Development of duodenal cancer in a patient with familial adenomatous polyposis

Abstract

A Japanese woman with familial adenomatous polyposis in whom a duodenal ampullary adenoma underwent malignant change during a 10-year follow-up period is reported. After restorative proctocolectomy in 1989, and extensive small bowel resection for desmoid disease in 1991, regular surveillance duodenoscopies, including three to nine biopsies (mean, 4.8) were performed annually or biannually. Until 1995, the endoscopic findings of duodenal polyposis (including an ampullary polyp) did not progress and the histopathology did not worsen. In 1996, there was an increase in the number and size of the duodenal polyps, and the ampulla of Vater looked enlarged. Open surgery was discussed but not proceeded with because of the risk for short bowel syndrome. In January 1998, she was admitted with a diagnosis of acute pancreatitis. Duodenoscopy and radiological examination revealed that an advanced ampullary cancer had developed, and histopathology revealed a well-differentiated adenocarcinoma. Multiple hepatic metastases and ascites led to her death, in June, 1998. This in-vivo demonstration of the adenoma-carcinoma sequence highlights current limitations in the surveillance and treatment of duodenal lesions.

Recurrent pancreatitis caused by ampullary carcinoma and minor papilla adenoma in familial polyposis: Report of a case

“…Our PubMed search of the literature, using "pancreatitis," "ampulla of Vater," and "FAP" as key words, identifi ed only 12 reported cases, including the present case ( Table 2). [7][8][9][10][11][12][13] This case is particularly interesting in that both the major and minor duodenal papillae were involved and obstructed by duodenal adenomas. Our survey of the literature revealed only one similar case, reported by Wright et al 11 Although the pathophysiology of the chronic obstructive pancreatitis in our patient involved a complex interaction of various factors, the retention of active pancreatic enzymes within the acinar cells and pancreatic ducts, and mechanical stress caused by high intraductal pressure might have played important roles in its development.…”

“…[1][2][3][4] Although patients with a periampullary tumor occasionally present with acute or recurrent pancreatitis (obstructive jaundice is the presenting symptom of ampullary neoplasm in approximately two-thirds of cases), 5,6 there is little information available regarding pancreatitis associated with ampullary neoplasms in FAP patients. [7][8][9][10][11][12][13] The pancreatitis that develops secondary to an ampullary tumor in patients with FAP is distinct from the disease in patients without FAP because it is frequently accompanied by multiple premalignant duodenal adenomas. [10][11][12][13] We report a rare case of relapsing pancreatitis secondary to ampullary carcinoma in a patient with FAP and severe duodenal adenomatosis.…”

“…[7][8][9][10][11][12][13] The pancreatitis that develops secondary to an ampullary tumor in patients with FAP is distinct from the disease in patients without FAP because it is frequently accompanied by multiple premalignant duodenal adenomas. [10][11][12][13] We report a rare case of relapsing pancreatitis secondary to ampullary carcinoma in a patient with FAP and severe duodenal adenomatosis. This case is particularly interesting because both the major and minor duodenal papillae were involved by these duodenal neoplasms simultaneously.…”

Relapsing acute pancreatitis due to ampullary adenoma in a patient with familial adenomatous polyposis

“…11,[16][17][18][19][20][21][22] Most patients in these reports had recurrent attacks of acute pancreatitis because of periampullary tumors, which were pathologically adenoma, 15,[19][20][21] carcinoma in situ, 17 or adenocarcinoma. 11,18,22 In our case, the history of the patient included no alcohol intake or drug use that might have caused pancreatitis. She had an ampullary adenoma with moderate atypia and dilatation of the main pancreatic duct, which apparently caused the relapsing acute pancreatitis.…”

Prevention and management of duodenal polyps in familial adenomatous polyposis

“…Also, case reports of duodenal carcinoma development in or near adenomas have been described. 32 33 Moreover, Kashiwagi et al noted p53 overexpression in 25% of tubular, 72% of tubulovillous/villous adenomas, and 100% of duodenal carcinomas, 34 and K-Ras codon 12 mutations have been detected in duodenal adenomas and carcinomas. 35 In addition, SMAD4 mutations play a role in polyp development in the upper intestine in mice.…”