Abstract
Multiple primary cancers occurring in the same patients have been reported to represent 1.8–3.9% of all cancers. The majority of all patients reported to have had a combination of simultaneous neoplastic changes in the ampulla of Vater and the colon showed familial adenomatous polyposis (FAP) syndrome.
Variants of familial adenomatous polyposis coli are: attenuated adenomatous polyposis coli (AAPC, previously also known as flat adenoma syndrome) and multiple adenoma coli. AAPC is characterized clinically by many, but usually fewer than 100, colonic lesions that are characteristically slightly elevated and plaque-like, with a reddish surface and sometimes central depression. Genetically it represents an extremely rare variant of FAP. Another group of individuals, so-called multiple adenoma patients, have a phenotype similar to AAPC, but most have no demonstrable germ-line adenomatous polyposis coli mutation, as do patients with FAP or AAPC.
However, there have been only a few reports that discussed concurrent neoplastic changes in the ampulla of Vater and colon in patients with multiple colonic flat adenomas, but without the florid phenotype of classical FAP. We present rare clinical course of a patient with multiple (more than 60) flat adenomas in the proximal colon and two primary cancers: of the ampulla of Vater and of the ascending colon. This patient and his family history did not show polyposis compatible with FAP or hereditary nonpolyposis colorectal cancer (HNPCC) syndrome.
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References
Warren S, Gates O. Multiple primary malignant tumors. A study of the literature and a statistical study. Am J Cancer 1932;16:1358–1414.
Utsunomiya J, Nakamura T. The occult osteomatous changes in the mandible in patients with familial polyposis coli: studies of lesions of the stomach, duodenum, bones and teeth. Gastrointest Radiol 1976;1:67–80.
Gardner EJ, Richards RC. Multiple cutaneous and subcutaneous lesions occurring simultaneously with hereditary polyposis and osteomatosis. Am J Hum Genet 1953;5:193–147.
Watanable H, Enjoji M, Yao T, et al. Accompanying gastroenteric lesions in famillial adenomatosis coli. Acta Pathol Jpn 1977;27:823–839.
Shemes E, Bat L. A prospective evaluation of the upper gastrointestinal tract and periampullary region in patients with Gardner syndrome. Am J Gastroenterol 1985;80:825–827.
Watanabe H, Iida M, Iwafuchi M, et al. Pathology of gastroenteric lesions in familial adenomatosis coli, in Hereditary Colorectal Cancer. Utsunomiya J, Lynch HT, eds. Tokyo: Springer-Verlag, 1990, pp. 321–330.
Yao T, Iida M, Ohsato K, Watanabe H, Omae T. Duodenal lesions in familial polyposis of the colon. Gastroenterology 1977;73:1086–1092.
Utsunomiya J, Maki T, Iwama T, et al. Gastric lesion of familial polyposis coli. Cancer 1974;34:745–754.
Jarvinen H, Nyberg M, Peltokallio P. Upper gastrointestinal tract polyposis in familial adenomatosis coli. Gut 1983;24:333–339.
Burt RW, Berenson MM, Lee RG, et al. Upper gastrointestinal polyps in Gardner’s syndrome. Gastroenterology 1984;86:295–301.
Iida M, Yao T, Itoh H, et al. Natural history of duodenal lesions in Japanese patients with with familial adenomatosis coli (Gardner’s syndrome). Gastroenterology 1989;96:1301–1306.
Pauli RM, Pauli ME, Hall JG. Gardner syndrome and periampullary malignancy. Am J Med Genet 1980;6:205–219.
Iida M, Yao T, Itoh H, et al. Natural history of gastric adenomas in patients with familial adenomatosis coli/Gardner’s syndrome. Cancer 1988;61:605–611.
Noda Y, Watanabe H, Iida M, et al. Histologic follow-up of ampullary adenomas in patients with familial adenomatosis coli. Cancer 1992;70:1847–1856.
Muto T, Kamiya J, Sawada T et al. Small “flat adenoma” of the large bowel with special reference to its clinicopathological features. Dis Colon Rectum 1985;28:847–851.
