrhythmogenic right ventricular dysplasia 8 849:(EGD, to search for premalignant gastric or 355:)—small abnormalities at the surface of the 268:The root cause of FAP is understood to be a 3928:Reticular pigmented anomaly of the flexures 1423: 1421: 1419: 1417: 1415: 1413: 1411: 674:precursor most commonly associated with FAP 513:—small abnormalities on the surface of the 4110: 3746: 3534: 3525: 3503: 3489: 3481: 3257: 3051: 2970: 2961: 2940: 2926: 2918: 2800: 2682: 2433: 2419: 2326: 2304: 2290: 2282: 2148: 601:. MYH glycosylase fixes these mistakes by 49: 32: 3196:Hereditary nonpolyposis colorectal cancer 2027: 1926: 1908: 1851: 1793: 1696: 1655: 1645: 1596: 1555: 1485: 1483: 1481: 1479: 1477: 1475: 1473: 1453: 718:Once the diagnosis of FAP is made, close 3923:Naegeli–Franceschetti–Jadassohn syndrome 2098:. Seattle WA: University of Washington. 2008:Clinical Gastroenterology and Hepatology 1004: 953:, also used in combination with NSAIDs. 3421:Intraductal papillary mucinous neoplasm 1407: 845:, and occasionally upper gastric tract 1170:Genetic overview and genetic detection 200:inherited condition in which numerous 4331:Epidermolysis bullosa simplex of Ogna 3600:DFN A3, 4, 11, 17, 22; B2, 30, 37, 48 2092:"APC-Associated Polyposis Conditions" 931:non-steroidal anti-inflammatory drugs 838:Monitoring involves the provision of 585:. During normal cellular activities, 459:(with or without abnormal scarring). 7: 3948:Desmin-related myofibrillar myopathy 3585:Hypertrophic cardiomyopathy 1, 8, 10 1982:The Lecturio Medical Concept Library 1530:Baranov E, Hornick JL (March 2020). 1499: 1497: 4040:Emery–Dreifuss muscular dystrophy 2 3470:Desmoplastic small round cell tumor 2779:Papillary serous cystadenocarcinoma 687:polyps throughout the colon, (c) a 4305:Striate palmoplantar keratoderma 2 4000:Charcot–Marie–Tooth disease 1F, 2E 3778:Striate palmoplantar keratoderma 3 3171:Polymerase proofreading-associated 2348:Combined small-cell lung carcinoma 497:responsible for the production of 25: 4156:Asphyxiating thoracic dysplasia 3 4045:Limb-girdle muscular dystrophy 1B 3033:Hereditary diffuse gastric cancer 1128:Possible non-colon manifestations 877:Treatment for FAP depends on the 284:The second form of FAP, known as 4151:Short rib-polydactyly syndrome 3 4128:Hereditary spastic paraplegia 10 3623:Hypertrophic cardiomyopathy 7, 2 3329:Nodular regenerative hyperplasia 1978:"Familial Adenomatous Polyposis" 1067:Typical core diagnostic criteria 969:Following surgery, if a partial 750:are often performed to rule out 235:hereditary flat adenoma syndrome 4050:Charcot–Marie–Tooth disease 2B1 2666:Syringocystadenoma papilliferum 1840:New England Journal of Medicine 593:, which causes it to pair with 379:, altered bowel habit, or even 215:. While these polyps start out 27:Pre-cancerous intestinal polyps 4259:Familial adenomatous polyposis 4213:Hereditary elliptocytosis 2, 3 4123:Charcot–Marie–Tooth disease 2A 4035:Familial partial lipodystrophy 3547:Hypertrophic cardiomyopathy 11 3431:Solid pseudopapillary neoplasm 3234:Gastrointestinal stromal tumor 3191:Familial adenomatous polyposis 2454:Familial adenomatous polyposis 2124:Familial Adenomatous Polyposis 2113:Familial adenomatous polyposis 1951:"Desmoid Tumor - Risk Factors" 507:gives rise to numerous cancers 289:familial adenomatous polyposis 254:) is caused by defects in the 244:defects on chromosome 5 while 190:Familial adenomatous polyposis 36:Familial adenomatous polyposis 18:Familial Adenomatous Polyposis 1: 4208:Hereditary spherocytosis 2, 3 4177:Cavernous venous malformation 4005:Amyotrophic lateral sclerosis 3975:Amyotrophic lateral sclerosis 3879:Epidermolysis bullosa simplex 3829:Epidermolysis bullosa simplex 3799:Ichthyosis bullosa of Siemens 3669:Hypertrophic cardiomyopathy 9 3646:Hypertrophic cardiomyopathy 3 3239:Krukenberg tumor (metastatic) 2829:Paget's disease of the breast 2771:Pancreatic serous cystadenoma 1355:St. Mark's polyposis registry 589:sometimes becomes altered by 309:both parents to be 'carriers' 3849:Epidermolytic hyperkeratosis 3783:Epidermolytic hyperkeratosis 3460:Primary peritoneal carcinoma 3134:traditional serrated adenoma 2833:Extramammary Paget's disease 2560:Multiple endocrine neoplasia 1698:10.1016/0016-5085(94)90408-1 1626:Proc. Natl. Acad. Sci. U.S.A 1178:(see also details under FAP) 1082:Age at which polyps manifest 1051:cancers may manifest in the 562:MUTYH gene mutation variants 4070:Buschke–Ollendorff syndrome 3400:Pancreatic ductal carcinoma 2818:Pancreatic ductal carcinoma 2735:Mucinous cystadenocarcinoma 2368:Transitional cell carcinoma 1264:Treatment of manifestations 1044:Typical polyp manifestation 311:to manifest the condition. 