{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T09:09:51Z","timestamp":1790068191391,"version":"4.0.1"},"reference-count":24,"publisher":"BMJ","issue":"10","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Gut"],"published-print":{"date-parts":[[1993,10]]},"abstract":"<jats:p>Familial adenomatous polyposis has been associated with several extraintestinal cancers, but the relative and absolute risks of these malignancies have not been determined. Extraintestinal cancers reportedly associated with adenomatous polyposis (thyroid gland, adrenal gland, pancreas, and biliary tract) were identified in polyposis patients and their at risk relatives in The Johns Hopkins Registry. The incidence rates of identified tumours were then compared with the general population through person year analysis with adjustment for population. For comparison, the incidence rates of the two most common cancers not associated with polyposis (breast cancer in women and lung cancer) were also calculated. There was an increased relative risk of thyroid cancer (relative risk 7.6; 95% confidence limits (CL) 2.5-17.7) and pancreatic adenocarcinoma (relative risk 4.46; 95% CL 1.2-11.4) in polyposis patients and at risk relatives. The absolute risk was 26.8 and 21.4 cases\/100,000 person years, respectively. No cases of adrenal or biliary cancer were found in this cohort. There was no increased relative risk of lung cancer (95% CL 0.04-1.4) or breast cancer (95% CL 0.04-1.4) over the general population. The relative risks of thyroid and pancreatic cancer are increased in familial adenomatous polyposis, but the absolute lifetime risk is low. Screening for pancreatic cancer may not be worthwhile with currently available methods, but careful physical examination of the thyroid gland is warranted along with consideration for ultrasonography.<\/jats:p>","DOI":"10.1136\/gut.34.10.1394","type":"journal-article","created":{"date-parts":[[2007,9,18]],"date-time":"2007-09-18T17:41:31Z","timestamp":1190137291000},"page":"1394-1396","source":"Crossref","is-referenced-by-count":291,"title":["Increased risk of thyroid and pancreatic carcinoma in familial adenomatous polyposis."],"prefix":"10.1136","volume":"34","author":[{"given":"F M","family":"Giardiello","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G J","family":"Offerhaus","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D H","family":"Lee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A J","family":"Krush","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A C","family":"Tersmette","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S V","family":"Booker","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N C","family":"Kelley","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S R","family":"Hamilton","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[1993,10,1]]},"reference":[{"key":"ref_1","article-title":"Familial polyposis coli. Family studies, histopatholog, differential diagnosis, and results of treatment","author":"Bussey, H.J.R.","year":"1975","unstructured":"Bussey HJR. Familial polyposis coli. Family studies, histopatholog, differential diagnosis, and results of treatment. Baltimore, Maryland: Johns Hopkins University Press, 1975."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"665","DOI":"10.1126\/science.1651563","article-title":"Mutations of chromosome 5q21 genes in FAP and colorectal cancer patients","volume":"253","author":"Nishisho, I.; Nakamura, Y.; Miyoshi, Y.","year":"1991","unstructured":"Nishisho I, Nakamura Y, Miyoshi Y, et al. Mutations of chromosome 5q21 genes in FAP and colorectal cancer patients. Science 1991; 253: 665-9.","journal-title":"Science"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"661","DOI":"10.1126\/science.1651562","article-title":"Identification of FAP locus genes from chromosome 5q21","volume":"253","author":"Kinzler, K.W.; Nilbert, M.C.; Su, L.K.","year":"1991","unstructured":"Kinzler KW, Nilbert MC, Su LK, et al. Identification of FAP locus genes from chromosome 5q21. Science 1991; 253: 661-5.","journal-title":"Science"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"589","DOI":"10.1016\/0092-8674(81)90021-0","article-title":"Identification and characterization of the familial adenomatous polyposis coli gene","volume":"66","author":"Groden, J.; Thliveris, A.; Samowitz, W.","year":"1991","unstructured":"Groden J, Thliveris A, Samowitz W, et al. Identification and characterization of the familial adenomatous polyposis coli gene. Cell 1991; 66: 589-600.","journal-title":"Cell"},{"key":"ref_5","first-page":"589","article-title":"Identification of deletion mutations and three new genes at the familial polyposis