Muto T, Masaki T, Suzuki K. DNA ploidy pattern of flat adenomas of the large bowel. Dis Colon Rectum 1991;34:696–698.
Kuramoto S, Oohora T. Flat early cancers of the large intestine. Cancer 1989;64:950–955.
Kudo S. Histological diagnosis of flat and depressed types of early colorectal cancer: strip biopsy and dealing with the material. Gastroenterol Endosc 1989;31:2845–2846.
Spirio L, Olschwang S, Groden J, et al. Alleles of the APC gene: an attenuated form of familial polyposis. Cell 1993;75:951–957.
Lynch HT, Smyrk TC, Watson P, et al. Hereditary flat adenoma syndrome: a variant of familial adenomatous polyposis? Dis Colon Rectum 1992;35:411–421.
Samowitz, Wade S. Gastrointestinal polyposis and nonpolyposis syndromes. N Engl J Med 1995;1;1518–1520.
Adachi M, Okinaga K, Muto T. Flat adenoma of the large bowel. Re-evaluation with special refference to central depression. Dis Colon Rectum 2000;43:782–787.
Rustgi AK. Medical progress: hereditary gastrointestinal polyposis and nonpolyposis syndromes. N Engl J Med 1994;331:1694–1702.
Beck NE, Tomlinson IPM, Homfray TFR, et al. Frequency of germline hereditary non-polyposis colorectal cancer gene mutations in patients with multiple or early onset colorectal adenomas. Gut 1997;41:235–238.
Harned RK, Williams SM. Familial polyposis coli and periampullary malignancy. Dis Colon Rectum 1982;25:227–229.
Qizilbash AH. Famillial polyposis coli and periampullary carcinoma. Can J Surg 1976;19:166–168.
MacDonald JM, Davis WC, Crago HR, Berk AD. Gardner’s syndrome and periampullary malignancy. Am J Surg 1967;113:425–430.
Lynch HT, Smyrk TC, Lanspa SJ, et al. Upper gastrointestinal manifestations in families with hereditary flat adenoma syndrome. Cancer 1993;71:2709–2714.
Morson B. The polyp-cancer sequence in the large bowel. Proc R Soc Med 1974;67:451–457.
Fuji T, Rembacken BJ, Dixon MF, et al. Flat adenomas in the United Kingdom: are treatable cancers beeing missed? Endoscopy 1998;30:437–443.
Kudo S, Tamura S, Hirota Y, et al. The problem of the novo colorectal carcinoma. Eur J Cancer 1995;31:1118–1120.
Rembacken BJ, Fujji T, Cairns A, et al. Flat and depressed colonic neoplasms: a prospective study of 1000 colonoscopies in the UK. The Lancet 2000;355:1211–1214.
Ljubicic N, Kujundzic M, Banic M, Roic G. The role of standard videochromocolonoscopy in distinuishing adenomatous from nonadenomatous diminutive colorectal polyps. Acta Clin Croat 2001;40:197–201.
Wolber RA, Owen DA. Flat adenoma of the colon. Hum Pathol 1991;34:981–986.
Capps WF Jr, Lewis MI, Gazzangia DA. Carcinoma of the colon, ampulla of Vater and urinary bladder associated with familial multiple polyposis: a case report. Dis Colon Rectum 1968;11:298–305.
Sieber OM, Lamlum H, Crabtree MD, Rowen AJ, et al. Whole-gene APC deletions cause classical familial adenomatous polyposis, but not attenuated polyposis or “multiple” colorectal adenomas. Proc Natl Acad Sci USA 2002;99:2954–2958.
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Doko, M., Zovak, M., Glavan, E. et al. Synchronous primary carcinomas of the ampulla of vater and ascending colon in a patient with multiple flat adenomas. Int J Gastrointest Canc 33, 117–121 (2003). https://doi.org/10.1385/IJGC:33:2-3:117
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DOI: https://doi.org/10.1385/IJGC:33:2-3:117