十二指腸癌を合併したGardner症候群の1例

Gardner Syndrome Associated with Duodenal Cancer, Report of a Case

　家族性大腸腺腫症に十二指腸傍乳頭部癌が合併することはCabotの報告¹⁾以来注目されている．また，同疾患に上部消化管ポリポージスが高頻度に随伴することが，最近の日本における調査研究にて明らかになってきた．このたび，われわれはGardner症候群に，直腸癌，十二指腸癌および胃・十二指腸腺腫症を合併した症例を経験したので報告する．

　A case of Gardner syndrome with interesting history was presented.

　A 29-year-old man received an abdominoperineal resection with rectal bleeding and an ulcerating growth on barium enema in August 1962. Histologic examination revealed a well-differentiated adenocarcinoma invading into the proper muscle of the lower rectum and a pedunculated adenoma with focal carcinoma, but no other adenoma was found (Fig. 2). When he visited our clinic with a polyp of his artificial anus in August 1973 he was found to have numerous colonic adenomas, osteomas of bilateral mandibles and epidermoid cysts on the back and diagnosed as Gardner syndrome for the first time (Fig. 3). He was followed with colonoscopy and polypectomy every several months. Although polyps did not change in size and number until February 1978, they seemed to increase in size and number during the next one year (Fig. 4). In August 1979, he was readmitted to our hospital with continuous epigastralgia and anemia. Barium meal and endoscopic examination revealed an ulcerating growth of the second portion of the duodenum and multiple gastroduodenal polyps (Fig. 5). A pancreaticoduodenectomy was performed with uneventful course.

　The second portion of the duodenum showed an ulcerating tumor (8.5×10 cm), a large pedunculated polyp (1.5×2.5cm) and numerous tiny polyps throughout the duodenum, and the antrum of the stomach showed several sessile polyps with central depression (Fig. 6). Histologically, the ulcerating tumor was a well-differentiated adenocarcinoma with small adenomatous remnant invading into the pancreas-head without obstruction of the choledochus and the pancreatic duct (Fig. 7). The pedunculated polyp was an adenoma with focal carcinoma (Fig. 8). Tiny sessile polyps were adenomas with slight dysplasia and adenomas consisted of one or several glands were also found in the flat mucosa (Fig. 9). Sessile polyps of the antrum were adenomas occupying only upper half of the gastric mucosa (Fig. 10). Step serial sections of the surgical specimen disclosed 256 adenomas in the duodenum and 91 adenomas in the stomach (Fig. 11).

　World literature was reviewed and 29 cases of a periampullary carcinoma and 12 cases of a gastric carcinoma associated with adenomatosis coli were summerised (Fig. 12). From our experience, importance of upper gastrointestinal examination in adenomatosis coli was stressed.