4424: 4229:Hereditary spherocytosis 1 4146:Primary ciliary dyskinesia 3552:Dilated cardiomyopathy 1AA 3416:Serous microcystic adenoma 3011:Signet ring cell carcinoma 2949:Digestive system neoplasia 2875:Medullary breast carcinoma 2857:Invasive lobular carcinoma 2767:Ovarian serous cystadenoma 2718:Signet ring cell carcinoma 2133:Colon, Polyposis Syndromes 2128:eMedicine Gastroenterology 1786:10.1136/gutjnl-2014-307235 1548:10.1007/s12105-019-01104-3 998:Comparison of FAP variants 847:esophagogastroduodenoscopy 670:of a tubular adenoma, the 568:MUTYH-associated polyposis 565: 499:adenomatous polyposis coli 483:APC gene mutation variants 305:MUTYH-associated polyposis 251:MUTYH-associated polyposis 154:MUTYH-associated polyposis 4373: 4060:Barraquer–Simons syndrome 3953:Dilated cardiomyopathy 1I 3699:Weill–Marchesani syndrome 3324:Focal nodular hyperplasia 2852:Lobular carcinoma in situ 2775:Serous cystadenocarcinoma 2612:Clear-cell adenocarcinoma 2401:Bartholin gland carcinoma 2312:Glandular and epithelial 2020:10.1016/j.cgh.2007.09.017 1910:10.1186/s12876-016-0494-4 1022: 1017: 1012: 1007: 621:gene are inherited in an 609:do not accumulate in the 57: 48: 4203:Spinocerebellar ataxia 5 4027:Mandibuloacral dysplasia 3595:Freeman–Sheldon syndrome 3426:Mucinous cystic neoplasm 3276:Hepatocellular carcinoma 3130:sessile serrated adenoma 2880:Medullary thyroid cancer 2813:Mammary ductal carcinoma 2748:Mucoepidermoid carcinoma 2602:Adenoid cystic carcinoma 2569:Adrenocortical carcinoma 2509:Hepatocellular carcinoma 1579:Gardner EJ (June 1951). 1096:Colorectal cancer risk ( 1055:portion of the colon or 941:typically used to treat 474:, it is inherited in an 272:—a change in the body's 221:malignant transformation 4403:Gastrointestinal cancer 4342:Skin fragility syndrome 4284:Giant axonal neuropathy 3889:Steatocystoma multiplex 3465:Peritoneal mesothelioma 3214:Squamous cell carcinoma 2983:Squamous cell carcinoma 2358:Squamous-cell carcinoma 2120:Genetics Home Reference 1738:10.1136/gut.2006.117796 1647:10.1073/pnas.0611690104 1536:Head and Neck Pathology 939:ornithine decarboxylase 274:tumour suppressor genes 246:autosomal recessive FAP 117:Classical or attenuated 3628:Nemaline myopathy 4, 5 3306:Hepatocellular adenoma 3094:Pseudomyxoma peritonei 2741:Pseudomyxoma peritonei 2565:Adrenocortical adenoma 2505:Hepatocellular adenoma 1233:Thyroid cancer and FAP 1100:) and age if untreated 904:ileorectal anastomosis 770: 675: 495:tumour suppressor gene 418: 145:Differential diagnosis 4378:Cytoskeletal proteins 2903:Acinic cell carcinoma 2627:Papillary hidradenoma 1853:10.1056/NEJMoa1916063 765: 666: 416: 318:, or proximal to the 3311:Cavernous hemangioma 3162:Familial adenomatous 3149:Polyposis syndromes: 2731:Mucinous cystadenoma 2590:Neuroendocrine tumor 2527:Renal cell carcinoma 2363:Basal-cell carcinoma 2343:Small-cell carcinoma 1897:BMC Gastroenterology 1359:polyposis registries 1344:Polyposis registries 1142:Other lifetime risks 1047:Hundreds / thousands 1024:MUTYH Associated FAP 603:base excision repair 359:, especially in the 183:1 in 10,000 - 15,000 4085:Pelger–Huet anomaly 3995:Parkinson's disease 3716:Boomerang dysplasia 3651:Nemaline myopathy 1 3605:May–Hegglin anomaly 3562:Nemaline myopathy 3 2867:Medullary carcinoma 2353:Verrucous carcinoma 1638:2007PNAS..104.4036A 1490:GeneReviews NBK1345 1293:Decision to monitor 1212:Genotype–Phenotype 1187:Genotype-Phenotype 742:of the abdomen and 697:virtual colonoscopy 645:conditional mutants 623:autosomal recessive 299:The third variant, 233:(originally called 207:form mainly