Tumor association in cases of Cronkhite-Canada syndrome

We report a 63-year-old lady with Cronkhite-Canada syndrome, who developed colorectal cancer. A hemicolectomy was performed, and the tumor specimen wa s prepared for DNA-analysis and immunohistochemical screening. We found a m utation of p53 gene without APC- and ras-gene alteration and expression of erbB2-protooncogen. The polyps in non-hereditary Cronkhite-Canada-syndrom are neither adenomato us nor hyperplastic, but patients often develop colorectal cancers. The ste ps of mutation do not follow the adenoma-carcinoma sequence, first describe d by Vogelstein 1988. This and previous observations suggest that carcinoge nesis in Cronkhite-Canada syndrome follows another independent sequence.