locus","volume":"66","author":"Joslyn, G.; Carlson, M.; Thliveris, A.","year":"1991","unstructured":"Joslyn G, Carlson M, Thliveris A, et al. Identification of deletion mutations and three new genes at the familial polyposis locus. Cell 1991; 66: 589-600.","journal-title":"Cell"},{"key":"ref_6","first-page":"1500","article-title":"Itzkowitz SH, Kim YS. Colonic polyps and the gastrointestinal polyposis syndromes","volume-title":"disease: pathophysiology, diagnosis, and management","author":"Boland, C.R.","year":"1989","unstructured":"Boland CR, Itzkowitz SH, Kim YS. Colonic polyps and the gastrointestinal polyposis syndromes. In: Sleisenger MH, Fordtran JS, eds. Gastrointestinal disease: pathophysiology, diagnosis, and management. 4th ed. Philadelphia: W B Saunders, 1989: 1500-7.","edition":"4th ed"},{"key":"ref_7","first-page":"871","article-title":"Hereditary gastrointestinal polyposis syndromes","volume":"10","author":"Haggitt, R.C.; Reid, B.J.","year":"1986","unstructured":"Haggitt RC, Reid BJ. Hereditary gastrointestinal polyposis syndromes. AmJ SurgPathol 1986; 10: 871-87.","journal-title":"AmJ SurgPathol"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1170","DOI":"10.1136\/gut.32.10.1170","article-title":"The value of combined phenotypic markers in identifying inheritance of familial adenomatous polyposis","volume":"32","author":"Giardiello, F.M.; Offerhaus, G.J.A.; El, Traboulsi; Graybeal, J.C.; Maumenee, I.H.; Krush, A.J.","year":"1991","unstructured":"Giardiello FM, Offerhaus GJA, Traboulsi El, Graybeal JC, Maumenee IH, Krush AJ, et al. The value of combined phenotypic markers in identifying inheritance of familial adenomatous polyposis. Gut 1991; 32: 1170-4.","journal-title":"Gut"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"1980","DOI":"10.1016\/0016-5085(92)90322-P","article-title":"The risk of upper gastrointestinal cancer in familial adenomatous polyposis","volume":"102","author":"Offerhaus, G.J.A.; Giardiello, F.M.; Krush, A.J.; Booker, S.V.; Tersmette, A.C.; Kelley, C.N.","year":"1992","unstructured":"Offerhaus GJA, Giardiello FM, Krush AJ, Booker SV, Tersmette AC, Kelley CN, et al. The risk of upper gastrointestinal cancer in familial adenomatous polyposis. Gastroenterology 1992; 102: 1980-2.","journal-title":"Gastroenterology"},{"key":"ref_10","first-page":"234","article-title":"A case of familial adenomatous polyposis complicated by thyroid carcinoma, carcinoma of the ampulla ofvater and adrenalcortical adenoma","volume-title":"J7pnJ Surg","volume":"21","author":"Ono, C.; Iwama, T.; Mishima, Y.","year":"1991","unstructured":"Ono C, Iwama T, Mishima Y. A case of familial adenomatous polyposis complicated by thyroid carcinoma, carcinoma of the ampulla ofvater and adrenalcortical adenoma.J7pnJ Surg 1991; 21: 234-40."},{"key":"ref_11","first-page":"357","article-title":"The association between thyroid neoplasia and intestinal polyps","volume":"72","author":"Reed, M.W.; Harris, S.C.; Quayle, A.R.; Talbot, C.H.","year":"1990","unstructured":"Reed MW, Harris SC, Quayle AR, Talbot CH. The association between thyroid neoplasia and intestinal polyps. Ann R Coll SurgEngl 1990; 72: 357-9."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"893","DOI":"10.1007\/BF02554564","article-title":"Familial adenomatous polyposis in association with thyroditis. Report of two cases","volume":"32","author":"Herrera, L.; Carrel, A.; Rao, U.; Castillo, N.; Petrelli, N.","year":"1989","unstructured":"Herrera L, Carrel A, Rao U, Castillo N, Petrelli N. Familial adenomatous polyposis in association with thyroditis. Report of two cases. Dis Colon Rectum 1989; 32: 893-6.","journal-title":"Dis Colon Rectum"},{"key":"ref_13","first-page":"229","article-title":"Familial thyroid cancer and rectocolic adenomatous polyposis","volume":"13","author":"Delmarre, J.; Capron, J.P.; Armand, A.; Dupas, J.L.; Deschepper, B.; Davion, T.","year":"1989","unstructured":"Delmarre J, Capron JP, Armand A, Dupas JL, Deschepper B, Davion T. Familial thyroid cancer and rectocolic adenomatous polyposis. Gastroenterol Clin Biol 1989; 13: 229.","journal-title":"Gastroenterol Clin Biol"},{"key":"ref_14","first-page":"29","article-title":"Papillary thyroid carcinoma in Danish patients with familial adenomatous polyposis. J7 Colorectal","volume":"31","author":"Bulow, S.; Holm, N.V.; Mellemgaard, A.","year":"1988","unstructured":"Bulow S, Holm NV, Mellemgaard A. Papillary thyroid carcinoma in Danish patients with familial adenomatous polyposis.J7 Colorectal Dis 1988; 31: 29-31.","journal-title":"Dis"},{"key":"ref_15","first-page":"377","article-title":"Adenomatous polyposis: an association with carcinoma of the thyroid","volume":"75","author":"Plail, R.O.; Bussey, H.J.; Glazer, G.; Thompson, J.P.","year":"1987","unstructured":"Plail