in the 101:<35 years of age 4408:Hereditary cancers 4254:Gardner's syndrome 4223:Long QT syndrome 4 3899:Familial cirrhosis 3869:White sponge nevus 3839:Familial cirrhosis 3819:White sponge nevus 3371:Gallbladder cancer 3353:Cholangiocarcinoma 3293:Liver angiosarcoma 3227:Upper and/or lower 3176:Serrated polyposis 2700:Cystadenocarcinoma 2532:Endometrioid tumor 2495:Cholangiocarcinoma 2383:Complex epithelial 2373:Inverted papilloma 2245:External resources 1386:Adenomatous polyps 1325:Germline mosaicism 937:, an inhibitor of 771: 734:genetic counseling 722:surveillance with 707:genetic counseling 676: 522:two-hit hypothesis 476:autosomal dominant 456:Gardner's syndrome 419: 406:is preferred over 343:Signs and symptoms 328:genetic blood test 198:autosomal dominant 4385: 4384: 4310:Carvajal syndrome 4185: 4184: 4100: 4099: 3964:Alexander disease 3736: 3735: 3677: 3676: 3590:Usher syndrome 1B 3580:Elejalde syndrome 3478: 3477: 3446: 3445: 3438:Pancreatoblastoma 3390:exocrine pancreas 3247: 3246: 3222: 3221: 3041: 3040: 3006:Gastric carcinoma 2915: 2914: 2911: 2910: 2888: 2887: 2844:Lobular carcinoma 2788: 2787: 2635: 2634: 2409: 2408: 2279: 2278: 1846:(11): 1028–1039. 1434:Am. J. Hum. Genet 1371:Colorectal cancer 1341: 1340: 1221:Duodenal adenomas 911:partial colectomy 861:may be required. 828:somatic mosaicism 672:colorectal cancer 617:Mutations in the 389:colorectal cancer 349:colorectal polyps 187: 186: 131:Diagnostic method 125:APC gene mutation 92:Colorectal cancer 30:Medical condition 16:(Redirected from 4415: 4398:Benign neoplasms 4376:Related topics: 4111: 3747: 3535: 3526: 3505: 3498: 3491: 3482: 3258: 3157:MUTYH-associated 3142:Cronkhite–Canada 3113:Colorectal polyp 3052: 3028:Linitis plastica 3016:Gastric lymphoma 2971: 2962: 2942: 2935: 2928: 2919: 2835: 2805:Ductal carcinoma 2801: 2781: 2737: 2723:Krukenberg tumor 2683: 2537:Renal oncocytoma 2449:Linitis plastica 2438:Gastrointestinal 2434: 2420: 2327: 2306: 2299: 2292: 2283: 2149: 2107: 2077: 2076: 2065:10.1157/13106937 2053:Medicina ClĂ­nica 2048: 2042: 2041: 2031: 1999: 1993: 1992: 1990: 1988: 1974: 1968: 1967: 1965: 1964: 1947: 1941: 1940: 1930: 1912: 1888: 1882: 1881: 1855: 1830: 1824: 1823: 1797: 1764: 1758: 1757: 1717: 1711: 1710: 1700: 1685:Gastroenterology 1676: 1670: 1669: 1659: 1649: 1617: 1611: 1610: 1600: 1576: 1570: 1569: 1559: 1527: 1521: 1520: 1518: 1517: 1501: 1492: 1487: 1468: 1467: 1457: 1440:(6): 1290–1301. 1425: 1391:Colorectal polyp 1376:Polyp (medicine) 1202: 1198: 1005: 966:by the surgery. 851:duodenal cancers 730:Prenatal testing 515:intestinal tract 427:Gardner fibromas 357:intestinal tract 353:polyps elsewhere 270:genetic mutation 237:) are caused by 76:Gastroenterology 53: 33: 21: 4423: 4422: 4418: 4417: 4416: 4414: 4413: 4412: 4388: 4387: 4386: 4381: 4369: 4289: 4233: 4181: 4160: 4132: 4096: 4075:Osteopoikilosis 4009: 3979: 3932: 3732: 3721:Larsen syndrome 3694:Marfan syndrome 3673: 3655: 3632: 3609: 3566: 3515: 3509: 3479: 3474: 3442: 3375: 3333: 3243: 3218: 3200: 3098: 3075: 3064:Duodenal cancer 3056:Small intestine 3037: 2992: 2951: 2946: 2916: 2907: 2884: 2861: 2838: 2827: 2823:Comedocarcinoma 2796: 2794: 2784: 2765: 2752: 2729: 2704: 2678: 2676: 2670: 2641: 2631: 2578: 2541: 2513: 2486:Somatostatinoma 2429:adenocarcinomas 2427: 2405: 2391:Warthin's tumor 2377: 2316: 2310: 2280: 2275: 2274: 2240: 2239: 2160: 2146: 2089: 2086: 2084:Further reading 2081: 2080: 2050: 2049: 2045: 2001: 2000: 1996: 1986: 1984: 1976: 1975: 1971: 1962: 1960: 1949: 1948: 1944: 1890: 1889: 1885: 1832: 1831: 1827: 1766: 1765: 1761: 1719: 1718: 1714: 1678: 1677: 1673: 1632:(10): 4036–41. 