Clinical Characteristics of Gastric Cancer in Patients with Familial Adenomatous Polyposis

Abstract

Familial adenomatous polyposis is an autosomal dominant hereditary disease leading to the development of numerous colorectal polyps with malignant potential. Extra-colonic neoplasms are observed often in patients with familial adenomatous polyposis, but clinical characteristics of gastric cancer associated with familial adenomatous polyposis are not well understood. We studied the clinical characteristics of five Japanese patients who developed gastric cancer after undergoing colectomy for familial adenomatous polyposis. Gastric cancer was found on gastroduodenal endoscopy performed during postoperative follow-up in all five patients. Mean ages at the time of colectomy and at the time of diagnosis of gastric cancer were 39.2 and 58 years, respectively. Importantly, gastric fundic gland polyps were undetectable in these five patients. The mean duration between colectomy and diagnosis of gastric cancer was more than 20 years in three of five patients (mean: 19 years and 1 month). Cancers were multiple in three of five patients. Two patients developed metachronous gastric cancer in the gastric remnant. All five patients presented with well to moderately differentiated adenocarcinoma; four of the five patients were stage IA. Characteristics of the gastric cancer with familial adenomatous polyposis include a long duration between occurrence of gastric cancer and colectomy, metachronous cancers, multicentric lesions, and a high rate of well to moderately differentiated adenocarcinoma. Long-term and periodic gastroduodenal surveillance endoscopy is recommended for patients with FAP who underwent colectomy.

Accuracy of Hereditary Diffuse Gastric Cancer Testing Criteria and Outcomes in Patients With a Germline Mutation in CDH1

“…Gastric cancer has also been associated with germline mutations in DNA mismatch repair genes, TP53, APC, BRCA1/2, STK11 and PTEN. [40][41][42][43][44][45] Nonetheless, the molecular basis for familial aggregation of gastric cancer remains largely unknown. Endoscopic surveillance strategy for families that fulfill the HDGC criteria, but in whom no pathogenic mutation can be identified, is complicated.…”

Family History and Survival of Patients with Gastric Cancer: A Meta-Analysis

“…Further, the post-operative prognosis for early hereditary diffuse gastric cancer is likely to be excellent (Guilford et al, 2007). In addition, gastric cancer development in patients with familial adenomatous polyposis (Shibata et al, 2013) usually yields a better prognosis. In this meta-analysis, only one study excluded hereditary gastric cancer, such as HNPCC (Fang et al, 2013).…”

Familial adenomatous polyposis: a review of gastrointestinal manifestations

“…62 These patients are symptomatic in only w12% cases 60 ; hence, clinical symptoms cannot be used as a guide to initiate an upper gastrointestinal workup. Gastric carcinomas can develop after a long interval following colectomy (>20 years), 63 but have been diagnosed as early as 11 years of age. 60 Pathologic findings: syndromic FGPs are morphologically similar to sporadic polyps, and are composed of polypoid gastric foveolar mucosa with cystically dilated oxyntic glands lined by parietal cells, chief cells, and mucous neck cells.…”

“…73,74 Reported gastric adenocarcinomas are WHO tubular type (Lauren intestinal type), well to moderately differentiated 63,75 multicentric or metachronous. 63 However, gastric carcinomas have also been seen arising de novo (without previous lesions/not arising in FGP or GA). 76 …”