A clinical overview of familial adenomatous polyposis derived from the database of the Polyposis Registry of Japan

Abstract

The clinical situation of familial adenomatous polyposis (FAP) in Japan has changed in the period since the last analysis of data of the Japanese Polyposis Center. To reevaluate our data and elucidate the changes we analyzed the records of the 1390 FAP patients in 900 families registered with the Polyposis Committee of the Japanese Society for Cancer of the Colon and Rectum. In the 13-year period 1990-2003, clinical differences between men and women with FAP diminished. The postoperative prognosis was substantially better in patients without advanced colorectal cancer (stage > or = T2) than in those with early cancer or no cancer. Mean age at death improved from 42.5 years in the period before 1990 to 46.0 years, and it was a result of a decreased proportion of deaths from colorectal cancer. The distribution of colorectal cancer in FAP patients was similar to that in the general population. Desmoid tumors accounted for about 10% of deaths in the recent 13 years (1990-2003). The cumulative risk of rectal cancer in the preserved rectum was 12% at 10 years and 23% at 15 years. The registry system in Japan revealed a new clinical situation in FAP patients, and the findings of this study will be useful to improve the prognosis of patients with FAP.

Surgical treatment of familial adenomatous polyposis: ileoretal anastomosis or restorative proctolectomy?

“…This variation may be attributed to colectomies in different extensions or surgical indication in different ages. This risk rises progressively with length of follow-up, reaching 5%-12% after 10 years (26,27) , 23% after 15 years (27) and even 50% after 20 years from operation (20,29) . Data from the Polyposis Registry in Sweden indicate a cumulative risk of 25.7% at 70 years of age (3) .…”

Solid Pseudopapillary Neoplasm of the Pancreas Associated with Familial Adenomatous Polyposis

“…Colorectal cancer inevitably develops unless the tumors are removed (15,16). FAP patients may also develop a variety of extra-large intestinal and extra-intestinal lesions, including duodenal papillary tumors, desmoid tumors and thyroid tumors (10,17). A point mutation in exon 3 of CTNNB1, which encodes β-catenin, is detected in most cases of SPN.…”

Advanced duodenal neoplasia and carcinoma in familial adenomatous polyposis: outcomes of surgical management

“…Clinical and surgical data concerning previous FAP treatment are shown in Table 1. Two women and 6 men with endoscopic features of classic FAP had undergone ileal-rectal anastomosis (IRA) or proctocolectomy (PC) at a mean age of 36.5 years (17-50 years), most of them [7] presenting family history of polyposis. CRC was present in the colorectal resected specimen in three patients, two of which developed duodenal cancer.…”

“…In the present series, most patients [7] were submitted to pancreatoduodenectomy (PD) (Whipple's operation), 5 of them for cancer and 2 involving exclusively the Vater papilla. This operation and also the pancreas-sparing duodenectomy (PSD) may be potentially curative, besides the operative risks.…”

“…As a consequence of the widespread indication of prophylactic colectomy, mortality from CRC has decreased substantially over the years (2)(3)(4), making duodenal cancer and desmoid tumors important causes of death in FAP patients (5)(6)(7)(8)(9).…”