1619: 1618: 1614: 1578: 1577: 1573: 1529: 1528: 1524: 1515: 1513: 1503: 1502: 1495: 1488: 1471: 1427: 1426: 1409: 1404: 1396:Genetic testing 1367: 1346: 1200: 1196: 1000: 987: 959: 943:trypanosomiasis 899:ileo-anal pouch 875: 836: 819: 760: 703:Genetic testing 661: 632: 583:MYH glycosylase 570: 564: 485: 465: 451:sebaceous cysts 439:sebaceous cysts 361:large intestine 351:(and sometimes 345: 320:splenic flexure 316:ascending colon 213:large intestine 172: 170:Upper endoscopy 168: 164: 139:Genetic testing 138: 31: 28: 23: 22: 15: 12: 11: 5: 4421: 4419: 4411: 4410: 4405: 4400: 4390: 4389: 4383: 4382: 4374: 4371: 4370: 4368: 4367: 4350: 4349: 4344: 4334: 4333: 4328: 4318: 4317: 4312: 4307: 4297: 4295: 4291: 4290: 4288: 4287: 4275: 4272:Naxos syndrome 4263: 4262: 4261: 4256: 4243: 4241: 4235: 4234: 4232: 4231: 4216: 4215: 4210: 4205: 4195: 4193: 4187: 4186: 4183: 4182: 4180: 4179: 4174: 4168: 4166: 4162: 4161: 4159: 4158: 4153: 4148: 4142: 4140: 4134: 4133: 4131: 4130: 4125: 4119: 4117: 4108: 4102: 4101: 4098: 4097: 4095: 4094: 4093: 4092: 4087: 4079: 4078: 4077: 4072: 4064: 4063: 4062: 4054: 4053: 4052: 4047: 4042: 4037: 4029: 4017: 4015: 4011: 4010: 4008: 4007: 4002: 3997: 3987: 3985: 3981: 3980: 3978: 3977: 3967: 3966: 3956: 3955: 3950: 3940: 3938: 3934: 3933: 3931: 3930: 3925: 3920: 3902: 3892: 3882: 3872: 3862: 3852: 3842: 3832: 3822: 3812: 3802: 3792: 3791: 3790: 3785: 3780: 3769:hyperkeratosis 3753: 3751: 3744: 3738: 3737: 3734: 3733: 3731: 3730: 3729: 3728: 3723: 3718: 3713: 3703: 3702: 3701: 3696: 3685: 3683: 3679: 3678: 3675: 3674: 3672: 3671: 3665: 3663: 3657: 3656: 3654: 3653: 3648: 3642: 3640: 3634: 3633: 3631: 3630: 3625: 3619: 3617: 3611: 3610: 3608: 3607: 3602: 3597: 3592: 3587: 3582: 3576: 3574: 3568: 3567: 3565: 3564: 3559: 3554: 3549: 3543: 3541: 3532: 3523: 3521:Microfilaments 3517: 3516: 3510: 3508: 3507: 3500: 3493: 3485: 3476: 3475: 3473: 3472: 3467: 3462: 3456: 3454: 3448: 3447: 3444: 3443: 3441: 3440: 3434: 3433: 3428: 3423: 3418: 3403: 3402: 3397: 3395:Adenocarcinoma 3385: 3383: 3377: 3376: 3374: 3373: 3361: 3360: 3358:Klatskin tumor 3355: 3343: 3341: 3335: 3334: 3332: 3331: 3326: 3314: 3313: 3308: 3296: 3295: 3290: 3288:Hepatoblastoma 3285: 3284: 3283: 3266: 3264: 3255: 3249: 3248: 3245: 3244: 3242: 3241: 3236: 3230: 3228: 3224: 3223: 3220: 3219: 3217: 3216: 3210: 3208: 3202: 3201: 3199: 3198: 3193: 3188: 3186:Adenocarcinoma 3179: 3178: 3173: 3168: 3159: 3154: 3145: 3144: 3108: 3106: 3100: 3099: 3097: 3096: 3091: 3085: 3083: 3077: 3076: 3074: 3073: 3072: 3071: 3069:Adenocarcinoma 3060: 3058: 3049: 3043: 3042: 3039: 3038: 3036: 3035: 3030: 3025: 3024: 3023: 3013: 3008: 3002: 3000: 2994: 2993: 2991: 2990: 2988:Adenocarcinoma 2985: 2979: 2977: 2968: 2959: 2953: 2952: 2947: 2945: 2944: 2937: 2930: 2922: 2913: 2912: 2909: 2908: 2906: 2905: 2899: 2897: 2890: 2889: 2886: 2885: 2883: 2882: 2877: 2871: 2869: 2863: 2862: 2860: 2859: 2854: 2848: 2846: 2840: 2839: 2837: 2836: 2825: 2820: 2815: 2809: 2807: 2798: 2790: 2789: 2786: 2785: 2783: 2782: 2762: 2760: 2754: 2753: 2751: 2750: 2745: 2744: 2743: 2727: 2726: 2725: 2714: 2712: 2706: 2705: 2703: 2702: 2692: 2690: 2680: 2672: 2671: 2669: 2668: 2663: 2662: 2661: 2656: 2645: 2643: 2642:skin appendage 2637: 2636: 2633: 2632: 2630: 2629: 2624: 2619: 2614: 2609: 2604: 2599: 2598: 2597: 2586: 2584: 2583:Other/multiple 2580: 2579: 2577: 2576: 2571: 2562: 2557: 2551: 2549: 2543: 2542: 2540: 2539: 2534: 2529: 2523: 2521: 2515: 2514: 2512: 2511: 2502: 2500:Klatskin tumor 2497: 2491: 2490: 2489: 2488: 2483: 2478: 2473: 2468: 2457: 2456: 2451: 2442: 2440: 2431: 2417: 2411: 2410: 2407: 2406: 2404: 2403: 2398: 2393: 2387: 2385: 2379: 2378: 2376: 2375: 2370: 2365: 2360: 2355: 2350: 2345: 2339: 2337: 2324: 2318: 2317: 2311: 2309: 2308: 2301: 2294: 2286: 2277: 2276: 2273: 2272: 2261: 2249: 2248: 2246: 2242: 2241: 2238: 2237: 2226: 2215: 2204: 2196: 2181: 2161: 2156: 2155: 2153: 2152:Classification 2145: 2144:External links 2142: 2141: 2140: 2135: 2130: 2121: 2109: 2085: 2082: 2079: 2078: 2055:(in Spanish). 2043: 1994: 1969: 1942: 1883: 1825: 1780:(2): 286–295. 1759: 1712: 1671: 1612: 1585:Am J Hum Genet 1571: 1522: 1493: 1469: 1446:10.1086/301883 1406: 1405: 1403: 1400: 1399: 1398: 1393: 1388: 1383: 1378: 1373: 1366: 1363: 1345: 1342: 1339: 1338: 1335: 1332: 1308: 1304: 1303: 1300: 1297: 1294: 1290: 1289: 1286: 1283: 1280: 1276: 1275: 1272: 1269: 1265: 1261: 1260: 1257: 1254: 1251: 1247: 1246: 1243: 1240: 1225:Desmoid tumors 1213: 1209: 1208: 1205: 1192: 1188: 1184: 1183: 1180: 1174: 1171: 1167: 1166: 1163: 1160: 1157: 1153: 1152: 1149: 1146: 1143: 1139: 1138: 1135: 1132: 1129: 1125: 1124: 1121: 1118: 1115: 1111: 1110: 1107: 1104: 1101: 1093: 1092: 1089: 1086: 1083: 1079: 1078: 1075: 1071: 1068: 1064: 1063: 1060: 1048: 1045: 1041: 1040: 1037: 1034: 1031: 1027: 1026: 1021: 1019:Attenuated FAP 1016: 1011: 999: 996: 986: 983: 958: 955: 915: 914: 907: 874: 871: 835: 832: 818: 817:Family history 815: 814: 813: 810: 805: 804: 803: 802: 799:histopathology 783: 782: 779: 759: 756: 754:to the liver. 