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In familial adenomatous polyposis a person inherits a fault in the APC gene and grows hundreds or thousands of polyps in the large bowel from their teens. Left alone, almost all of them become cancer by about 40, so the bowel is watched from childhood and usually removed before that happens; the cancers that do occur are treated like ordinary bowel cancer.
What it is. Familial adenomatous polyposis is an inherited condition caused by a germline variant in APC, the gatekeeper of the Wnt pathway whose loss also opens the sporadic adenoma-carcinoma sequence. Carriers develop hundreds to thousands of colorectal adenomas from adolescence; an attenuated form produces fewer polyps later in life. The World Health Organization lists it among the genetic tumour syndromes of the digestive system. It is not only a bowel condition: duodenal and gastric polyposis occurs in almost all patients, desmoid tumours are a leading cause of death after colectomy, and thyroid cancer, hepatoblastoma and medulloblastoma occur (Nagtegaal 2020; Gastrointest Endosc Clin N Am 2022).
How it differs from its parent. The cancer that arises in polyposis is an ordinary colorectal adenocarcinoma; what differs is everything before and after it. Cancer Research UK records that almost all untreated patients develop bowel cancer by age 40, against a median age at diagnosis of 66 in the United States for colorectal cancer generally. The tumours are the endpoint of a field of hundreds of adenomas rather than of one, which is why segmental resection is rarely enough and why surveillance of the retained rectum or ileal pouch continues for life after surgery (Best Pract Res Clin Gastroenterol 2022).
How common it is. Fewer than 1 percent of bowel cancers (Cancer Research UK). Because carriers are identified and operated on before cancer develops, the share of cancers is much smaller than the share of the polyp burden.
How it is treated. Colonoscopy is recommended from about age 10 to 12 at intervals of one to two years, and prophylactic colectomy is the intervention that prevents the cancer; after colectomy, endoscopic surveillance of the retained rectum or the ileal pouch continues, as does upper gastrointestinal surveillance with visualisation of the ampulla, because duodenal and ampullary cancer becomes the leading gastrointestinal risk once the colon is gone (Gastrointest Endosc Clin N Am 2022; Best Pract Res Clin Gastroenterol 2022). The type and timing of colectomy are not settled by consensus. A cancer that does occur is staged and treated as colorectal adenocarcinoma, with the systemic rows of the parent page. UK surveillance and surgical practice follow the British Society of Gastroenterology, ACPGBI and UK Cancer Genetics Group guidelines (Monahan 2020).
Familial adenomatous polyposis accounts for fewer than 1 percent of bowel cancers, and almost all untreated patients develop bowel cancer by age 40 (Cancer Research UK).
Right-sided tumours behave differently from left-sided and rectal ones; the colon drains along its mesenteric vessels, the rectum into the mesorectum and pelvic side wall.
Same organ: Colon cancer (adenocarcinoma of the colon), Micropapillary adenocarcinoma of the colon and rectum, Adenoma-like adenocarcinoma of the colon and rectum, Lynch syndrome-associated colorectal cancer, Mucinous adenocarcinoma of the colon and rectum, Signet ring cell carcinoma of the colon and rectum, Medullary carcinoma of the colon, Serrated adenocarcinoma of the colon and rectum, Peritoneal mesothelioma, Colorectal cancer, Rectal cancer, Mismatch-repair deficient (MSI-high) colorectal cancer, BRAF V600E-mutant colorectal cancer, HER2-amplified colorectal cancer, KRAS G12C-mutant colorectal cancer, Early-onset colorectal cancer (under 50), Anal cancer (squamous cell carcinoma), Appendiceal cancer and pseudomyxoma peritonei, Small intestine cancer (small bowel adenocarcinoma), Small intestinal neuroendocrine tumours, Anal high-grade squamous intraepithelial lesions (precursor), Localised anal squamous cell carcinoma (stage I to III), Metastatic and recurrent anal squamous cell carcinoma, Low-grade appendiceal mucinous neoplasm and pseudomyxoma peritonei, Appendiceal adenocarcinoma (mucinous and non-mucinous, including signet ring cell), Goblet cell adenocarcinoma of the appendix, Localised small bowel adenocarcinoma (stage I to III, resected), Advanced and metastatic small bowel adenocarcinoma
Colonoscopy from about age 10 to 12 at one to two-yearly intervals; prophylactic colectomy before cancer develops, with the type and timing decided case by case; lifelong endoscopic surveillance of the retained rectum or ileal pouch afterwards; upper gastrointestinal endoscopy with ampullary visualisation at intervals set by duodenal and gastric findings.
Staged and treated as colorectal adenocarcinoma of the same stage, with the extent of resection decided by the field of polyps rather than by the single tumour; the parent record carries the systemic therapy rows.
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Query for this cancer: (TITLE:"Familial adenomatous polyposis-associated colorectal cancer" OR ABSTRACT:"Familial adenomatous polyposis-associated colorectal cancer" OR TITLE:"Familial adenomatous polyposis-associated colorectal cancer inherited APC variant; fewer than 1 percent of bowel cancers" OR ABSTRACT:"Familial adenomatous polyposis-associated colorectal cancer inherited APC variant; fewer than 1 percent of bowel cancers" OR TITLE:"FAP colorectal cancer" OR ABSTRACT:"FAP colorectal cancer" OR TITLE:"Polyposis coli" OR ABSTRACT:"Polyposis coli" OR TITLE:"Adenomatous polyposis coli bowel cancer" OR ABSTRACT:"Adenomatous polyposis coli bowel cancer" OR TITLE:"Attenuated familial adenomatous polyposis" OR ABSTRACT:"Attenuated familial adenomatous polyposis") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Familial adenomatous polyposis-associated colorectal cancer, not a curated reading list.
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Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
The bowel cancer regimens share low blood counts, tiredness, sickness and a sore mouth. Oxaliplatin adds cold-triggered tingling, irinotecan adds early and late diarrhoea, capecitabine adds hand-foot syndrome and needs a DPD test first, and cetuximab or panitumumab add an acne-like rash and low magnesium. The rule for all of them: ring the 24-hour number rather than wait.
See all on the product pages:CAPOX (capecitabine, oxaliplatin)FOLFOX (5-FU, leucovorin, oxaliplatin)·Printable cards in the navigator
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