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.. â—¦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
1190:
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.
993:
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
973:
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
961:
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
821:
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
686:
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
1348:
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
808:
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
334:
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
989:
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).
773:
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
1194:
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
1050:
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
295:
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
642:
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
709:
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
654:
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
1310:
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
864:
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
1267:
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
1203:
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.
2184:
2165:
714:
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.
394:
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
335:
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
478:
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).
1299:
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:
613:
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.
1833:
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).
962:
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
732:
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
339:
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.
4325:
2199:
2939:
547:
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.
398:
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
3858:
3808:
3502:
2303:
781:
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.
3170:
691:
diagnosis (cell/cancer type detection) and (d) where polyps exist, it can suggest whether outpatient excision (removal) is viable or surgery is recommended.
3927:
812:
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.
923:
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.
4346:
4314:
467:
Familial adenomatous polyposis can have different inheritance patterns and different genetic causes. When this condition results from mutations in the
3195:
994:
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.
710:
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
4122:
3999:
3947:
930:
774:
defective gene but as yet appears not to have any actual medical issue as a result of this). Clinical management can cover several areas:
3469:
3425:
2925:
2778:
2207:
347:
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.
981:
Desmoid tumors, with their infiltrative nature and potential proximity to vital structures, are the second highest cause of death.
50:
4402:
4150:
3328:
2828:
4069:
789:
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
4309:
4304:
4202:
4026:
3777:
3275:
3129:
2879:
2812:
2747:
2621:
2601:
2568:
2546:
2508:
2218:
220:
4084:
3604:
1215:
Prominent extracolonic manifestations often correlate (though not completely) with more distal APC mutations.
929:
Various medications are being investigated for slowing malignant degeneration of polyps, most prominently the
762:
1720:
Hes FJ, Nielsen M, Bik EC, Konvalinka D, Wijnen JT, Bakker E, Vasen HF, Breuning MH, Tops CM (January 2008).
1504:
1428:
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
909:
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).
1633:
1358:
826:
mutation, and of those with an apparent de novo APC mutation (i.e. no known family history) 20% have
747:
602:
582:
1977:
3715:
2866:
2518:
2352:
2188:
696:
622:
543:
The normal function of the APC gene product is still being investigated; it is present in both the
889:
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
729:
663:
514:
510:
356:
352:
348:
269:
204:
75:
1679:
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
4074:
3756:
3720:
3693:
3080:
3063:
2822:
2485:
1395:
942:
898:
850:
702:
426:
364:
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327:
319:
315:
212:
169:
2573:
410:
for this, as it provides better observation of the common right-side location of polyps.
2123:
1637:
1315:
mutations. Siblings have classic 50% risk of sharing the condition if inherited and not
4271:
3768:
3589:
3394:
3357:
3287:
3185:
3125:
3068:
2997:
2987:
2499:
2428:
2028:
2003:
1927:
1892:
1656:
1621:
1597:
1580:
1556:
1531:
1454:
1429:
798:
506:
450:
438:
430:
323:
308:
265:
outside the colon can greatly reduce and in many cases eliminate the spread of cancer.
229:
149:
2193:
528:
is mutated or deleted, accelerating generation of polyps. Further mutations (e.g., in
4391:
3990:
3520:
3338:
3020:
1877:
1697:
1680:
1231:
is associated with: mutations between codons 311 and 1444; whole APC gene deletions.
407:
62:
1753:
1134:
As for FAP but "CHRPE and desmoid tumors are rare" and also thyroid cancer is added.
3511:
3300:
2757:
2709:
2653:
2554:
1819:
934:
882:
692:
548:
544:
524:
has already taken place: the inherited APC mutation. Often, the remaining "normal"
2281:
2258:
502:
2223:
4337:
4300:
4266:
4105:
4020:
3916:
3764:
3710:
3637:
3529:
3365:
3318:
3261:
3205:
2695:
2470:
2382:
1509:
917:
842:
723:
719:
683:
403:
376:
314:
In some cases FAP can manifest higher in the colon than usual (for example, the
286:
227:
occurs when they are left untreated. Three variants are known to exist, FAP and
165:
135:
58:
1785:
1547:
437:("CHRPE—congenital hypertrophy of the retinal pigment epithelium"), jaw cysts,
4353:
3970:
3894:
3884:
3874:
3864:
3854:
3844:
3794:
3451:
2606:
2475:
2465:
2321:
2234:
2132:
2091:
2019:
1955:
1909:
1097:
975:
839:
822:
of the disease in the affected parent." In addition around 20% of cases are a
751:
743:
667:
578:
537:
489:
380:
262:
208:
2917:
1918:
1861:
1803:
1307:
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
2127:
1737:
1646:
970:
946:
920:
910:
890:
869:
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:
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731:
727:
726:is required.
725:
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708:
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690:
685:
682:
681:
680:
673:
669:
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637:
630:Animal models
629:
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448:
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428:
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415:
411:
409:
408:sigmoidoscopy
405:
401:
396:
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378:
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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:
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1674:
1629:
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1615:
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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:
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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::
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3993::
3973::
3962::
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3767:,
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3742:IF
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2831:/
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2067:.
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1980:.
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1538:.
1534:.
1507:.
1496:^
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1458:.
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