748:liver function 712: 711: 700: 660: 657: 631: 628: 566:Main article: 563: 560: 551:organization. 484: 481: 464: 461: 431:desmoid tumors 400:duodenal tract 363:including the 344: 341: 324:thyroid cancer 230:attenuated FAP 185: 184: 181: 177: 176: 161: 157: 156: 150:Lynch syndrome 147: 141: 140: 133: 127: 126: 123: 119: 118: 115: 111: 110: 107: 103: 102: 99: 95: 94: 89: 83: 82: 73: 67: 66: 55: 54: 46: 45: 42: 38: 37: 29: 26: 24: 14: 13: 10: 9: 6: 4: 3: 2: 4420: 4409: 4406: 4404: 4401: 4399: 4396: 4395: 4393: 4380: 4379: 4372: 4365: 4361: 4360: 4355: 4352: 4351: 4348: 4345: 4343: 4339: 4336: 4335: 4332: 4329: 4327: 4323: 4320: 4319: 4316: 4313: 4311: 4308: 4306: 4302: 4299: 4298: 4296: 4292: 4285: 4281: 4280: 4276: 4273: 4269: 4268: 4264: 4260: 4257: 4255: 4252: 4251: 4250: 4249: 4245: 4244: 4242: 4240: 4236: 4230: 4227: 4226: 4225: 4224: 4220: 4214: 4211: 4209: 4206: 4204: 4200: 4197: 4196: 4194: 4192: 4188: 4178: 4175: 4173: 4170: 4169: 4167: 4163: 4157: 4154: 4152: 4149: 4147: 4144: 4143: 4141: 4139: 4135: 4129: 4126: 4124: 4121: 4120: 4118: 4116: 4112: 4109: 4107: 4103: 4091: 4088: 4086: 4083: 4082: 4080: 4076: 4073: 4071: 4068: 4067: 4065: 4061: 4058: 4057: 4055: 4051: 4048: 4046: 4043: 4041: 4038: 4036: 4033: 4030: 4028: 4025: 4024: 4022: 4019: 4018: 4016: 4012: 4006: 4003: 4001: 3998: 3996: 3992: 3991:Neurofilament 3989: 3988: 3986: 3982: 3976: 3972: 3969: 3968: 3965: 3961: 3958: 3957: 3954: 3951: 3949: 3945: 3942: 3941: 3939: 3935: 3929: 3926: 3924: 3921: 3918: 3914: 3910: 3906: 3903: 3900: 3896: 3893: 3890: 3886: 3883: 3880: 3876: 3873: 3870: 3866: 3863: 3860: 3856: 3853: 3850: 3846: 3843: 3840: 3836: 3833: 3830: 3826: 3823: 3820: 3816: 3813: 3810: 3806: 3803: 3800: 3796: 3793: 3789: 3786: 3784: 3781: 3779: 3776: 3775: 3774: 3770: 3766: 3762: 3758: 3757:Keratinopathy 3755: 3754: 3752: 3748: 3745: 3743: 3739: 3727: 3724: 3722: 3719: 3717: 3714: 3712: 3711:FG syndrome 2 3709: 3708: 3707: 3704: 3700: 3697: 3695: 3692: 3691: 3690: 3687: 3686: 3684: 3680: 3670: 3667: 3666: 3664: 3662: 3658: 3652: 3649: 3647: 3644: 3643: 3641: 3639: 3635: 3629: 3626: 3624: 3621: 3620: 3618: 3616: 3612: 3606: 3603: 3601: 3598: 3596: 3593: 3591: 3588: 3586: 3583: 3581: 3578: 3577: 3575: 3573: 3569: 3563: 3560: 3558: 3555: 3553: 3550: 3548: 3545: 3544: 3542: 3540: 3536: 3533: 3531: 3527: 3524: 3522: 3518: 3513: 3506: 3501: 3499: 3494: 3492: 3487: 3486: 3483: 3471: 3468: 3466: 3463: 3461: 3458: 3457: 3455: 3453: 3449: 3439: 3436: 3435: 3432: 3429: 3427: 3424: 3422: 3419: 3417: 3413: 3412: 3409: 3405: 3404: 3401: 3398: 3396: 3393: 3391: 3387: 3386: 3384: 3382: 3378: 3372: 3369: 3367: 3363: 3362: 3359: 3356: 3354: 3351: 3349: 3345: 3344: 3342: 3340: 3339:Biliary tract 3336: 3330: 3327: 3325: 3322: 3320: 3316: 3315: 3312: 3309: 3307: 3304: 3302: 3298: 3297: 3294: 3291: 3289: 3286: 3282: 3281:Fibrolamellar 3279: 3278: 3277: 3274: 3272: 3268: 3267: 3265: 3263: 3259: 3256: 3254: 3250: 3240: 3237: 3235: 3232: 3231: 3229: 3225: 3215: 3212: 3211: 3209: 3207: 3203: 3197: 3194: 3192: 3189: 3187: 3184: 3181: 3180: 3177: 3174: 3172: 3169: 3167: 3163: 3160: 3158: 3155: 3153: 3150: 3147: 3146: 3143: 3139: 3138:Peutz–Jeghers 3135: 3131: 3127: 3123: 3119: 3116: 3114: 3110: 3109: 3107: 3105: 3101: 3095: 3092: 3090: 3087: 3086: 3084: 3082: 3078: 3070: 3067: 3066: 3065: 3062: 3061: 3059: 3057: 3053: 3050: 3048: 3044: 3034: 3031: 3029: 3026: 3022: 3021:MALT lymphoma 3019: 3018: 3017: 3014: 3012: 3009: 3007: 3004: 3003: 3001: 2999: 2995: 2989: 2986: 2984: 2981: 2980: 2978: 2976: 2972: 2969: 2967: 2963: 2960: 2958: 2954: 2950: 2943: 2938: 2936: 2931: 2929: 2924: 2923: 2920: 2904: 2901: 2900: 2898: 2895: 2891: 2881: 2878: 2876: 2873: 2872: 2870: 2868: 2864: 2858: 2855: 2853: 2850: 2849: 2847: 2845: 2841: 2834: 2830: 2826: 2824: 2821: 2819: 