RO, Bussey HJ, Glazer G, Thompson JP. Adenomatous polyposis: an association with carcinoma of the thyroid. BrJ Surg 1987; 75: 377-80.","journal-title":"BrJ Surg"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"375","DOI":"10.1002\/1097-0142(19850115)55:2<375::AID-CNCR2820550214>3.0.CO;2-Y","article-title":"Adenomatous polyposis coli and multiple endocrine neoplasia type 2b. A pathogenetic relationship","volume":"55","author":"Perkins, J.T.; Blackstone, M.O.; Riddell, R.H.","year":"1985","unstructured":"Perkins JT, Blackstone MO, Riddell RH. Adenomatous polyposis coli and multiple endocrine neoplasia type 2b. A pathogenetic relationship. Cancer 1985; 55: 375-81.","journal-title":"Cancer"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"639","DOI":"10.1007\/BF02150736","article-title":"Mortality in patients with familial adenomatous polyposis","volume":"33","author":"Arvanitis, M.L.; Jagelman, D.G.; Fazio, V.W.; Lavery, I.C.; McGannon, E.","year":"1990","unstructured":"Arvanitis ML, Jagelman DG, Fazio VW, Lavery IC, McGannon E. Mortality in patients with familial adenomatous polyposis. Dis Colon Rectum 1990; 33: 639-42.","journal-title":"Dis Colon Rectum"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"242","DOI":"10.1111\/imj.1967.16.3.242","article-title":"Gardner's syndrome with adrenal carcinoma","volume":"16","author":"Marshall, W.H.; Martin, F.I.R.; MacKay, I.R.","year":"1967","unstructured":"Marshall WH, Martin FIR, MacKay IR. Gardner's syndrome with adrenal carcinoma. Australian Annals ofMedicine 1967; 16:242-4.","journal-title":"Australian Annals ofMedicine"},{"key":"ref_19","first-page":"1","article-title":"Adrenal adenomas in a patient with Gardner's syndrome","volume":"20","author":"Naylor, E.W.; Gardner, E.J.","year":"1981","unstructured":"Naylor EW, Gardner EJ. Adrenal adenomas in a patient with Gardner's syndrome. Clin Genet 1981; 20: 1.","journal-title":"Clin Genet"},{"key":"ref_20","first-page":"200","article-title":"Adrenal adenomas and adrenal carcinomas in association with hereditary adenomatosis of the colon and rectum","volume":"55","author":"Painter, T.A.; Jagelman, D.G.","year":"1985","unstructured":"Painter TA, Jagelman DG. Adrenal adenomas and adrenal carcinomas in association with hereditary adenomatosis of the colon and rectum. Cancer 1985; 55: 200-1.","journal-title":"Cancer"},{"key":"ref_21","first-page":"76","article-title":"Biliary neoplasia in Gardner's syndrome","volume":"111","author":"Walsh, N.; Qizilbash, A.; Banerjee, R.; Waugh, G.A.","year":"1987","unstructured":"Walsh N, Qizilbash A, Banerjee R, Waugh GA. Biliary neoplasia in Gardner's syndrome. Arch Pathol Lab Med 1987; 111: 76-7.","journal-title":"Arch Pathol Lab Med"},{"key":"ref_22","article-title":"Results (SEER) Program public use tape","author":"Surveillance, Epidemiology; End","year":"1990","unstructured":"Surveillance, Epidemiology, and End Results (SEER) Program public use tape. Washington, DC: National Cancer Institute, DCPC, Surveillance Program. Cancer Statistics Branch, 1990."},{"key":"ref_23","first-page":"698","article-title":"Cohort study analysis with a Fortran computer program","volume":"15","author":"Coleman, M.; Doublas, A.; Hermon, C.; Peto, L.","year":"1986","unstructured":"Coleman M, Doublas A, Hermon C, Peto L. Cohort study analysis with a Fortran computer program. IntJ Epidemiol 1986; 15: 698-703.","journal-title":"IntJ Epidemiol"},{"key":"ref_24","article-title":"Statistical methods in cancer research. Vol II. The design and analysis of cohort studies (IARC Scientific Publications No 82)","author":"Breslow, N.E.; Day, N.E.","year":"1987","unstructured":"Breslow NE, Day NE. Statistical methods in cancer research. Vol II. The design and analysis of cohort studies (IARC Scientific Publications No 82). Lyon: International Agency for Research on Cancer, 1987."}],"container-title":["Gut"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/gut.34.10.1394","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,11,21]],"date-time":"2025-11-21T19:33:32Z","timestamp":1763753612000},"score":1,"resource":{"primary":{"URL":"https:\/\/gut.bmj.com\/lookup\/doi\/10.1136\/gut.34.10.1394"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1993,10]]},"references-count":24,"journal-issue":{"issue":"10","published-online":{"date-parts":[[1993,10,1]]},"published-print":{"date-parts":[[1993,10]]}},"alternative-id":["10.1136\/gut.34.10.1394"],"URL":"https:\/\/doi.org\/10.1136\/gut.34.10.1394","relation":{},"ISSN":["0017-5749","1468-3288"],"issn-type":[{"value":"0017-5749","type":"print"},{"value":"1468-3288","type":"electronic"}],"subject":[],"published":{"date-parts":[[1993,10]]}}}