Upper gastrointestinal cancer in patients with familial adenomatous polyposis.

https://www.giejournal.org/article/S0016-5107(15)02599-7/pdf

The role of endoscopy in ampullary and duodenal adenomas

Familial adenomatous polyposis coli (FAP) is an autosomal dominant genetic disorder caused by mutations of the APC gene on the long arm of chromosome 5. While multiple colorectal adenomas usually developing in early adolescence represent the most conspicuous phenotypic feature, the disease represents a generalized hyperproliferative disorder with various extracolonic manifestations. Duodenal cancer and desmoids are the leading causes of death in FAP patients after prophylactic colectomy. The prevalence of duodenal adenomatosis among FAP patients varies from 50% to greater than 90%, while only few patients (3-5%) develop duodenal cancer. Periampullary adenomas seem to carry a high risk of malignant transformation. The sensitivity of endoscopic procedures for early detection of severely dysplastic or malignant duodenal lesions is low, and the prognosis of duodenal cancer is poor. Thus the question arises whether it is possible to define a subgroup of high-risk patients for duodenal malignancy, and whether severe duodenal adenomatosis should lead to an aggressive prophylactic surgical approach. This contribution discusses the current literature and presents the experience of the Heidelberg Polyposis Register based on gastroduodenoscopy findings in 231 FAP patients. In 135 cases (58.4%) macroscopic duodenal adenomas were observed. The majority of patients displayed numerous lesions throughout the duodenum, while adenomas were restricted to the periampullary region in ten. Four patients suffered from duodenal cancer. Twenty-two required an endoscopic or operative intervention. Five were treated by laparotomy and duodenotomy, while ampullary excision was indicated in six cases. Eight patients underwent partial pancreaticoduodenectomy for severe duodenal adenomatosis.

Duodenal adenomatosis in familial adenomatous polyposis coli. A review of the literature and results from the Heidelberg Polyposis Register

Gastrointestinal polyposis syndromes.

Pancreas-sparing duodenectomy: indications, surgical technique, and results.

Upper gastrointestinal polyps and carcinoma, especially of the periampullary area, may occur in patients with familial adenomatous polyposis. Upper gastrointestinal polyps were present in about 43% of patients in the polyposis registry of the Cleveland Clinic who underwent screening esophagogastroduodenoscopy. Approximately one-third of these polyps were neoplastic. Three distribution patterns were recognized: 1. fundic, 2. duodenal, and 3. fundic plus duodenal. Despite the occurrence of a few gastric cancers in patients with familial adenomatous polyposis, the risk of carcinoma of the major duodenal papilla and periampullary area is more significant. It is necessary to examine the duodenal papilla with a side-viewing instrument and to obtain biopsy specimens from this structure in these patients. The major duodenal papilla was abnormal in about one-half of our patients in whom this structure was specifically examined with a side-viewing endoscope. The preliminary results of a trial of endoscopic treatment of upper gastrointestinal polyps and lesions of the major duodenal papilla in patients with familial adenomatous polyposis are described.

Endoscopic management of upper gastrointestinal polyps and periampullary lesions in familial adenomatous polyposis and Gardner's syndrome.

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Endoscopic management of upper gastrointestinal polyps and periampullary lesions in familial adenomatous polyposis and Gardner's syndrome.