2816: 2814: 2811: 2810: 2808: 2806: 2802: 2799: 2797:and medullary 2791: 2780: 2776: 2772: 2768: 2764: 2763: 2761: 2759: 2755: 2749: 2746: 2742: 2739: 2738: 2736: 2732: 2728: 2724: 2721: 2720: 2719: 2716: 2715: 2713: 2711: 2707: 2701: 2697: 2694: 2693: 2691: 2688: 2684: 2681: 2673: 2667: 2664: 2660: 2657: 2655: 2652: 2651: 2650: 2647: 2646: 2644: 2638: 2628: 2625: 2623: 2620: 2618: 2615: 2613: 2610: 2608: 2605: 2603: 2600: 2596: 2593: 2592: 2591: 2588: 2587: 2585: 2581: 2575: 2572: 2570: 2566: 2563: 2561: 2558: 2556: 2553: 2552: 2550: 2548: 2544: 2538: 2535: 2533: 2530: 2528: 2525: 2524: 2522: 2520: 2516: 2510: 2506: 2503: 2501: 2498: 2496: 2493: 2492: 2487: 2484: 2482: 2479: 2477: 2474: 2472: 2469: 2467: 2464: 2463: 2462: 2459: 2458: 2455: 2452: 2450: 2447: 2444: 2443: 2441: 2439: 2435: 2432: 2430: 2425: 2421: 2418: 2416: 2412: 2402: 2399: 2397: 2394: 2392: 2389: 2388: 2386: 2384: 2380: 2374: 2371: 2369: 2366: 2364: 2361: 2359: 2356: 2354: 2351: 2349: 2346: 2344: 2341: 2340: 2338: 2336: 2332: 2328: 2325: 2323: 2319: 2315: 2307: 2302: 2300: 2295: 2293: 2288: 2287: 2284: 2271: 2267: 2266: 2262: 2260: 2256: 2255: 2251: 2250: 2247: 2243: 2236: 2232: 2231: 2227: 2225: 2221: 2220: 2216: 2214: 2210: 2209: 2205: 2202: 2201: 2197: 2195: 2191: 2190: 2186: 2182: 2180: 2176: 2172: 2171: 2167: 2163: 2162: 2159: 2154: 2150: 2143: 2139: 2136: 2134: 2131: 2129: 2125: 2122: 2119: 2115: 2114: 2110: 2105: 2101: 2097: 2093: 2088: 2087: 2083: 2074: 2070: 2066: 2062: 2058: 2054: 2047: 2044: 2039: 2035: 2030: 2025: 2021: 2017: 2013: 2009: 2005: 1998: 1995: 1983: 1979: 1973: 1970: 1958: 1957: 1952: 1946: 1943: 1938: 1934: 1929: 1924: 1920: 1916: 1911: 1906: 1902: 1898: 1894: 1887: 1884: 1879: 1875: 1871: 1867: 1863: 1859: 1854: 1849: 1845: 1841: 1837: 1829: 1826: 1821: 1817: 1813: 1809: 1805: 1801: 1796: 1795:10044/1/49491 1791: 1787: 1783: 1779: 1775: 1771: 1763: 1760: 1755: 1751: 1747: 1743: 1739: 1735: 1731: 1727: 1723: 1716: 1713: 1708: 1704: 1699: 1694: 1691:(6): 1542–7. 1690: 1686: 1682: 1675: 1672: 1667: 1663: 1658: 1653: 1648: 1643: 1639: 1635: 1631: 1627: 1623: 1616: 1613: 1608: 1604: 1599: 1594: 1591:(2): 167–76. 1590: 1586: 1582: 1575: 1572: 1567: 1563: 1558: 1553: 1549: 1545: 1541: 1537: 1533: 1526: 1523: 1512: 1511: 1506: 1500: 1498: 1494: 1491: 1486: 1484: 1482: 1480: 1478: 1476: 1474: 1470: 1465: 1461: 1456: 1451: 1447: 1443: 1439: 1435: 1431: 1424: 1422: 1420: 1418: 1416: 1414: 1412: 1408: 1401: 1397: 1394: 1392: 1389: 1387: 1384: 1382: 1379: 1377: 1374: 1372: 1369: 1368: 1364: 1362: 1360: 1356: 1352: 1343: 1336: 1333: 1330: 1326: 1322: 1318: 1314: 1309: 1306: 1305: 1301: 1298: 1295: 1292: 1291: 1287: 1284: 1281: 1278: 1277: 1273: 1270: 1266: 1263: 1262: 1258: 1255: 1252: 1249: 1248: 1244: 1241: 1238: 1234: 1230: 1226: 1222: 1218: 1214: 1211: 1210: 1206: 1193: 1189: 1186: 1185: 1181: 1179: 1175: 1172: 1169: 1168: 1164: 1161: 1158: 1155: 1154: 1150: 1147: 1144: 1141: 1140: 1136: 1133: 1130: 1127: 1126: 1122: 1119: 1116: 1113: 1112: 1108: 1105: 1102: 1099: 1095: 1094: 1090: 1087: 1084: 1081: 1080: 1076: 1072: 1069: 1066: 1065: 1061: 1058: 1054: 1049: 1046: 1043: 1042: 1038: 1035: 1032: 1029: 1028: 1025: 1020: 1015: 1010: 1006: 1003: 997: 995: 991: 984: 982: 979: 977: 972: 967: 965: 956: 954: 952: 948: 944: 940: 936: 932: 927: 924: 922: 919: 912: 908: 905: 900: 896: 892: 888: 887: 886: 884: 880: 872: 870: 868: 862: 860: 856: 852: 848: 844: 841: 833: 831: 829: 825: 816: 811: 807: 806: 800: 796: 792: 787: 786: 785: 784: 780: 777: 776: 775: 768: 764: 757: 755: 753: 749: 745: 741: 737: 735: 731: 727: 726:is required. 725: 721: 716: 708: 704: 701: 698: 694: 690: 685: 682: 681: 680: 673: 669: 665: 658: 656: 652: 650: 646: 641: 637: 630:Animal models 629: 627: 624: 620: 615: 612: 608: 604: 600: 596: 592: 588: 584: 580: 576: 575: 569: 561: 559: 557: 552: 550: 546: 541: 539: 535: 531: 527: 523: 518: 516: 512: 508: 504: 500: 496: 492: 491: 482: 480: 477: 473: 471: 462: 460: 458: 457: 452: 448: 444: 440: 436: 432: 428: 424: 415: 411: 409: 408:sigmoidoscopy 405: 401: 396: 392: 390: 386: 382: 378: 374: 370: 366: 362: 358: 354: 350: 342: 340: 338: 333: 329: 325: 321: 317: 312: 310: 306: 302: 297: 294: 290: 288: 282: 280: 275: 271: 266: 264: 259: 258: 253: 252: 247: 243: 241: 236: 232: 231: 226: 222: 218: 214: 210: 206: 203: 199: 195: 191: 182: 178: 175: 171: 167: 162: 158: 155: 151: 148: 146: 142: 137: 134: 132: 128: 124: 120: 116: 112: 108: 104: 100: 96: 93: 90: 88: 87:Complications 84: 81: 77: 74: 72: 68: 64: 63:sigmoid colon 60: 56: 52: 47: 43: 39: 34: 19: 4375: 4357: 4277: 4265: 4258: 4246: 4217: 4106:Microtubules 3512:Cytoskeletal 3406: 3388: 3364: 3346: 3317: 3299: 3269: 3190: 3182: 3161: 3148: 3122:hyperplastic 3111: 3104:Colon/rectum 2654:Hidrocystoma 2648: 2574:HĂĽrthle cell 2555:Prolactinoma 2460: 2453: 2445: 2263: 2252: 2228: 2217: 2206: 2198: 2183: 2164: 2111: 2095: 2056: 2052: 2046: 2014:(1): 46–52. 2011: 2007: 1997: 1985:. Retrieved 1981: 1972: 1961:. Retrieved 1959:. 2020-09-02 1954: 1945: 1900: 1896: 1886: 1843: 1839: 1828: 1777: 1773: 1762: 1729: 1725: 1715: 1688: 1684: 1674: 1629: 1625: 1615: 1588: 1584: 1574: 1542:(1): 43–58. 1539: 1535: 1525: 1514:. Retrieved 1508: 1437: 1433: 1347: 1320: 1316: 1312: 1236: 1232: 1228: 1224: 1220: 1216: 1177: 1056: 1052: 1023: 1018: 1013: 1008: 1001: 992: 988: 985:Epidemiology 980: 968: 963: 960: 935:eflornithine 928: 925: 918:Prophylactic 916: 883:prophylactic 876: 863: 837: 823: 820: 794: 793:number or a 790: 772: 738: 728: 720:colonoscopic 717: 713: 693:Barium enema 677: 653: 639: 635: 633: 618: 616: 605:, such that 572: 571: 555: 554:Mutation of 553: 549:cytoskeleton 545:cell nucleus 542: 533: 519: 488: 486: 469: 466: 454: 420: 397: 393: 346: 336: 331: 313: 304: 300: 298: 292: 285: 283: 267: 255: 249: 245: 239: 234: 228: 225:colon cancer 193: 189: 188: 163:Colonoscopy 4338:plakophilin 4301:desmoplakin 4267:plakoglobin 4021:Laminopathy 3917:Monilethrix 3765:keratoderma 3638:Tropomyosin 3530:Myofilament 3366:gallbladder 3319:hyperplasia 2696:Cystadenoma 2649:sweat gland 2640:Adnexal and 2471:Glucagonoma 2096:GeneReviews 2059:(2): 51–2. 1732:(1): 71–6. 1510:MedlinePlus 1361:now exist. 1162:Same as FAP 1156:Inheritance 1114:Variability 893:(permanent 843:colonoscopy 791:substantial 746:evaluating 744:blood tests 724:polypectomy 684:Colonoscopy 597:instead of 404:Colonoscopy 377:weight loss 202:adenomatous 166:Polypectomy 136:Colonoscopy 98:Usual onset 41:Other names 4392:Categories 4354:centrosome 3971:Peripherin 3452:Peritoneum 2679:and serous 2622:Cylindroma 2607:Oncocytoma 2519:Urogenital 2476:Gastrinoma 2466:Insulinoma 2322:Epithelium 2230:DiseasesDB 2106:. NBK1345. 1963:2023-08-19 1956:Cancer.Net 1516:2023-06-09 1402:References 1268:developed" 1250:Prevalence 1098:penetrance 976:metastasis 974:return or 840:outpatient 834:Monitoring 758:Management 752:metastasis 740:Ultrasound 689:histologic 668:Micrograph 579:DNA repair 538:epithelial 453:is termed 381:metastasis 287:attenuated 263:metastasis 209:epithelium 59:Endoscopic 4172:Tauopathy 3761:keratosis 3689:Fibrillin 3411:neoplasms 3348:bile duct 3271:malignant 3253:Accessory 3183:Neoplasm: 3166:Gardner's 3089:Carcinoid 2975:Esophagus 2677:mucinous, 2659:Syringoma 2595:Carcinoid 2547:Endocrine 2335:carcinoma 2331:Papilloma 2254:eMedicine 1919:1471-230X 1903:(1): 87. 1878:221620374 1862:0028-4793 1804:0017-5749 971:colectomy 957:Prognosis 947:celecoxib 921:colectomy 891:ileostomy 873:Treatment 795:profusion 767:Colectomy 659:Diagnosis 607:mutations 503:β-catenin 443:osteomata 180:Frequency 174:Colectomy 160:Treatment 71:Specialty 61:image of 4199:Spectrin 4191:Membrane 4032:Dunnigan 3615:Troponin 3381:Pancreas 3152:Juvenile 3126:juvenile 3081:Appendix 2957:GI tract 2795:lobular, 2710:Mucinous 2461:pancreas 2424:Adenomas 2265:Orphanet 2203:: 8220/0 2104:20301519 2073:17588361 2038:18063416 1937:27480131 1870:32905675 1812:25792707 1754:25082664 1746:17604324 1666:17360473 1607:14902760 1566:31950474 1365:See also 1351:probands 951:sulindac 879:genotype 599:cytosine 577:encodes 463:Genetics 447:fibromas 291:has the 196:) is an 109:Lifelong 106:Duration 80:Oncology 4322:plectin 4239:Catenin 4219:Ankyrin 4115:Kinesin 4023:: LMNA 3706:Filamin 3514:defects 3118:adenoma 2998:Stomach 2793:Ductal, 2689:general 2675:Cystic, 2617:Apudoma 2396:Thymoma 2259:med/769 2224:D011125 2029:2245898 1987:22 July 1928:4969736 1820:8059715 1707:8194700 1657:1805486 1634:Bibcode 1598:1716321 1557:7021862 1464:9585611 1455:1377162 1381:Adenoma 1334:See FAP 1321:de novo 1317:de novo 1313:de novo 1120:See FAP 964:in toto 867:proband 859:surgery 855:excised 824:de novo 595:adenine 587:guanine 581:enzyme 540:cells. 423:stomach 383:to the 330:of the 211:of the 4138:Dynein 4066:LEMD3 3944:Desmin 3572:Myosin 3557:DFNA20 3408:cystic 3301:benign 2894:Acinar 2758:Serous 2687:Cystic 2481:VIPoma 2446:tract: 2415:Glands 2314:cancer 2213:175100 2102:  2071:  2036:  2026:  1935:  1925:  1917:  1876:  1868:  1860:  1818:  1810:  1802:  1752:  1744:  1705:  1664:  1654:  1605:  1595:  1564:  1554:  1462:  1452:  1353:. The 1039:MUTYH 591:oxygen 526:allele 511:polyps 441:, and 435:retina 373:anemia 369:rectum 279:cancer 217:benign 205:polyps 122:Causes 4294:Other 4165:Other 4056:LMNB 3913:KRT86 3909:KRT83 3905:KRT81 3895:KRT18 3885:KRT17 3875:KRT14 3865:KRT13 3855:KRT12 3845:KRT10 3795:KRT2E 3682:Other 3661:Titin 3539:Actin 3262:Liver 3047:Lower 2966:Upper 2200:ICD-O 2194:211.3 1874:S2CID 1816:S2CID 1750:S2CID 1229:CHRPE 1204:well. 1074:years 1057:upper 1053:upper 895:stoma 634:The " 619:MUTYH 574:MUTYH 493:is a 385:liver 365:colon 257:MUTYH 223:into 114:Types 4359:PCNT 4081:LBR 3960:GFAP 3835:KRT8 3825:KRT5 3815:KRT4 3805:KRT3 3788:IHCM 3773:KRT1 3206:Anus 2896:cell 2235:4678 2219:MeSH 2208:OMIM 2189:9-CM 2100:PMID 2069:PMID 2034:PMID 1989:2021 1933:PMID 1915:ISSN 1866:PMID 1858:ISSN 1808:PMID 1800:ISSN 1742:PMID 1703:PMID 1662:PMID 1603:PMID 1562:PMID 1460:PMID 1030:Gene 1009:Item 695:and 534:kRAS 487:The 472:gene 449:and 429:and 248:(or 242:gene 4279:GAN 4248:APC 3771:): 3750:1/2 2270:733 2185:ICD 2179:D12 2175:C18 2166:ICD 2118:NLM 2116:at 2061:doi 2057:129 2024:PMC 2016:doi 1923:PMC 1905:doi 1848:doi 1844:383 1790:hdl 1782:doi 1774:Gut 1734:doi 1726:Gut 1693:doi 1689:106 1652:PMC 1642:doi 1630:104 1593:PMC 1552:PMC 1544:doi 1450:PMC 1442:doi 1329:DNA 1036:APC 1033:APC 1014:FAP 640:Apc 636:Apc 611:DNA 556:APC 532:or 530:p53 490:APC 470:APC 367:or 337:APC 332:APC 303:or 293:APC 240:APC 194:FAP 44:FAP 4394:: 4356:: 4340:: 4324:: 4303:: 4221:: 4201:: 3993:: 3973:: 3962:: 3946:: 3767:, 3763:, 3742:IF 3414:: 3140:, 3136:, 3132:, 3128:, 3124:, 3120:, 2831:/ 2777:/ 2773:/ 2769:/ 2733:/ 2268:: 2257:: 2233:: 2222:: 2211:: 2192:: 2177:, 2173:: 2170:10 2067:. 2032:. 2022:. 2010:. 2006:. 1980:. 1953:. 1931:. 1921:. 1913:. 1901:16 1899:. 1895:. 1872:. 1864:. 1856:. 1842:. 1838:. 1814:. 1806:. 1798:. 1788:. 1778:65 1776:. 1772:. 1748:. 1740:. 1730:57 1728:. 1724:. 1701:. 1687:. 1683:. 1660:. 1650:. 1640:. 1628:. 1624:. 1601:. 1587:. 1583:. 1560:. 1550:. 1540:14 1538:. 1534:. 1507:. 1496:^ 1472:^ 1458:. 1448:. 1438:62 1436:. 1432:. 1410:^ 1337:? 1302:? 1288:? 1274:? 1259:? 1245:? 1207:? 1182:? 1151:? 1137:? 1123:? 1109:? 1091:? 1077:? 1062:? 906:). 736:. 651:. 517:. 391:. 219:, 152:, 78:, 4366:) 4362:( 4286:) 4282:( 4274:) 4270:( 4014:5 3984:4 3937:3 3919:) 3915:( 3911:/ 3907:/ 3901:) 3897:( 3891:) 3887:( 3881:) 3877:( 3871:) 3867:( 3861:) 3857:( 3851:) 3847:( 3841:) 3837:( 3831:) 3827:( 3821:) 3817:( 3811:) 3807:( 3801:) 3797:( 3759:( 3504:e 3497:t 3490:v 3392:: 3368:: 3350:: 3321:: 3303:: 3273:: 3164:/ 3115:: 2941:e 2934:t 2927:v 2698:/ 2567:/ 2507:/ 2426:/ 2333:/ 2305:e 2298:t 2291:v 2187:- 2168:- 2158:D 2126:— 2075:. 2063:: 2040:. 2018:: 2012:6 1991:. 1966:. 1939:. 1907:: 1880:. 1850:: 1822:. 1792:: 1784:: 1756:. 1736:: 1709:. 1695:: 1668:. 1644:: 1636:: 1609:. 1589:3 1568:. 1546:: 1519:. 1466:. 1444:: 1331:. 1242:? 1201:′ 1197:′ 1148:? 1088:? 902:( 192:( 20:)

Index

Familial Adenomatous Polyposis

Endoscopic
sigmoid colon
Specialty
Gastroenterology
Oncology
Complications
Colorectal cancer
Diagnostic method
Colonoscopy
Differential diagnosis
Lynch syndrome
MUTYH-associated polyposis
Polypectomy
Upper endoscopy
Colectomy
autosomal dominant
adenomatous
polyps
epithelium
large intestine
benign
malignant transformation
colon cancer
attenuated FAP
APC gene
MUTYH-associated polyposis
MUTYH
metastasis

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